Congenital EP Featured Articles of January 2017

Relation of Left Atrial Size to Atrial Fibrillation in Patients Aged ≤22 Years.

Mah DY, Shakti D, Gauvreau K, Colan SD, Alexander ME, Abrams DJ, Brown DW.

Am J Cardiol. 2017 Jan 1;119(1):52-56. doi: 10.1016/j.amjcard.2016.09.008.

PMID: 27780555

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Select item 27780553

Take Home Points

  • Left atrial dilation is not typically associated with atrial fibrillation in young patients without underlying heart disease
  • Close attention to triggers of atrial fibrillation, including systemic infection and environmental exposures, can offer individualized non-medical treatment options in young patients who have experienced an episode of lone atrial fibrillation
  • A stepwise management approach to lone atrial fibrillation in young patients can include integration of diagnostic EPS in patients with persistent symptoms or documented atrial fibrillation recurrence, with consideration of ablation therapies directed toward atrial fibrillation substrates (i.e. pulmonary vein isolation in most) for symptomatic patients without other conventional arrhythmia substrates/mechanisms and evidence indicating pulmonary vein targets


Commentary from Dr. Philip Chang (Los Angeles), section editor of Pediatric Cardiac EP Journal Watch:

chang-philip-1780821827Article summary:The occurrence and management of lone atrial fibrillation (AF), or AF in individuals without underlying heart disease, is more commonly encountered in adult electrophysiology settings, but is relatively rare in most pediatric electrophysiology practices.  Atrial fibrillation in adults can be associated with left atrial dilation (LAD).  In this article, Mah et al sought to evaluate if LAD is an associated echocardiographic finding among young patients without underlying heart disease who have experienced at least 1 documented episode of AF prompting medical attention.

The study was a retrospective review of all patients <22 years of age who presented to a single institution with AF over a 10-year period (1/2002-12/2012).  Those with congenital heart disease (excluding hemodynamically inconsequential bicuspid aortic valve and simple shunt lesions), cardiomyopathies, channelopathies, prior cardiac surgery, prior arrhythmias treated with catheter ablation, as well as end-stage renal disease and severe lung disease were excluded.  Demographic data, clinical presentation, ECG, echo findings, and EPS findings if performed were analyzed.

In total, 135 patients presented with atrial fibrillation over the study period, of whom 87 were excluded given the above mentioned criteria.  Table 1 describes the baseline characteristics of the lone AF cohort.

ep 1

Of these 48 patients all had structurally normal hearts and the majority were male (79%).  The median age was 17.2 years (3.7-22.9 years).  Most patients had only 1 documented episode of AF (“isolated AF” = 37 patients), with 11 patients (23%) having at least 1 recurrence (“recurrent AF”).  Median follow-up was longer among those with recurrent AF (4.9 years vs. 0.1 years) and 15 patients with a single AF event had no follow-up.  Nearly 30% of patients were obese (BMI >30 kg/m2) and 21% were hypertensive.  Among the entire cohort, alcohol use (17%), illicit drug use (6%), and stimulant use (4%) were documented.

The authors noted that an inciting event was attributable in 10 of 37 isolated AF patients – 4 systemic infections, 3 chest trauma, 1 post-scoliosis surgery, 1 bronchodilator therapy in the setting of asthma exacerbation, and 1 macrophage activation syndrome.  Among females with lone AF (10 patients), most had some attributable event or exposure (7/10).

Echo findings (Table 2) among all lone AF patients revealed incidental LVH in 4 patients (9%) and only 1 isolated AF and 1 recurrent AF patient with LA dimension z-scores >2.  For the isolated AF patient, binge drinking was documented.  For the recurrent AF patient, polymorphic VT was noted on EPS and short QT syndrome was suspected.

ep 2

Clinical presentation and AF termination were reported for most patients, with the majority of isolated AF patients experiencing spontaneous termination (59%), 19% undergoing electrical cardioversion, and 7% receiving medications for pharmacologic conversion.  Medication was infrequently initiated afterward (total 4 patients).  Among recurrent AF patients, most experienced spontaneous conversion at their first episode (6/10), with 3 electrical and 2 pharmacologic conversions among the other patients.

Diagnostic EPS was employed in 7/48 patients (4 isolated AF, 3 recurrent AF).  For the 4 isolated AF patients, EPS was performed secondary to persistent palpitations.  In 3 of these 4 patients, AVRT (2) and atrial flutter (1) were diagnosed.  Among the recurrent AF patients, 1 had AVNRT and focal AT, with recurrent AF still noted afterward, and 1 patient underwent PVI following failure of antiarrhythmic therapy with AF recurrences.  The third recurrent AF patient was mentioned above with suspected short QT syndrome.

In their discussion, the authors noted the known high frequency of LAD in adult AF, with chamber enlargement being a predictor of first AF occurrence, as well as AF recurrence.  Its finding is also frequently seen in AF associated with other structural heart disease including HCM, coronary disease, and prior cardiac surgery.  The contribution of LAD to AF is likely complex and multifactorial.  Its absence in pediatric lone AF would suggest other factors and contributors to AF occurrence in this young population.  The authors noted other published data on altered left atrial function as being more predictive of AF recurrence compared to chamber dilation, noting that this could possibly be an unrecognized finding that contributes to AF in young patients where chamber dilation is absent and perhaps before dilation develops in the future.

Study limitations include a small study cohort (despite it being the largest published lone AF study cohort) that is further limited by a substantial number of patients that were lost to follow-up.  Family history of AF was not investigated, but would certainly be valuable to know, particularly with growing data on AF-associated genes and potential for familial AF cohorts.


Reviewer perspective and thoughts for pediatric/CHD EP:

Pulmonary vein firing and fibrillatory behavior is generally considered the dominant driver of AF in adult paroxysmal AF.  This forms the basis for RF or cryo-based ablative interventions for the condition in symptomatic adult patients.  Alterations in the electrophysiological characteristics of atrial myocytes in dilated atria, along with fibrosis (either due to structural disease or AF-related remodeling), are probably significant contributors to the development and/or propagation of AF.  Autonomics are also frequently involved in the development and recurrence of AF.

The lack of LAD among pediatric lone AF patients is not particularly surprising, particularly if one views pediatric lone AF as being on a general continuous spectrum of AF in the pediatric and adult populations.  In this regard, 2 conclusions that can be made from this study’s findings are: 1) pediatric lone AF may represent an early-staged presentation of the overall AF disease process, and 2) similar to adult paroxysmal AF, pediatric AF, at least early on, is likely and predominantly driven by pulmonary vein triggers (particularly if other primary and common arrhythmias are absent).  Associated factors such as alcohol and illicit drug exposure and systemic illnesses (which could alter systemic autonomics) may drive pulmonary vein firing resulting in lone AF occurrence in susceptible individuals.

The findings from the study provide an important starting point for further investigation into other mechanisms and features in pediatric lone AF that may permit better determination of those at risk of AF development and AF recurrence, as well as more optimal and targeted approaches to treatment.

A systematic approach to lone AF management is suggested and encouraged from this study and appears quite reasonable.  Identification of external triggers and treatment directed toward these suspected contributing factors as well as obesity and general CV risk factors are important.  Diagnostic EPS is reasonable to consider in individuals with persistent symptoms or document AF recurrence.  Conventional ablation therapy similar to what is employed in adult AF seems reasonable to consider in patients with recurrent, drug refractory AF in the absence of other treatable arrhythmia substrates and following thorough discussion with patients and families regarding potential risks.


Prognostic Implications of Defibrillation Threshold Testing in Patients with Hypertrophic Cardiomyopathy.

Francia P, Adduci C, Semprini L, Palano F, Santini D, Musumeci B, Santolamazza C, Volpe M, Autore C.

J Cardiovasc Electrophysiol. 2017 Jan;28(1):103-108. doi: 10.1111/jce.13121.

PMID:  27862589

Similar articles


Take Home Points

  • Defibrillation efficacy with conventional ICD programming and contemporary ICD devices in HCM is high regardless of whether implant defibrillation testing is performed
  • Performance of defibrillation testing at ICD implant does not appear to identify those at risk of defibrillation failure during spontaneous VT/VF events in HCM
  • Routine defibrillation testing at ICD implant may be unnecessary in most patients with HCM undergoing primary and secondary ICD implants


Commentary from Dr. Philip Chang (Los Angeles), section editor of Pediatric Cardiac EP Journal Watch:

Article summary:

The routine inclusion of defibrillation testing during ICD implantation is increasingly being eliminated across various cardiac diseases.  However, its inclusion in primary and secondary ICD implants in HCM remains debated.  In this study, Francia et al sought to evaluate outcomes of functional defibrillation testing during implant and its usefulness in predicting shock efficacy during spontaneous VT/VF events among an adult cohort undergoing ICD implantation and follow-up.

The authors retrospectively reviewed all HCM patients who underwent ICD implantation at a single institution from 2003 to 2015.  A total of 66 patients were included (41 male patients, mean age 45 years).  The vast majority of patients underwent primary prevention implant (65/66) with standard accepted indications for ICD implant in HCM.  This contemporary cohort also included 3 patients who received the subcutaneous ICD (SICD) while the remainder received transvenous devices.

Functional defibrillation testing was performed in 38% of patients (25/66), with the decision to test left to the discretion of the implanting physician.  Table 1 compares the baseline characteristics among those who did and did not undergo testing.

ep 3

Testing was performed with ICD-induced VF and subsequent shock delivery at an output at least 10 J below the maximum output of the device (safety margin testing).  All patients were subsequently followed with routine clinical and ICD follow-up.  The occurrences of appropriate and inappropriate ICD shocks were documented.

Among the entire cohort, most patients had family history of sudden death (60%) and nonsustained VT (68%) as major risk factors driving ICD implant.  Massive LVH defined as maximal LV thickness ≥30mm was present in 35%.  Patients who underwent defibrillation testing tended to be younger, to have massive LVH, and to have single coil transvenous systems.  Other clinical and device variables did not differ significantly among those who did or did not undergo defibrillation testing.

The authors noted differences in maximum ICD device shock outputs based on implant “eras” (2003-3005, 2005-2012, and 2009-2015).  Successful first-shock defibrillation at safety margin-derived values at least 10 J below maximum output was achieved in all 25 patients who underwent testing.  Of the 3 SICD patients who underwent testing, all were successfully defibrillated with a single shock of 65 J or less (as recommended by the manufacturer at the time of implant).  The mean shock output was 27 ± 15 J (22 ± 3 J after excluding the SICDs).  The mean difference between tested shock output and maximum device output was 15 ± 3 J, which did not change with or without inclusion of SICD patients.  The authors also reported no intraprocedural complications during defibrillation testing.  The authors further reviewed the subgroup of patients with massive LVH, noting that 15/23 massive LVH patients underwent defibrillation testing and that 15/25 patients who underwent defibrillation testing had massive LVH.  Tested shock outputs and safety margins were comparable among patients with and without massive LVH. (Table 2)

During follow-up (median 53 months), a total of 14 patients experienced 54 VT/VF events.  Among these 54 events, 12 were treated with eventual ICD shocks.  Shock therapy was the first ICD treatment delivered in 7 episodes and after failed ATP in 5 episodes among 7 patients. (Fig 1)

ep 5


The first shock was successful in 9 episodes.  For the remaining 3 episodes, the authors reported 1 patient with massive LVH and implant testing success at <20 J who failed defibrillation at 35 J twice before successfully converting with a third 35 J shock.  Another individual without massive LVH and without implant testing failed to convert with an initial 30 J shock but successfully converted with a 36 J delivery.  A third individual without massive LVH or implant testing was shocked for fast VT, resulting in VF that spontaneously converted.  A total of 4 VT/VF episodes occurred in 2 patients who underwent implant testing while 8 occurred in 5 who did not undergo testing.  A 75% successful first shock efficacy was present in both sets of patients.

The authors noted that massive LVH, younger age, and single-coil systems were more likely to prompt implant defibrillation testing.  It is possible that these 3 variables are inter-related, with massive LVH tending to be observed in younger patients and younger patients tending to get single-coil ICD leads.  They further concluded that defibrillation testing using a safety margin driven approach was 100% successful and that implant testing did not predict shock efficacy for subsequent spontaneous events.

Study limitations include a retrospectively studied and relatively small cohort of patients.  Furthermore, VT/VF events were infrequent, making more robust conclusions regarding shock efficacy or failure more difficult.  It is also worth noting that with a safety margin driven approach to device testing, the tested shock output would actually increase with more contemporary devices that are capable of delivering higher maximum shock energy values, which would intuitively and simplistically be expected to increase implant testing success.  Precise defibrillation thresholds were not measured by ULV or step-down shock testing and therefore the margin between the safety margin determined shock output and true defibrillation threshold is unknown.  The authors did not evaluate or report on changes that may have occurred over time in terms of HCM disease progression, HCM-related symptoms, echo findings and/or changes, or other environmental and non-cardiac factors that could potentially have affected shock efficacy, though efficacy was high overall regardless of these factors.  Finally, this study did not specifically include pediatric patients and extrapolation of data from adults may be limited.

Reviewer perspective and thoughts for pediatric/CHD EP:

Findings from this study would suggest that defibrillation testing at ICD implant is not routinely necessary in pediatric patients with HCM by general extrapolation of this adult patient experience.  Risks of defibrillation testing have been previously described and, while infrequent, can result in very serious clinical sequelae including consequences of protracted and potentially hemodynamically significant episodes of VT/VF (if shocks are unsuccessful), risks associated with CPR and code drug administration, risks of potential lead dislodgement from interventions, and prolongation of procedure durations (particularly if lead repositioning or replacement are needed).  With the very high shock efficacy of contemporary high-energy ICDs across a variety of primary cardiac processes, and this study’s observation of high shock efficacy even in massive LVH forms of HCM, this would stand to favor grouping HCM with other cardiac diagnoses where routine testing is not performed rather than singling it out as a condition that always requires special testing considerations.  Exceptions to this would be in situations with extreme LVH (>45mm) and in the setting of medications that can increase defibrillation thresholds (both also noted by the authors).  Though not principally evaluated by the authors, the study also showed that single coil ICD systems in HCM were highly efficacious.  Results with the SICD, though used in only a few patients in this study, are also encouraging in terms of implant safety, successful defibrillation testing at implant, and lack of reported complications during follow-up.

CHD EP Jan 2017

1. Cardiac resynchronization therapy in adults with congenital heart disease.

Koyak Z, de Groot JR, Krimly A, Mackay TM, Bouma BJ, Silversides CK, Oechslin EN, Hoke U, van Erven L, Budts W, Van Gelder IC, Mulder BJ, Harris L.

Europace. 2017 Jan 20. pii: euw386. doi: 10.1093/europace/euw386. [Epub ahead of print]

2. Utility and safety of the SafeSept™ transseptal guidewire for electrophysiology studies with catheter ablation in pediatric and congenital heart disease.

Knadler JJ, Anderson JB, Chaouki AS, Czosek RJ, Connor C, Knilans TK, Spar DS.

J Interv Card Electrophysiol. 2017 Jan 14. doi: 10.1007/s10840-017-0224-z. [Epub ahead of print]

3. The Role of Conventional and Right-Sided ECG Screening for Subcutaneous ICD in a Tetralogy of Fallot Population.

Alonso P, Osca J, Cano O, Pimenta P, Andrés A, Yagüe J, Millet J, Rueda J, Sancho-Tello MJ.

Pacing Clin Electrophysiol. 2017 Jan 10. doi: 10.1111/pace.13017. [Epub ahead of print]

4. Electrocardiographic RR and QT Interval Variability in Patients with Atrial Septal Defect and Healthy Children.

Eryu Y, Hata T, Nagatani A, Funamoto Y, Uchida H, Fujino M, Boda H, Miyata M, Yoshikawa T.

Pediatr Cardiol. 2017 Jan 5. doi: 10.1007/s00246-016-1551-z. [Epub ahead of print]

5. Relation of Left Atrial Size to Atrial Fibrillation in Patients Aged ≤22 Years.

Mah DY, Shakti D, Gauvreau K, Colan SD, Alexander ME, Abrams DJ, Brown DW.

Am J Cardiol. 2017 Jan 1;119(1):52-56. doi: 10.1016/j.amjcard.2016.09.008.

6. Usefulness of Fragmented QRS Complexes in Patients With Congenital Heart Disease to Predict Ventricular Tachyarrhythmias.

Vogels RJ, Teuwen CP, Ramdjan TT, Evertz R, Knops P, Witsenburg M, Roos-Hesselink JW, Bogers AJ, de Groot NM.

Am J Cardiol. 2017 Jan 1;119(1):126-131. doi: 10.1016/j.amjcard.2016.09.021.

7. Inverse problems in reduced order models of cardiovascular haemodynamics: aspects of data assimilation and heart rate variability.

Pant S, Corsini C, Baker C, Hsia TY, Pennati G, Vignon-Clementel IE.

J R Soc Interface. 2017 Jan;14(126). pii: 20160513. doi: 10.1098/rsif.2016.0513.

8. Sinoatrial node cardiomyocytes derived from human pluripotent cells function as a biological pacemaker.

Protze SI, Liu J, Nussinovitch U, Ohana L, Backx PH, Gepstein L, Keller GM.

Nat Biotechnol. 2017 Jan;35(1):56-68. doi: 10.1038/nbt.3745.

9. Dexmedetomidine use in patients undergoing electrophysiological study for supraventricular tachyarrhythmias.

Tirotta CF, Nguyen T, Fishberger S, Velis E, Olen M, Lam L, Madril DR, Hughes J, Lagueruela RG.

Paediatr Anaesth. 2017 Jan;27(1):45-51. doi: 10.1111/pan.13019.

10. Molecular Pathophysiology of Congenital Long QT Syndrome.

Bohnen MS, Peng G, Robey SH, Terrenoire C, Iyer V, Sampson KJ, Kass RS.

Physiol Rev. 2017 Jan;97(1):89-134. Review.

11. Overlap of Arrhythmogenic Cardiomyopathy, Spongiform Cardiomyopathy, and Congenital Heart Disease.

Bermúdez-Jiménez FJ, Jiménez-Jáimez J, López-Fernández S.

Rev Esp Cardiol (Engl Ed). 2017 Jan;70(1):51. doi: 10.1016/j.rec.2016.02.018. English, Spanish. No abstract available.

12. [A rare cause of 2:1 atrioventricular block and congestive heart failure in preterm infants: Hypocalcemia].

Azak E, Tatar Aksoy H, Ünsal H, Çetin İİ.

Turk Kardiyol Dern Ars. 2017 Jan;45(1):82-84. doi: 10.5543/tkda.2016.82453. Turkish.


13. Thyroid hormone intoxication as a not yet described cause of J-wave syndrome in a pediatric patient.

Flores-González JC, Grujic B, Lechuga-Sancho AM.

Endocrine. 2017 Jan 25. doi: 10.1007/s12020-017-1228-2. [Epub ahead of print] No abstract available.

14. Flecainide as first-line treatment for fetal supraventricular tachycardia.

Ekiz A, Kaya B, Bornaun H, Acar DK, Avci ME, Bestel A, Yildirim G.

J Matern Fetal Neonatal Med. 2017 Jan 23:1-15. doi: 10.1080/14767058.2017.1286317. [Epub ahead of print]

15. Junctional Ectopic Tachycardia Localization and Procedural Approach using Cryoablation.

Pierick AR, Law IH, Muldonado JR, VON Bergen NH.

Pacing Clin Electrophysiol. 2017 Jan 18. doi: 10.1111/pace.13022. [Epub ahead of print]

16. PocketECG: A New Noninvasive Method for Continuous and Real-Time ECG Monitoring-Initial Results in Children and Adolescents.

Bieganowska K, Kaszuba A, Bieganowski M, Kaczmarek K.

Pediatr Cardiol. 2017 Jan 18. doi: 10.1007/s00246-016-1534-0. [Epub ahead of print]

17. Sick Sinus Syndrome with HCN4 Mutations Shows Early Onset and Frequent Association with Atrial Fibrillation and Left Ventricular Non-compaction.

Ishikawa T, Ohno S, Murakami T, Yoshida K, Mishima H, Fukuoka T, Kimoto H, Sakamoto R, Ohkusa T, Aiba T, Nogami A, Sumitomo N, Shimizu W, Yoshiura KI, Horigome H, Horie M, Makita N.

Heart Rhythm. 2017 Jan 16. pii: S1547-5271(17)30067-X. doi: 10.1016/j.hrthm.2017.01.020. [Epub ahead of print]

18. Supraventricular tachycardia during the first year of life: is subclinical inflammation the trigger?

Bassareo PP, Fanos V, Pala M, Antonucci L, Neroni P, Antonucci R, Mercuro G.

J Matern Fetal Neonatal Med. 2017 Jan 12:1-9. doi: 10.1080/14767058.2016.1275545. [Epub ahead of print]

19. Effects of Triple Cryoenergy Application on Lesion Formation and Coronary Arteries in the Developing Myocardium.

Krause U, Abreu da Cunha FD, Backhoff D, Jacobshagen C, Klehs S, Schneider HE, Paul T.

Pediatr Cardiol. 2017 Jan 11. doi: 10.1007/s00246-016-1564-7. [Epub ahead of print]

20. Study design for control of HEART rate in inFant and child tachyarrhythmia with heart failure Using Landiolol (HEARTFUL): A prospective, multicenter, uncontrolled clinical trial.

Sumitomo N, Horigome H, Miura M, Ono H, Ueda H, Takigiku K, Yoshimoto J, Ohashi N, Suzuki T, Sagawa K, Ushinohama H, Takahashi K, Miyazaki A, Sakaguchi H, Iwamoto M, Takamuro M, Tokunaga C, Nagano T; Heartful Investigators..

J Cardiol. 2017 Jan 7. pii: S0914-5087(16)30343-4. doi: 10.1016/j.jjcc.2016.12.002. [Epub ahead of print]

21. A Precision Medicine Approach to the Rescue of Function on Malignant Calmodulinopathic Long-QT Syndrome.

Limpitikul WB, Dick IE, Tester DJ, Boczek NJ, Limphong P, Yang W, Choi MH, Babich J, DiSilvestre D, Kanter RJ, Tomaselli GF, Ackerman MJ, Yue DT.

Circ Res. 2017 Jan 6;120(1):39-48. doi: 10.1161/CIRCRESAHA.116.309283.

22. Looking forward: PACES Task Force on prevention of sudden cardiac death in the young: a comprehensive approach to a preventable problem.

Erickson CC, Salerno JC.

Cardiol Young. 2017 Jan;27(S1):S147-S148. doi: 10.1017/S1047951116002407. No abstract available.

23. The role of illicit drug use in sudden death in the young.

Fischbach P.

Cardiol Young. 2017 Jan;27(S1):S75-S79. doi: 10.1017/S1047951116002274.

24. Wolff-Parkinson-White syndrome: lessons learnt and lessons remaining.

Benson DW, Cohen MI.

Cardiol Young. 2017 Jan;27(S1):S62-S67. doi: 10.1017/S1047951116002250.

25. Sports participation in long QT syndrome.

Aziz PF, Saarel EV.

Cardiol Young. 2017 Jan;27(S1):S43-S48. doi: 10.1017/S1047951116002225.

26. Evaluating the survivor or the relatives of those who do not survive: the role of genetic testing.

Tester DJ, Ackerman MJ.

Cardiol Young. 2017 Jan;27(S1):S19-S24. doi: 10.1017/S1047951116002183.

27. Discrimination of the “Athlete’s Heart” from real disease by electrocardiogram and echocardiogram.

Erickson CC.

Cardiol Young. 2017 Jan;27(S1):S80-S88. doi: 10.1017/S1047951116002286.

28. Arrhythmogenic right ventricular dysplasia/cardiomyopathy.

Orgeron GM, Crosson JE.

Cardiol Young. 2017 Jan;27(S1):S57-S61. doi: 10.1017/S1047951116002249.

29. Fascicular Ventricular Arrhythmias: Pathophysiologic Mechanisms, Anatomical Constructs, and Advances in Approaches to Management.

Kapa S, Gaba P, DeSimone CV, Asirvatham SJ.

Circ Arrhythm Electrophysiol. 2017 Jan;10(1). pii: e002476. doi: 10.1161/CIRCEP.116.002476. No abstract available.

30. Genetic Insurance Discrimination in Sudden Arrhythmia Death Syndromes: Empirical Evidence From a Cross-Sectional Survey in North America.

Mohammed S, Lim Z, Dean PH, Potts JE, Tang JN, Etheridge SP, Lara A, Husband P, Sherwin ED, Ackerman MJ, Sanatani S.

Circ Cardiovasc Genet. 2017 Jan;10(1). pii: e001442. doi: 10.1161/CIRCGENETICS.116.001442.

31. Successful radiofrequency catheter ablation for ventricular tachycardia of a 2.9 kg infant with Ebstein’s anomaly.

Takeshita N, Kajiyama Y, Morishita Y, Itoi T, Yamagishi M, Suzuki T.

Europace. 2017 Jan;19(1):131. doi: 10.1093/europace/euw172. No abstract available.

32. A novel variant in RyR2 causes familiar catecholaminergic polymorphic ventricular tachycardia.

Bosch C, Campuzano O, Sarquella-Brugada G, Cesar S, Perez-Serra A, Coll M, Mademont I, Mates J, Del Olmo B, Iglesias A, Brugada J, Petersen V, Brugada R.

Forensic Sci Int. 2017 Jan;270:173-177. doi: 10.1016/j.forsciint.2016.12.001.

33. A type 2 ryanodine receptor variant associated with reduced Ca2+ release and short-coupled torsades de pointes ventricular arrhythmia.

Fujii Y, Itoh H, Ohno S, Murayama T, Kurebayashi N, Aoki H, Blancard M, Nakagawa Y, Yamamoto S, Matsui Y, Ichikawa M, Sonoda K, Ozawa T, Ohkubo K, Watanabe I, Guicheney P, Horie M.

Heart Rhythm. 2017 Jan;14(1):98-107. doi: 10.1016/j.hrthm.2016.10.015.

34. Atrioventricular Nodal Reentrant Tachycardia With a Displaced His-Bundle in an Atrioventricular Canal Defect.

Yamada T, Lau YR, Kay GN.

J Cardiovasc Electrophysiol. 2017 Jan;28(1):120-121. doi: 10.1111/jce.13099. No abstract available.

35. Heart Rate Variability as a Valuable and Easy Method for the Evaluation of Cardiac Autonomic Function.

Gulgun M.

Med Princ Pract. 2017;26(1):100. doi: 10.1159/000452628. No abstract available.

36. Heart Rate Variability, Heart Rate Recovery, and Heart Rate Turbulence Represent Different Components of the Cardiac Autonomic Nervous System.

Gulgun M.

Med Princ Pract. 2017;26(1):98-99. doi: 10.1159/000452335. No abstract available.

37. Ten-Year Clinical Experience with the Lumenless, Catheter-Delivered, 4.1-Fr Diameter Pacing Lead in Patients with and without Congenital Heart.

Bansal N, Samuel S, Zelin K, Karpawich PP.

Pacing Clin Electrophysiol. 2017 Jan;40(1):17-25. doi: 10.1111/pace.12995.

38. Emergency Pacing via the Umbilical Vein and Subsequent Permanent Pacemaker Implantation in a Neonate.

Li XM, Zhang DY, Li HY, Wang JY, Jiang H, Jia GZ, Wu H, Ge HY.

Pediatr Cardiol. 2017 Jan;38(1):199-201. doi: 10.1007/s00246-016-1524-2.

39. Diagnostic Yield of Outpatient Pediatric Echocardiograms: Impact of Indications and Specialty.

Lang SM, Bolin E, Hardy S, Tang X, Collins RT 2nd.

Pediatr Cardiol. 2017 Jan;38(1):162-169. doi: 10.1007/s00246-016-1497-1.

40. High g-Force Rollercoaster Rides Induce Sinus Tachycardia but No Cardiac Arrhythmias in Healthy Children.

Pieles GE, Husk V, Blackwell T, Wilson D, Collin SM, Williams CA, Stuart AG.

Pediatr Cardiol. 2017 Jan;38(1):15-19. doi: 10.1007/s00246-016-1477-5.

41. A Novel Electrocardiogram Algorithm Utilizing ST-Segment Instability for Detection of Cardiopulmonary Arrest in Single Ventricle Physiology: A Retrospective Study.

Vu EL, Rusin CG, Penny DJ, Kibler KK, Easley RB, Smith B, Andropoulos D, Brady K.

Pediatr Crit Care Med. 2017 Jan;18(1):44-53. doi: 10.1097/PCC.0000000000000980.

42. ECGs in the ED.

Tanel RE.

Pediatr Emerg Care. 2017 Jan;33(1):70-71. doi: 10.1097/PEC.0000000000001016. No abstract available.

43. Aneurysm of the muscular septum associated with Wolf-Parkinson-White syndrome presenting as dilated cardiomyopathy; A report of two cases.

Doğan V, Ertuğrul İ, Kayalı Ş, Örün UA, Karademir S.

Turk Kardiyol Dern Ars. 2017 Jan;45(1):85-88. doi: 10.5543/tkda.2016.44038.

44. Para-Hisian Pacing: A Paradoxical Response?

Kumar DS, Zarraga IG, Raitt MH, Balaji S.

J Cardiovasc Electrophysiol. 2017 Jan;28(1):124-125. doi: 10.1111/jce.13106. No abstract available.


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ACHD Featured Articles of January 2017


Incidence, risk factors, and predictors of infective endocarditis in adult congenital heart disease: focus on the use of prosthetic material.

Kuijpers JM, Koolbergen DR, Groenink M, Peels KC, Reichert CL, Post MC, Bosker HA, Wajon EM, Zwinderman AH, Mulder BJ, Bouma BJ.

Eur Heart J. 2017 Jan 8. pii: ehw591. doi: 10.1093/eurheartj/ehw591. [Epub ahead of print]

PMID: 28065906

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Take Home Points

  • The incidence of IE in a contemporary cohort of ACHD patients is 1.33/1000 patient years – this is higher than reported in previous pediatric cohorts
  • The presence of valve-containing prosthetics are associated with a markedly higher rate of IE in the short- (<6 months), intermediate- (6-12 months) and long term (>12 months)
  • The risk of IE in non-valve containing prostheses (including valve repairs) is significant in the first 6 months after implantation only
  • A prediction model for the 5 and 10 year incidence of IE was derived – this requires further validation


Blanche CupidoCommentary from Dr. Blanche J Cupido (Cape Town), section editor of ACHD Journal Watch: Over the last few decades, there has been a steady increase in the number of patients with congenital heart disease [CHD reaching adulthood]. The surgical and interventional pedigree of ACHD patients also has changed substantially as surgical and percutaneous advances have led to greater numbers of prosthetic materials and devices being used. This has therefore lead to an increased number of potential infective endocarditis [IE] “targets” within the cardiovascular system of ACHD patients. All current IE data has largely been based on retrospective, often single center, case series. In the current study, a contemporary population-based cohort (from the CONCOR Registry, Netherlands) is followed prospectively to assess the incidence and determinants of IE with a view to aid the further development IE prevention guidelines.

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The cohort comprised 14224 ACHD patients [>=18 years] with 42.6% of the cohort having multiple defects. There was a prior history of IE in 2.5% comprising an incident rate of 1.33 cases/1000 patient years. At baseline, 12.3% of patients had a valve-containing prosthesis (+/- non-valve containing prostheses), 36.8% had only non-valve containing prostheses and 50.9% had no prosthetic material at all.

 Time related risk factors on Cox Regression analysis included the following risk factors: presence of valve-containing prostheses [HR 5.5], multiple defects [1.5], the presence of pulmonary atresia with VSD [2.6], a prior history of IE [HR 1.9] and male gender [HR1.8]. Valve containing prostheses remained the strongest independent risk factor for infective endocarditis (HR 5.48) on, and the focus of infection was located on the prosthesis in the vast majority of cases (81%).

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The temporal impact of valve containing and non-valve containing prostheses is summarized in Table 3. For valve containing prostheses the IE risk did not abate beyond 12 months whereas for non-valve containing prostheses the IE risk abated beyond 6 months.


Utilizing these findings, the authors developed a risk prediction model (Table 4) that showed a good correlation between the predicted and observed IE rates over both a 5- and 10-year period. 

This model would however require further validation before its implementation for clinical use.

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The authors concluded that the presence of valve-containing prosthetics remain a major risk for the development of IE in both the short and the long term and, as per major societal guidelines, require lifelong antibiotic prophylaxis for dental procedures. Both the American and European guidelines currently categorize valve repair and valve containing prosthesis together but this paper speaks to the short-term IE risk in those with valve repairs only, thus negating the need for antibiotic prophylaxis in the longer term.



Three decades later: The fate of the population of patients who underwent the Atriopulmonary Fontan procedure.

Poh CL, Zannino D, Weintraub RG, Winlaw DS, Grigg LE, Cordina R, Hornung T, Bullock A, Justo RN, Gentles TL, Verrall C, du Plessis K, Celermajer DS, d’Udekem Y.

Int J Cardiol. 2017 Jan 7. pii: S0167-5273(16)33033-9. doi: 10.1016/j.ijcard.2017.01.057. [Epub ahead of print]

PMID: 28100430

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Take Home Points

  • Two thirds of patients who went classic atrio-pulmonary Fontan are still alive 3 decades later: At 28 year follow-up, 69% of patients were alive, 64% had freedom from death or transplantation, and 45% were free from Fontan failure.
  • Atrial arrhythmias were associated with increased risk of death, cardiac transplantation, and Fontan failure (defined as death, transplantation, takedown, conversion, protein losing enteropathy, plastic bronchitis, severe ventricular dysfunction (ejection fraction < 30%), or NYHA class III–IV).
  • Almost 80% of patients had atrial arrhythmias, which was strongly associated with right atrial dilation.
  • A small subset of patients had normal atrial size. These patients had a much lower risk of arrhythmia and better survival.
  • Follow-up by a cardiologist with congenital heart disease expertise was associated with better outcomes when compared with patients who are followed by general cardiologists without such expertise.
  • At the time of follow-up, there were 59% of patients who were still alive with intact atriopulmonary Fontan circulation. The median age of these patients was 33.6 years with a range of 16-68 years of age. Of these surviving patients with intact atriopulmonary Fontan, two thirds remain physically active with most of them currently employed.

jokhadar-maanCommentary from Dr. Maan Jokhadar (Atlanta), section editor of ACHD Journal Watch:
The atriopulmonary connection is an abandoned form of the Fontan operation that involves a direct connection between the right atrium and pulmonary artery, though many variations exist.  This operation is associated with progressive right atrial dilation and fibrosis, which is associated with a number of complications that include arrhythmias, thromboembolic events, pulmonary vein compression, and Fontan failure.  The atriopulmonary Fontan has largely been replaced by total cavopulmonary connection Fontan in the current epoch.  There still remain a significant number of patients who are still alive after atriopulmonary connection and this study describes long-term morbidity and mortality in these patients.

Using the Australia and New Zealand Fontan Registry, Dr. Poh and colleagues performed a retrospective analysis of 215 patients who survived atriopulmonary Fontan operations between 1975 and 1994. During this time, 238 patients underwent atriopulmonary Fontan.  Of these, 22 patients died and 4 patients had Fontan takedown, leaving 215 patients who survived until hospital discharge.

The median age for patients who underwent atriopulmonary Fontan completion was 5.6 but there were a few adults, including a 40-year-old.  Approximately 19% had a dominant right ventricle, 91% had Blalock-Taussig shunt, and 22% had pulmonary artery banding.

The median follow-up was 26 years post Fontan completion.  There were 52 deaths from various causes that include Fontan failure, perioperative heart failure, sudden cardiac death, pulmonary embolus, stroke, and there was 1 patient who had a post-transplant rejection. During this period of follow-up, there were 12 cardiac transplantations, 3 Fontan takedowns, 39 Fontan conversions, 7 patients with protein losing enteropathy, 16 patients with pulmonary embolus, 19 patients with embolic stroke, 12 with right atrial thrombus, and 2 with splenic infarcts. Approximately 74% of patients were under the care of a cardiologist with congenital heart disease expertise.  The remaining patients were followed by cardiologists without such expertise.  The risk of Fontan failure was nearly double (HR 1.94) in the patients who were not followed by a cardiologist with congenital heart disease expertise.

Approximately 18% of patients underwent Fontan conversion, which may confer a survival advantage in patients with atrial arrhythmias but this is not clearly established.

Factors associated with long-term mortality after Fontan procedures: a systematic review.

Alsaied T, Bokma JP, Engel ME, Kuijpers JM, Hanke SP, Zuhlke L, Zhang B, Veldtman GR.

Heart. 2017 Jan 15;103(2):104-110. doi: 10.1136/heartjnl-2016-310108. Review.

PMID: 28057809

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Select item 27798056


Take Home Points:

  • Risk of late mortality in this systematic review was found to be 2.1% per year, although this was across several surgical eras.
  • Heart failure (including Fontan failure) was the most common cause of mortality, followed closely by sudden death/arrhythmia.
  • Other common causes of mortality included respiratory failure, renal disease, thrombosis/bleeding, and infection.
  • Thromboembolic events account for up to 25% of late mortality in some studies
  • Noninvasive cardiac imaging factors to predict mortality need further development; factors that may correlate with risk of death include lower ejection fraction, global circumferential strain, and a ventricular end-diastolic volume index > 125 mL/m2
  • Peak VO2 has been shown to independently predict mortality in the Fontan population, but other CPEX parameters have not been associated with increased mortality.
  • Limited heart rate reserve on stress testing appears to be an emerging risk factor.


Kay_William_MD.16.CVaCommentary from Dr. W. Aaron Kay (Indianapolis), section editor of ACHD Journal Watch:  This paper is a systemic review of 28 papers evaluating factors associated with late mortality after Fontan procedure.  Inclusion criteria were having > 90 patients studied or > 20 mortalities and/or transplants.  A total of 6707 patients with an average follow-up time of 8.23 + 5.42 years was identified.  There were 1000 late deaths, with the cause of death reported in 697 cases.  The most common causes of death were heart/Fontan failure (22%), arrhythmia (16%), and respiratory failure (15%).  Renal failure, thrombosis/bleeding, infection, PLE, liver disease, and malignancy were also reported as causes of death.

            A total of 35 risk factors for late mortality were identified.  The authors divided these into 9 broad categories including: era of surgery, preoperative, operative, postoperative, cardiovascular complications, non-invasive cardiac imaging variables, exercise stress testing variables, non-cardiac complications (protein-losing enteropathy, plastic bronchitis, Fontan-associated liver disease, and high altitude exposure), and serological factors (BNP and sodium levels).

            There is clearly an era effect on mortality risk.  Older style Fontans (atriopulmonary or AV connection) clearly have worse outcomes than the newer lateral tunnel or extracardiac Fontans.  Much of this was driven by a very high incidence of atrial arrhythmia.  Fontan procedures are generally done at much earlier ages now; a large study just published in 2015 reviewing 1052 patients showed that optimal late survival was observed in patients having Fontan surgery between 2 and 4 years of age, however this signal was only found on univariate, rather than multivariate analysis.

Several preoperative variables have been found to have an association with late mortality, including AV valve regurgitation, hypoplastic left ventricle, heterotaxy, lack of sinus rhythm, and preoperative mean pulmonary artery pressure > 15 mmHg, all of which were risk factors for late mortality.

In this study, although protein losing enteropathy, liver failure, and malignancy were the cause of death in some patients, mortality was more commonly due to other factors.  It is uncertain, for example, how many cases of PLE may have been misclassified as “Fontan failure,” and vice versa.

Very few papers have been written evaluating novel risk factors, such as specific noninvasive measurements on echo or MRI, or serologic factors.  BNP and sodium levels have been studied.  There is still a paucity of research evaluating risk factors for poor hepatic outcomes.

Much of the data in this study was obtained from the elegant Australia/New Zealand Fontan registry.  Further research will be greatly benefited by additional registries to track outcomes.

 achd 4

Figure 2 (reproduced for improved legibility):

Causes of late mortality after Fontan procedure.


Advance Care Planning in Adults with Congenital Heart Disease: A Patient Priority.

Deng LX, Gleason LP, Khan AM, Drajpuch D, Fuller S, Goldberg LA, Mascio CE, Partington SL, Tobin L, Kim YY, Kovacs AH.

Int J Cardiol. 2017 Jan 4. pii: S0167-5273(16)32556-6. doi: 10.1016/j.ijcard.2016.12.185. [Epub ahead of print]

PMID: 28096041

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Take Home Points:

  • Adult patients with Congenital Heart Disease want their providers to talk them about advanced care planning
  • Advanced care planning is especially important in those with moderate or complex heart lesions who continue to have substantial morbidity and mortality.
  • Such discussion is preferably made when patients attain the age of 18 years and before significant morbidity sets in.
  • Patients especially those of female gender, high anxiety levels and with simple cardiac lesions are more likely to need advanced care planning.


Leong Ming ChernCommentary from Dr. M.C. Leong (Malaysia), section editor of ACHD Journal Watch:  Advance care planning (ACP) is the process of planning for future medical care and typically includes the completion of advance directives and the appointment of a health care power of attorney, before patients reach the terminal stage of their disease. Prior Canadian studies demonstrated the usefulness of an ACP in the care of ACHD patients (1-2). This study was conducted in a north eastern American centre and aims to describe patients’ experience and preferences with respect to ACP.


152 ACHD patients participated and completed questions regarding ACP [including questions such as “had they discussed ACP with a health care provider, whether they had completed an ACD, how important discussing ACP was to them, and when the right age and time was to do so], completed the Hospital Anxiety and Depression Scale as the investigators graded severity of the patients heart disease by hospital record review.

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Though most patients thought discussing ACP with their healthcare provider was important, only 13% could actually recall having had such discussions in the past. In fact 70% have had some sort of end of life discussions with other people including spouses [40%] and friends and family [51%]. Patients preferred an age of 18 years and older for such discussions. Most patients (78%) preferred ACP discussions to be initiated while healthy compared to 31% who did not. Patients of female gender, who had lower disease complexity and had elevated anxiety symptoms, are more likely to appreciate ACP. Twenty one % of patients had actually completed an advanced care directive and 18% reported that they had a power of attorney. These results suggest that many sought to obtain this outside the health care environment.

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Ninety-three patients (61%) reported that they wanted information about the estimated life expectancy of patients with their heart condition. Although patients with more complex disease have the lower life expectancy, patients with lower disease complexity and those with a history of 2 or more cardiac surgeries are the ones most  interested in life expectancy. The percentage of patients who thought they had a shorter life expectancy did not differ by complexity of the heart lesions (simple =63%; moderate =65%; great=66%; p=0.96) or education level (less than college=56%; college or above =67%, p=0.30).


Unlike in Canada, in American institutions (1) ACP discussions rarely occur (2) patients with greater defect complexity deemed ACP discussion less important and are more ignorant to their life expectancy (3) anxious patients are more likely to be interested in ACP discussion and life expectancy.

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  1. Tobler, M. Greutmann, J.M. Colman, M. Greutmann-Yantiri, S.L. Librach, A.H. Kovacs, Knowledge of and preference for advance care planning by adults with congenital heart disease, Am. J. Cardiol. 109 (12) (2012) 1797–1800.
  2. Tobler, M. Greutmann, J.M. Colman, M. Greutmann-Yantiri, L.S. Librach, A.H. Kovacs, End-of-life in adults with congenital heart disease: a call for early communication, Int. J. Cardiol. 155 (3) (22 2012) 383–387.


Prevalence and outcome of thrombotic and embolic complications in adults after Fontan operation.

Egbe AC, Connolly HM, Niaz T, Yogeswaran V, Taggart NW, Qureshi MY, Poterucha JT, Khan AR, Driscoll DJ.

Am Heart J. 2017 Jan;183:10-17. doi: 10.1016/j.ahj.2016.09.014.

PMID: 27979032

Similar articles


Take Home Points:

  • Patients with atriopulmonary Fontan are at high risk for thromboembolic complications
  • Patients with Fontan physiology and atrial arrhythmias are also at increased risk for the development of thromboembolic complications.
  • Patients who discontinue vitamin K antagonism for the therapeutic intervention of documented thrombus secondary to a bleeding complication are likely to have a recurrence.


Moe_Tabitha-WEBCommentary from Dr. Tabitha Moe (Phoenix), section editor of ACHD Journal Watch:   There are limited studies in patients with Fontan physiology to understand the burden of thrombotic and embolic complications.  This nice study from the group at Mayo Rochester evaluates a fairly large cohort of 387 adult patients (pediatric patients were excluded) and found that patients with an atriopulmonary type Fontan and those with documented atrial arrhythmias are at the highest risk for thromboembolic complications. They report a 25% risk of thromboembolic complication which is in line with previously reported risk ranges of 5-33%.  They note that patients are effectively treated with warfarin alone, but do not comment on the use of novel oral anticoagulants in this cohort.  It is also important to note that they defined the risk of repeat thromboembolic complications directly correlates with the need to discontinue oral vitamin K antagonism for bleeding complications. However, bleeding events were common, occurring in 30% of the study cohort. Additionally concerning, those patients who experienced a thromboembolic complication were more likely to go on and develop heart failure requiring hospitalization, and decompensation necessitating evaluation for transplant. Previously reported cohorts of thromboembolic complications included significant numbers of children, which decreases then the age of participants in the reports which may account for the seeming increase in this study. Because of the high risk of recurrence of thrombus when VKA is discontinued it may be reasonable to pursue a hematologic evaluation prior to discontinuing VKA in patients with bleeding issues, as these patients are also the most likely to have derangements in their cascade.

ACHD Jan 2017

1. Surveillance for liver complications after the Fontan procedure.

Hilscher MB, Johnson JN, Cetta F, Driscoll DJ, Poterucha JJ, Sanchez W, Connolly HM, Kamath PS.

Congenit Heart Dis. 2017 Jan 31. doi: 10.1111/chd.12446. [Epub ahead of print] Review.

2. Obstetric and perinatal outcome in anti-Ro/SSA-positive pregnant women: a prospective cohort study.

Martínez-Sánchez N, Pérez-Pinto S, Robles-Marhuenda Á, Arnalich-Fernández F, Martín Cameán M, Hueso Zalvide E, Bartha JL.

Immunol Res. 2017 Jan 30. doi: 10.1007/s12026-016-8888-5. [Epub ahead of print]

3. How best to train doctors in adult congenital heart disease?

Acar P.

Arch Cardiovasc Dis. 2017 Jan 27. pii: S1875-2136(16)30205-4. doi: 10.1016/j.acvd.2016.11.001. [Epub ahead of print] No abstract available.

4. Corrigendum to ‘Ventricular tachyarrhythmia during pregnancy in women with heart disease: Data from the ROPAC, a registry from the European Society of Cardiology’ [Int. J. Cardiol. 220 (2016) 131-136].

Ertekin E, van Hagen IM, Salam AM, Ruys TP, Johnson MR, Popelova J, Parsonage WA, Ashour Z, Shotan A, Oliver JM, Veldtman GR, Hall R, Roos-Hesselink JW.

Int J Cardiol. 2017 Jan 27. pii: S0167-5273(17)30190-0. doi: 10.1016/j.ijcard.2017.01.077. [Epub ahead of print] No abstract available.

5. Abnormal Wave Reflections and Left Ventricular Hypertrophy Late After Coarctation of the Aorta Repair.

Quail MA, Short R, Pandya B, Steeden JA, Khushnood A, Taylor AM, Segers P, Muthurangu V.

Hypertension. 2017 Jan 23. pii: HYPERTENSIONAHA.116.08763. doi: 10.1161/HYPERTENSIONAHA.116.08763. [Epub ahead of print]

6. Characteristics, aetiological spectrum and management of valvular heart disease in a Tunisian cardiovascular centre.

Triki F, Jdidi J, Abid D, Tabbabi N, Charfeddine S, Ben Kahla S, Hentati M, Abid L, Kammoun S.

Arch Cardiovasc Dis. 2017 Jan 20. pii: S1875-2136(16)30190-5. doi: 10.1016/j.acvd.2016.08.003. [Epub ahead of print]

7. Determinants of Aortic Size and Stiffness and the Impact on Exercise Physiology in Patients After the Fontan Operation.

Ohuchi H, Hayama Y, Negishi J, Noritake K, Miyazaki A, Yamada O, Shiraishi I.

Int Heart J. 2017 Jan 20. doi: 10.1536/ihj.16-183. [Epub ahead of print]

8. Multiple pregnancy in a primigravida with uncorrected Pentalogy of Fallot.

Partana P, Tan JK, Tan JL, Tan LK.

BMJ Case Rep. 2017 Jan 18;2017. pii: bcr2016216809. doi: 10.1136/bcr-2016-216809.

9. Outcomes and trends of peripartum maternal admission to the intensive care unit.

Farr A, Lenz-Gebhart A, Einig S, Ortner C, Holzer I, Elhenicky M, Husslein PW, Lehner R.

Wien Klin Wochenschr. 2017 Jan 18. doi: 10.1007/s00508-016-1161-z. [Epub ahead of print]

10. Prognostic Value of N-Terminal Pro-B-Type Natriuretic Peptide, Troponin-T, and Growth-Differentiation Factor 15 in Adult Congenital Heart Disease.

Baggen VJ, van den Bosch AE, Eindhoven JA, Schut AW, Cuypers JA, Witsenburg M, de Waart M, van Schaik RH, Zijlstra F, Boersma E, Roos-Hesselink JW.

Circulation. 2017 Jan 17;135(3):264-279. doi: 10.1161/CIRCULATIONAHA.116.023255.

11. Residents’ understanding of adult congenital heart disease.

Cooper P, Hindes M, Maul TM, Cook SC.

Congenit Heart Dis. 2017 Jan 16. doi: 10.1111/chd.12441. [Epub ahead of print]

12. Factors associated with long-term mortality after Fontan procedures: a systematic review.

Alsaied T, Bokma JP, Engel ME, Kuijpers JM, Hanke SP, Zuhlke L, Zhang B, Veldtman GR.

Heart. 2017 Jan 15;103(2):104-110. doi: 10.1136/heartjnl-2016-310108. Review.

13. From bosentan to macitentan for pulmonary arterial hypertension and adult congenital heart disease: Further improvement?

Blok IM, van Riel AC, van Dijk AP, Mulder BJ, Bouma BJ.

Int J Cardiol. 2017 Jan 15;227:51-52. doi: 10.1016/j.ijcard.2016.11.211. No abstract available.

14. Usefulness of stroke volume monitoring during upright ramp incremental cycle exercise in young patients with Fontan circulation.

Legendre A, Guillot A, Ladouceur M, Bonnet D.

Int J Cardiol. 2017 Jan 15;227:625-630. doi: 10.1016/j.ijcard.2016.10.087.

15. Clinical recommendations of cardiac magnetic resonance, Part II: inflammatory and congenital heart disease, cardiomyopathies and cardiac tumors: a position paper of the working group ‘Applicazioni della Risonanza Magnetica’ of the Italian Society of Cardiology.

Pontone G, Di Bella G, Silvia C, Maestrini V, Festa P, Ait-Ali L, Masci PG, Monti L, di Giovine G, De Lazzari M, Cipriani A, Guaricci AI, Dellegrottaglie S, Pepe A, Marra MP, Aquaro GD.

J Cardiovasc Med (Hagerstown). 2017 Jan 13. doi: 10.2459/JCM.0000000000000499. [Epub ahead of print]

16. Prevalence of Neuroendocrine Tumors in Patients With Cyanotic Congenital Heart Disease.

Ponz de Antonio I, Ruiz Cantador J, González García AE, Oliver Ruiz JM, Sánchez-Recalde Á, López-Sendón JL.

Rev Esp Cardiol (Engl Ed). 2017 Jan 13. pii: S1885-5857(16)30421-2. doi: 10.1016/j.rec.2016.09.036. [Epub ahead of print] English, Spanish. No abstract available.

17. Management of Pregnancy in Patients With Complex Congenital Heart Disease: A Scientific Statement for Healthcare Professionals From the American Heart Association.

Canobbio MM, Warnes CA, Aboulhosn J, Connolly HM, Khanna A, Koos BJ, Mital S, Rose C, Silversides C, Stout K; American Heart Association Council on Cardiovascular and Stroke Nursing; Council on Clinical Cardiology; Council on Cardiovascular Disease in the Young; Council on Functional Genomics and Translational Biology; and Council on Quality of Care and Outcomes Research..

Circulation. 2017 Jan 12. pii: CIR.0000000000000458. doi: 10.1161/CIR.0000000000000458. [Epub ahead of print]

18. Individual risk stratification in adult congenital heart disease: the way to go?

Budts W.

Eur Heart J. 2017 Jan 10. pii: ehw641. doi: 10.1093/eurheartj/ehw641. [Epub ahead of print] No abstract available.

19. Risk factors for excess mortality in adults with congenital heart diseases.

Oliver JM, Gallego P, Gonzalez AE, Garcia-Hamilton D, Avila P, Yotti R, Ferreira I, Fernandez-Aviles F.

Eur Heart J. 2017 Jan 10. pii: ehw590. doi: 10.1093/eurheartj/ehw590. [Epub ahead of print]

20. Incidence, risk factors, and predictors of infective endocarditis in adult congenital heart disease: focus on the use of prosthetic material.

Kuijpers JM, Koolbergen DR, Groenink M, Peels KC, Reichert CL, Post MC, Bosker HA, Wajon EM, Zwinderman AH, Mulder BJ, Bouma BJ.

Eur Heart J. 2017 Jan 8. pii: ehw591. doi: 10.1093/eurheartj/ehw591. [Epub ahead of print]

21. Three decades later: The fate of the population of patients who underwent the Atriopulmonary Fontan procedure.

Poh CL, Zannino D, Weintraub RG, Winlaw DS, Grigg LE, Cordina R, Hornung T, Bullock A, Justo RN, Gentles TL, Verrall C, du Plessis K, Celermajer DS, d’Udekem Y.

Int J Cardiol. 2017 Jan 7. pii: S0167-5273(16)33033-9. doi: 10.1016/j.ijcard.2017.01.057. [Epub ahead of print]

22. Acquired coronary artery disease in adult patients with congenital heart disease: a true or a false problem?

Giamberti A, Lo Rito M, Conforti E, Varrica A, Carminati M, Frigiola A, Menicanti L, Chessa M.

J Cardiovasc Med (Hagerstown). 2017 Jan 7. doi: 10.2459/JCM.0000000000000495. [Epub ahead of print]

23. Effects of acute oxygen supplementation on functional capacity and heart rate recovery in Eisenmenger syndrome.

Gonzaga LR, Matos-Garcia BC, Rocco IS, Begot I, Bolzan DW, Tatani SB, Santos VB, Silva CM, Carvalho AC, Arena R, Gomes WJ, Guizilini S.

Int J Cardiol. 2017 Jan 6. pii: S0167-5273(16)32465-2. doi: 10.1016/j.ijcard.2017.01.025. [Epub ahead of print]

24. Advance Care Planning in Adults with Congenital Heart Disease: A Patient Priority.

Deng LX, Gleason LP, Khan AM, Drajpuch D, Fuller S, Goldberg LA, Mascio CE, Partington SL, Tobin L, Kim YY, Kovacs AH.

Int J Cardiol. 2017 Jan 4. pii: S0167-5273(16)32556-6. doi: 10.1016/j.ijcard.2016.12.185. [Epub ahead of print]

25. Endothelial microparticles are increased in congenital heart diseases and contribute to endothelial dysfunction.

Lin ZB, Ci HB, Li Y, Cheng TP, Liu DH, Wang YS, Xu J, Yuan HX, Li HM, Chen J, Zhou L, Wang ZP, Zhang X, Ou ZJ, Ou JS.

J Transl Med. 2017 Jan 4;15(1):4. doi: 10.1186/s12967-016-1087-2.


26. Noncompaction cardiomyopathy in Hirschsprung’s disease: a case report.

Visonà SD, Thiene G, Mannarino S, Corana G, Osculati A, Angelini A, Rizzo S.

Cardiovasc Pathol. 2017 Jan 2;27:51-53. doi: 10.1016/j.carpath.2016.12.005. [Epub ahead of print]

27. Thrombosis of a mechanical prosthetic aortic valve in early pregnancy: histopathological findings.

Scheidmann R, Foth R, Sigler M.

Cardiovasc Pathol. 2017 Jan 2;27:35-36. doi: 10.1016/j.carpath.2016.12.003. [Epub ahead of print]

28. Acyanotic congenital heart disease and transesophageal echocardiography.

Sreedhar R.

Ann Card Anaesth. 2017 Jan;20(Supplement):S36-S42. doi: 10.4103/0971-9784.197795. Review.

29. Health-related quality of life of young people with long-term illnesses before and after transfer from child to adult healthcare.

While AE, Heery E, Sheehan AM, Coyne I.

Child Care Health Dev. 2017 Jan;43(1):144-151. doi: 10.1111/cch.12410.

30. Cognitive and attentional functioning in adolescents and young adults with Tetralogy of Fallot and d-transposition of the great arteries.

Murphy LK, Compas BE, Reeslund KL, Gindville MC, Mah ML, Markham LW, Jordan LC.

Child Neuropsychol. 2017 Jan;23(1):99-110.

31. Update on the Role of Cardiac Magnetic Resonance Imaging in Congenital Heart Disease.

Rajiah P, Tandon A, Greil GF, Abbara S.

Curr Treat Options Cardiovasc Med. 2017 Jan;19(1):2. doi: 10.1007/s11936-017-0504-z. Review.

32. Resilience and personal growth: A potential resource for therapeutic programmes in people with congenital heart disease.

Dorka R.

Eur J Cardiovasc Nurs. 2017 Jan 1:1474515116687223. doi: 10.1177/1474515116687223. [Epub ahead of print] No abstract available.

33. Brain metabolite alterations in Eisenmenger syndrome: Evaluation with MR proton spectroscopy.

Dokumacı DŞ, Doğan F, Yıldırım A, Boyacı FN, Bozdoğan E, Koca B.

Eur J Radiol. 2017 Jan;86:70-75. doi: 10.1016/j.ejrad.2016.11.005.

34. Assessment of ventriculo-vascular properties in repaired coarctation using cardiac magnetic resonance-derived aortic, left atrial and left ventricular strain.

Shang Q, Sarikouch S, Patel S, Schuster A, Steinmetz M, Ou P, Danford DA, Beerbaum P, Kutty S.

Eur Radiol. 2017 Jan;27(1):167-177.

35. Three-dimensional Echocardiography in Congenital Heart Disease: An Expert Consensus Document from the European Association of Cardiovascular Imaging and the American Society of Echocardiography.

Simpson J, Lopez L, Acar P, Friedberg MK, Khoo NS, Ko HH, Marek J, Marx G, McGhie JS, Meijboom F, Roberson D, Van den Bosch A, Miller O, Shirali G.

J Am Soc Echocardiogr. 2017 Jan;30(1):1-27. doi: 10.1016/j.echo.2016.08.022.

36. Cardiac Adenylyl Cyclase and Phosphodiesterase Expression Profiles Vary by Age, Disease, and Chronic Phosphodiesterase Inhibitor Treatment.

Nakano SJ, Sucharov J, van Dusen R, Cecil M, Nunley K, Wickers S, Karimpur-Fard A, Stauffer BL, Miyamoto SD, Sucharov CC.

J Card Fail. 2017 Jan;23(1):72-80. doi: 10.1016/j.cardfail.2016.07.429.

37. MRI-based computational hemodynamics in patients with aortic coarctation using the lattice Boltzmann methods: Clinical validation study.

Mirzaee H, Henn T, Krause MJ, Goubergrits L, Schumann C, Neugebauer M, Kuehne T, Preusser T, Hennemuth A.

J Magn Reson Imaging. 2017 Jan;45(1):139-146. doi: 10.1002/jmri.25366.


38. Genetic determinants of myocardial dysfunction.

Li X, Zhang P.

J Med Genet. 2017 Jan;54(1):1-10. doi: 10.1136/jmedgenet-2016-104308. Review.

39. Telecardiology and its settings of application: An update.

Molinari G, Molinari M, Di Biase M, Brunetti ND.

J Telemed Telecare. 2017 Jan 1:1357633X16689432. doi: 10.1177/1357633X16689432. [Epub ahead of print]

40. [Congenital Heart Diseases and Sports].

Wippermann F, Oberhoffer R, Hager A.

Klin Padiatr. 2017 Jan;229(1):21-26. doi: 10.1055/s-0042-120184. German.

41. The Long-Term Management of Children and Adults with a Fontan Circulation: A Systematic Review and Survey of Current Practice in Australia and New Zealand.

Gnanappa GK, Celermajer DS, Sholler GF, Gentles T, Winlaw D, d’Udekem Y, Ayer J.

Pediatr Cardiol. 2017 Jan;38(1):56-69. doi: 10.1007/s00246-016-1484-6.

42. Gas exchange responses during 6-min walk test in patients with pulmonary arterial hypertension.

Morris NR, Seale H, Harris J, Hall K, Lin AC, Kermeen F.

Respirology. 2017 Jan;22(1):165-171. doi: 10.1111/resp.12868.

43. Impact of Surgery and Valvuloplasty on Liver Stiffness in a Patient With Pericarditis and Pulmonary Valvulopathy.

Jalal Z, Iriart X, de Lédinghen V, Hiriart JB, Thambo JB.

World J Pediatr Congenit Heart Surg. 2017 Jan;8(1):103-105. doi: 10.1177/2150135115614575.

44. Changes in the Professional Lives of Cardiologists Over 2 Decades.

Lewis SJ, Mehta LS, Douglas PS, Gulati M, Limacher MC, Poppas A, Walsh MN, Rzeszut AK, Duvernoy CS; American College of Cardiology Women in Cardiology Leadership Council..

J Am Coll Cardiol. 2017 Jan 31;69(4):452-462. doi: 10.1016/j.jacc.2016.11.027. Review.

45. Cardiac Mechanoperception: A Life-Long Story from Early Beats to Aging and Failure.

Pesce M, Messina E, Chimenti I, Beltrami AP.

Stem Cells Dev. 2017 Jan 15;26(2):77-90. doi: 10.1089/scd.2016.0206.

46. An Unusual Case of Chest Pain in an Adolescent Male: Important Cues to Differential Diagnosis.

Jordan KS, Mannle SE.

Adv Emerg Nurs J. 2017 Jan/Mar;39(1):10-17. doi: 10.1097/TME.0000000000000135.

47. Prevalence and outcome of thrombotic and embolic complications in adults after Fontan operation.

Egbe AC, Connolly HM, Niaz T, Yogeswaran V, Taggart NW, Qureshi MY, Poterucha JT, Khan AR, Driscoll DJ.

Am Heart J. 2017 Jan;183:10-17. doi: 10.1016/j.ahj.2016.09.014.

48. Interassociation consensus statement on cardiovascular care of college student-athletes.

Hainline B, Drezner J, Baggish A, Harmon KG, Emery MS, Myerburg RJ, Sanchez E, Molossi S, Parsons JT, Thompson PD.

Br J Sports Med. 2017 Jan;51(2):74-85. doi: 10.1136/bjsports-2016-096323.

49. Effects of sildenafil on cardiac structure and function, cardiopulmonary exercise testing and health-related quality of life measures in heart failure patients with preserved ejection fraction and pulmonary hypertension.

Liu LC, Hummel YM, van der Meer P, Berger RM, Damman K, van Veldhuisen DJ, Voors AA, Hoendermis ES.

Eur J Heart Fail. 2017 Jan;19(1):116-125. doi: 10.1002/ejhf.662.

50. Severe Cardiomyopathy as the Isolated Presenting Feature in an Adult with Late-Onset Pompe Disease: A Case Report.

Mori M, Bailey LA, Estrada J, Rehder CW, Li JS, Rogers JG, Bali DS, Buckley AF, Kishnani PS.

JIMD Rep. 2017;31:79-83. doi: 10.1007/8904_2016_563.

51. Circulating microRNA as a Novel Biomarker for Pulmonary Arterial Hypertension Due to Congenital Heart Disease.

Chen W, Li S.

Pediatr Cardiol. 2017 Jan;38(1):86-94. doi: 10.1007/s00246-016-1487-3.

52. Impaired Vascular Function of the Aorta in Adolescents with Turner Syndrome.

An HS, Baek JS, Kim GB, Lee YA, Song MK, Kwon BS, Bae EJ, Noh CI.

Pediatr Cardiol. 2017 Jan;38(1):20-26. doi: 10.1007/s00246-016-1478-4.



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Fetal Cardiology Featured Articles of January 2017

Assessment of Progressive Pathophysiology after Early Prenatal Diagnosis of the Ebstein Anomaly or Tricuspid Valve Dysplasia.

Selamet Tierney ES, McElhinney DB, Freud LR, Tworetzky W, Cuneo BF, Escobar-Diaz MC, Ikemba C, Kalish BT, Komarlu R, Levasseur SM, Puchalski MD, Satou GM, Silverman NH, Moon-Grady AJ.

Am J Cardiol. 2017 Jan 1;119(1):106-111. doi: 10.1016/j.amjcard.2016.09.022.



Take Home Points:

  • Echocardiographic findings in Ebstein anomaly or tricuspid valve dysplasia (EA/TVD) fetuses presenting <24 weeks of gestation are unreliable indicators of physiologic status later in pregnancy.
  • In Ebstein anomaly or tricuspid valve dysplasia (EA/TVD) fetuses, progression of hemodynamic compromise later in pregnancy was common and we should be careful about counselling parents based on early gestation fetal echo findings.


Shaji Menon Portrait 12.15.14Comment from Dr. Shaji Menon (Salt Lake City), section editor of Pediatric Cardiology Journal Watch: This multicenter, retrospective study included 51 fetuses presenting at <24 weeks of gestation with EA/TVD and serial fetal echocardiograms ‡4 weeks apart. Absence of anterograde flow across the pulmonary valve, pulmonary valve regurgitation, cardiothoracic area ratio >0.48, left ventricular (LV) dysfunction, or tricuspid valve (TV) annulus Z-score >5.6 was considered markers of poor outcome. Eighteen fetuses (35%) had no markers for poor outcome on initial fetal echo, whereas only 7 had no markers of poor outcome in the third trimester. Pulmonary atresia developed in 33% of fetuses with antegrade pulmonary flow in the third trimester and 18% developed new pulmonary valve regurgitation. LV dysfunction was present in 2 (4%) patients at <24 weeks but in 14 (37%) to trimester (p <0.001). The TV annulus Z-score and cardiothoracic area both increased with advancing gestation.


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Does First Trimester Screening Modify the Natural History of Congenital Heart Disease? Analysis of Outcome of Regional Cardiac Screening at Two Different Time Periods.

Jicinska H, Vlasin P, Jicinsky M, Grochova I, Tomek V, Volaufova J, Skovranek J, Marek J.

Circulation. 2017 Jan 31. pii: CIRCULATIONAHA.115.020864. doi: 10.1161/CIRCULATIONAHA.115.020864. [Epub ahead of print]



Take Home Points:

  • First trimester ultrasound screening for congenital heart disease has made it possible to detect major cardiovascular abnormality is very early in gestation.
  • Early identification of congenital heart disease results in higher rates of termination of pregnancy with major congenital heart disease in the first trimester.
  • Fetuses diagnosed with complex congenital heart disease in the first trimester should be referred to fetal cardiologist for further evaluation and counseling.


Commentary from Dr. Shaji Menon (Salt Lake City), section editor of Pediatric Cardiology Journal Watch: This study evaluated fetus was diagnosed with congenital heart disease in the first trimester or in the second trimester screening retrospectively. A significantly higher number of fetuses with comorbidities and univentricular congenital heart diseases were diagnosed in first trimester screening compared to second trimester screening. Intrauterine deaths and termination of pregnancies were also significantly higher in first trimester screening.

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Assessment of Progressive Pathophysiology after Early Prenatal Diagnosis of the Ebstein Anomaly or Tricuspid Valve Dysplasia.

Selamet Tierney ES, McElhinney DB, Freud LR, Tworetzky W, Cuneo BF, Escobar-Diaz MC, Ikemba C, Kalish BT, Komarlu R, Levasseur SM, Puchalski MD, Satou GM, Silverman NH, Moon-Grady AJ.

Am J Cardiol. 2017 Jan 1;119(1):106-111. doi: 10.1016/j.amjcard.2016.09.022.

PMID: 27793395

Similar articles

Select item 27780555


Take Home Points:

  • In this study, Ebstein anomaly or tricuspid valve dysplasia diagnosed in utero tends to progress in severity over the course of the pregnancy with significant development of left and right ventricular dysfunction as well as ascites.
  • The only predictor of disease severity progression at the initial time of diagnosis (<24 weeks gestation) was the tricuspid valve z-score, in particular a z-score of 3 or greater. A larger cardiothoracic area ratio was also associated with development of right ventricular dysfunction.


Abarbanell picture smallComment from Dr. Ginnie Abarbanell (Atlanta), section editor of Fetal Cardiology Journal Watch: This multicenter study of 51 fetuses with Ebstein anomaly or tricuspid valve dysplasia sought to determine if echocardiographic indices at less than 24 weeks gestation could predict later pregnancy outcomes.  Researchers found that most fetuses that initially presented with no markers of poor hemodynamic status tended to develop markers of poor outcome by the third trimester.  Over a third of fetuses that initially had antegrade pulmonary blood flow developed pulmonary atresia while 18% developed new pulmonary valve insufficiency.  Left ventricular dysfunction developed in over a third of fetuses over the course of pregnancy.  Right ventricular dysfunction was present in 45% on follow up echocardiogram.  Ascites was present on follow up echocardiogram in slightly over 15% of fetuses.  The tricuspid valve annulus Z-score at time of diagnosis (<24 weeks gestation) was the only predictor of disease severity progression in initially low risk fetuses.  A tricuspid valve Z-score of 3 or greater showed the highest sensitivity and specificity.  Additionally, a larger cardiothoracic area ratio of >0.48 at initial diagnosis was associated with development of right ventricular dysfunction during the pregnancy.  This study demonstrates that a fetal low risk profile early in the pregnancy does not predict future hemodynamic status in pregnancy and a significant portion of fetuses with Ebstein anomaly/tricuspid valve dysplasia progressed with worsening disease severity over the course of pregnancy.

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Timing and Mode of Delivery in Prenatally Diagnosed Congenital Heart Disease- an Analysis of Practices within the University of California Fetal Consortium (UCfC).

Peyvandi S, Nguyen TA, Almeida-Jones M, Boe N, Rhee L, Anton T, Sklansky M, Tarsa M, Satou G, Moon-Grady AJ; University of California Fetal Consortium (UCfC)..

Pediatr Cardiol. 2017 Jan 11. doi: 10.1007/s00246-016-1552-y. [Epub ahead of print]

PMID: 28078382

Similar articles

Select item 28073791


Take Home Points:

  • Prenatal diagnosis of congenital heart disease (CHD) confers several benefits for the newborn with studies showing decreased mortality as well probable improved neurodevelopmental outcomes.
  • However, as this study from the University of California Fetal Consortium has demonstrated with prenatal diagnosis of CHD there is higher rate of earlier birth, lower birth weights and higher C-section rates. Researchers found that for every week increase in gestation age there was a 10-15% decrease in length of hospital stay as well as for every 100g increase in birth weight there was about a 4% decrease in hospital length of stay.
  • Researchers from this study conclude that neonates born with CHD will most likely have the best outcomes when delivered closer to term gestation and by vaginal delivery after natural onset of labor.


Comment from Dr. Ginnie Abarbanell (Atlanta), section editor of Fetal Cardiology Journal Watch:  Prenatal diagnosis of congenital heart disease (CHD) has been associated with decreased mortality as well as improved neurodevelopmental outcomes.  This study from the University of California Fetal Consortium also unfortunately demonstrates a higher rate of earlier births (38 vs. 39 weeks gestation), lower birth weight and increased C-section rate with prenatal diagnosis of CHD.  This study included 296 maternal–neonatal pairs (186 prenatally diagnosed and 110 postnatally diagnosed).  Within this cohort researches did not find a significant difference in 30 day survival, survival to hospital discharge or total length of hospital stay between the prenatal vs. postnatal diagnosis groups.  However, researchers did find that for every week increase in gestation age there was a 10-15% decrease in length of hospital stay as well as for every 100g increase in birth weight there was about a 4% decrease in hospital length of stay.  Additionally, there was a trend towards longer hospital stay for infants born via C-section.  Researchers conclude “full-term delivery (for the neonate) and vaginal delivery after natural onset of labor (for the mother and neonate) may lead to better outcomes for maternal–fetal pairs and has the potential to decrease healthcare costs” and that temporary relocation of some mothers closer to a tertiary care center may help achieve these goals.


Fetal Cardiology and Morphogenetics Jan 2017

1. Does First Trimester Screening Modify the Natural History of Congenital Heart Disease? Analysis of Outcome of Regional Cardiac Screening at Two Different Time Periods.

Jicinska H, Vlasin P, Jicinsky M, Grochova I, Tomek V, Volaufova J, Skovranek J, Marek J.

Circulation. 2017 Jan 31. pii: CIRCULATIONAHA.115.020864. doi: 10.1161/CIRCULATIONAHA.115.020864. [Epub ahead of print]

2. Fetal Echocardiographic Findings in Right Pulmonary Artery to Left Atrium Communication: A Case Report and Review of the Literature.

Ide T, Miyoshi T, Matsuyama TA, Kurosaki KI, Yoshimatsu J.

J Matern Fetal Neonatal Med. 2017 Jan 31:1-15. doi: 10.1080/14767058.2017.1289166. [Epub ahead of print]

3. NOTCH1-dependent nitric oxide signaling deficiency in hypoplastic left heart syndrome revealed through patient-specific phenotypes detected in bioengineered cardiogenesis.

Hrstka SC, Li X, Nelson TJ; Wanek Program Genetics Pipeline Group..

Stem Cells. 2017 Jan 31. doi: 10.1002/stem.2582. [Epub ahead of print]

4. Maternal exosomes in diabetes contribute to the cardiac development deficiency.

Shi R, Zhao L, Cai W, Wei M, Zhou X, Yang G, Yuan L.

Biochem Biophys Res Commun. 2017 Jan 29;483(1):602-608. doi: 10.1016/j.bbrc.2016.12.097.

5. Abnormal neurogenesis and cortical growth in congenital heart disease.

Morton PD, Korotcova L, Lewis BK, Bhuvanendran S, Ramachandra SD, Zurakowski D, Zhang J, Mori S, Frank JA, Jonas RA, Gallo V, Ishibashi N.

Sci Transl Med. 2017 Jan 25;9(374). pii: eaah7029. doi: 10.1126/scitranslmed.aah7029.

6. Temporally Distinct Six2-Positive Second Heart Field Progenitors Regulate Mammalian Heart Development and Disease.

Zhou Z, Wang J, Guo C, Chang W, Zhuang J, Zhu P, Li X.

Cell Rep. 2017 Jan 24;18(4):1019-1032. doi: 10.1016/j.celrep.2017.01.002.

7. Characterization of soluble N-ethylmaleimide-sensitive factor attachment protein receptor gene STX18 variations for possible roles in congenital heart diseases.

Li X, Shi S, Li FF, Cheng R, Han Y, Diao LW, Zhang Q, Zhi JX, Liu SL.

Gene. 2017 Jan 20;598:79-83. doi: 10.1016/j.gene.2016.10.043.

8. Endothelium in the pharyngeal arches 3, 4 and 6 is derived from the second heart field.

Wang X, Chen D, Chen K, Jubran A, Ramirez A, Astrof S.

Dev Biol. 2017 Jan 15;421(2):108-117. doi: 10.1016/j.ydbio.2016.12.010.

9. High throughput in vivo functional validation of candidate congenital heart disease genes in Drosophila.

Zhu JY, Fu Y, Nettleton M, Richman A, Han Z.

Elife. 2017 Jan 13;6. pii: e22617. doi: 10.7554/eLife.22617. [Epub ahead of print]

10. Timing and Mode of Delivery in Prenatally Diagnosed Congenital Heart Disease- an Analysis of Practices within the University of California Fetal Consortium (UCfC).

Peyvandi S, Nguyen TA, Almeida-Jones M, Boe N, Rhee L, Anton T, Sklansky M, Tarsa M, Satou G, Moon-Grady AJ; University of California Fetal Consortium (UCfC)..

Pediatr Cardiol. 2017 Jan 11. doi: 10.1007/s00246-016-1552-y. [Epub ahead of print]

11. Early transverse tubule development begins in utero in the sheep heart.

Munro ML, Soeller C.

J Muscle Res Cell Motil. 2017 Jan 6. doi: 10.1007/s10974-016-9462-4. [Epub ahead of print]

12. Alcohol Exposure Causes Overexpression of Heart Development-Related Genes by Affecting the Histone H3 Acetylation via BMP Signaling Pathway in Cardiomyoblast Cells.

Shi J, Zhao W, Pan B, Zheng M, Si L, Zhu J, Liu L, Tian J.

Alcohol Clin Exp Res. 2017 Jan;41(1):87-95. doi: 10.1111/acer.13273.

13. Role of microRNAs in cardiac development and disease.

Tian J, An X, Niu L.

Exp Ther Med. 2017 Jan;13(1):3-8. doi: 10.3892/etm.2016.3932.

14. Ultrasound Examination of the Fetal Heart.

Bishop KC, Kuller JA, Boyd BK, Rhee EH, Miller S, Barker P.

Obstet Gynecol Surv. 2017 Jan;72(1):54-61. doi: 10.1097/OGX.0000000000000394.

15. A discrete in continuous mathematical model of cardiac progenitor cells formation and growth as spheroid clusters (Cardiospheres).

Di Costanzo E, Giacomello A, Messina E, Natalini R, Pontrelli G, Rossi F, Smits R, Twarogowska M.

Math Med Biol. 2017 Jan 23. pii: dqw022. doi: 10.1093/imammb/dqw022. [Epub ahead of print]

16. Nkx2.5 regulates Endothelin Converting Enzyme-1 during pharyngeal arch patterning.

Iklé JM, Tavares AL, King M, Ding H, Colombo S, Firulli BA, Fiulli AB, Targoff KL, Yelon D, Clouthier DE.

Genesis. 2017 Jan 20. doi: 10.1002/dvg.23021. [Epub ahead of print]

17. Antenatal diagnosis of double-outlet left atrium.

Vaksmann G, Bouzguenda I, Houyel L.

Cardiol Young. 2017 Jan 12:1-3. doi: 10.1017/S104795111600250X. [Epub ahead of print]

18. Main pulmonary artery cross-section ratio is low in fetuses with tetralogy of Fallot and ductus arteriosus-dependent pulmonary circulation.

Ebishima H, Kurosaki K, Yoshimatsu J, Shiraishi I.

Cardiol Young. 2017 Jan 12:1-5. doi: 10.1017/S1047951116002675. [Epub ahead of print]


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CHD Surgery Featured Articles of January 2017

Is a hybrid strategy a lower-risk alternative to stage 1 Norwood operation?

Wilder TJ, McCrindle BW, Hickey EJ, Ziemer G, Tchervenkov CI, Jacobs ML, Gruber PJ, Blackstone EH, Williams WG, DeCampli WM, Caldarone CA, Pizarro C; Congenital Heart Surgeons’ Society..

J Thorac Cardiovasc Surg. 2017 Jan;153(1):163-172.e6. doi: 10.1016/j.jtcvs.2016.08.021.

PMID: 27671550

Similar articles

Select item 27665221



Take Home Points

  • When comparing all infants undergoing single ventricle palliation, the Norwood procedure with an RV-to-PA shunt may confer the best overall survival.
  • The hybrid procedure may mitigate the negative impact of lower birth weight, especially for very low birth weight infants.


jeremy-herrmannCommentary from Dr. Jeremy Herrmann (Indianapolis), section editor of Congenital Heart Surgery Journal Watch:   The authors present a study conducted through the Congenital Heart Surgeons’ Society multi-institutional database of neonates with critical left ventricular outflow tract obstruction (LVOTO) in which their primary aim was to compare outcomes of patients who underwent one of three initial palliative procedures: hybrid procedure (including stenting of the ductus arteriosus and banding of the pulmonary arteries, Norwood procedure with a modified Blalock-Taussig shunt (NW-BT), or a Norwood procedure with a right ventricular-to-pulmonary artery shunt (NW-RVPA). A total of 564 neonates were enrolled through 21 institutions including 232 who underwent NW-BT, 222 with NW-RVPA, and 110 who underwent a hybrid procedure. The median follow-up duration was 2.2 years (range, 1 day to 8.3 years).


On average, patients who underwent a hybrid procedure were slightly older, weighed less, and were more likely to have RV dysfunction compared to the Norwood groups. The NW-RVPA had a significantly larger percentage of patients with aortic valve atresia (60%). In a multivariable risk-adjusted comparison, 4-year survival without having achieved a definitive repair remained significantly better after NW-RVPA (76% ± 2%; P = .001) compared with NW-BT (60% ± 4%) and hybrid (61% ± 4%) (Figure 3). Consistent with other reports, other risk factors for death in this analysis included small aortic size and lower birth weight. Using propensity-score matching for risk adjustment, survival was similar between NW-BT and hybrid groups, but NW-RVPA demonstrated better survival versus each group (Figure 4).


Interestingly, the negative effect of lower birth weight on survival was less significant for the hybrid group, indicating this procedure may offer a slight survival advantage versus NW-BT for a birth weight <2 kg and against NW-BT for a birth weight <3 kg. Center volume appeared to be a risk factor for death in three of 14 centers that enrolled at least 10 patients, though this was not significant with risk-adjustment.


The authors conclude that the hybrid procedure may not be a lower-risk alternative for higher-risk patients, particularly compared to NW-RVPA for all patients. However, for infants with very low birth weight, the hybrid procedure may confer a protective benefit. The reasons for this are on not immediately clear, but the authors theorize this may be due to postponing palliation requiring cardiopulmonary bypass and the associated altered physiology until after the neonatal period.


Inclusion of information regarding causes of death and other known or suspected risk factors (e.g., genetic abnormalities, presence of a restrictive or intact atrial septum, presence of congenital pulmonary disorders, etc.) may have provided more insight into how patients specifically fare along these pathways. Unfortunately, at this time, most databases do not track these factors, and current risk adjustment models are likely not truly comprehensive.


Overall, this study adds further support to the notion that RV-PA shunts may offer a survival advantage in certain single ventricle physiology populations. In addition, for very low birth weight patients, the hybrid procedure appears to be a viable, less-risky initial approach to either Norwood strategy.

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Impact of Heart Transplantation on the Functional Status of U.S. Children with End-Stage Heart Failure. Analysis of Data from the Organ Procurement and Transplantation Network.

Peng DM, Zhang Y, Rosenthal DN, Palmon M, Chen S, Kaufman BD, Maeda K, Hollander S, McDonald N, Smoot LB, Bernstein D, Almond CS.

Circulation. 2017 Jan 24. pii: CIRCULATIONAHA.115.016520. doi: 10.1161/CIRCULATIONAHA.115.016520. [Epub ahead of print]

PMID: 28119383

Similar articles

Select item 28104076



Take Home Points

  • A majority of children who survive heart transplantation for more than one year exhibit functional status.
  • Heart transplantation is associated with improved functional status in most children.
  • Factors associated with less than normal or near-normal functional status include early rejection, older age, African American race, chronic steroid use, hemodynamic support at transplant, and being hospitalized at transplant.


Commentary from Dr. Jeremy Herrmann (Indianapolis), section editor of Congenital Heart Surgery Journal Watch:  Peng and colleagues present an analysis of functional status assessment in patients <21 years old who underwent heart transplantation (HT) between 2005 a 2014 in the United Status. Data for 1,633 patients were compiled from the Organ Procurement and Transplantation Network including functional status assessment at the time of listing, at HT, and one year after HT. The Karnofsky scale was utilized for assessing functional status in patients older than 18 years, and the Lansky scale was used for younger patients. For the final cohort, the median patient age was 11.0 years (IQR, 5-15 years) and median weight was 37.2 kg (IQR, 17.3-57.3 kg). Approximately 31% of HT were for congenital heart disease, 61% for cardiomyopathy, and 9% for redo HT.


As shown in the figure, the median functional status score (FSS) at the time of listing and at HT was 6, with 10 representing a normal status. This improved to a median FSS of 10 by 1 year of follow-up. Overall, at 1-year post-HT, the FSS increased in 86% of patients, declined in 3%, and remained unchanged in 12%. Risk factors for abnormal FSS (defined as ≤8) included age ≥18 years, African American race, graft rejection, inpatient at the time of HT, and requiring pharmacologic or mechanical support at HT.


This study is not only the largest of its kind, but it represents contemporary outcomes at the national level.

Other interesting findings included the slightly improved FSS at time of HT compared to listing, which the authors attributed to advanced heart failure therapies. It is unclear whether the duration of listing or duration of advanced support had any effect. In addition to the other conclusions, the authors argue for a more standardized inclusion of functional status in such databases, which seems justified given the present findings.

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CHD Surgery Jan 2017

1. Early mortality and concomitant procedures related to Fontan conversion: Quantitative analysis.

Brida M, Baumgartner H, Gatzoulis MA, Diller GP.

Int J Cardiol. 2017 Jan 25. pii: S0167-5273(16)34513-2. doi: 10.1016/j.ijcard.2017.01.111. [Epub ahead of print]

2. Pediatric airway surgery.

Maeda K.

Pediatr Surg Int. 2017 Jan 28. doi: 10.1007/s00383-016-4050-7. [Epub ahead of print] Review.

3. Early to mid-term results after total cavopulmonary connection performed in the second decade of life.

Metras A, Fouilloux V, Al-Yamani M, Roques X, Macé L, Thambo JB, Metras D, Kreitmann B, Roubertie F.

Interact Cardiovasc Thorac Surg. 2017 Jan 25. pii: ivw427. doi: 10.1093/icvts/ivw427. [Epub ahead of print]

4. The role of 3D printing in preoperative planning for heart transplantation in complex congenital heart disease.

Smith ML, McGuinness J, O’Reilly MK, Nolke L, Murray JG, Jones JF.

Ir J Med Sci. 2017 Jan 25. doi: 10.1007/s11845-017-1564-5. [Epub ahead of print]

5. Closing the gap in paediatric ventricular assist device therapy with the Berlin Heart EXCOR® 15-ml pump†.

De Rita F, Griselli M, Sandica E, Miera O, Karimova A, d’Udekem Y, Goldwasser R, Januszewska K, Amodeo A, Jurrmann N, Ersel S, Menon AK.

Interact Cardiovasc Thorac Surg. 2017 Jan 23. pii: ivw437. doi: 10.1093/icvts/ivw437. [Epub ahead of print]

6. Effect of Fenestration on Early Postoperative Outcome in Extracardiac Fontan Patients with Different Risk Levels.

Fan F, Liu Z, Li S, Yi T, Yan J, Yan F, Wang X, Wang Q.

Pediatr Cardiol. 2017 Jan 23. doi: 10.1007/s00246-016-1561-x. [Epub ahead of print]

7. Natural history of nonimmune-mediated thrombocytopenia and acute kidney injury in pediatric open-heart surgery.

Tew S, Fontes ML, Greene NH, Kertai MD, Ofori-Amanfo G, Jaquiss RD, Lodge AJ, Ames WA, Homi HM, Machovec KA, Jooste EH.

Paediatr Anaesth. 2017 Jan 18. doi: 10.1111/pan.13063. [Epub ahead of print]

8. The impact of liver disorders on perioperative management of reoperative cardiac surgery: a retrospective study in adult congenital heart disease patients.

Adachi K, Toyama H, Kaiho Y, Adachi O, Hudeta H, Yamauchi M.

J Anesth. 2017 Jan 16. doi: 10.1007/s00540-017-2308-4. [Epub ahead of print]

9. Bidirectional cavopulmonary anastomosis with additional pulmonary blood flow: good or bad pre-Fontan strategy.

Nichay NR, Gorbatykh YN, Kornilov IA, Soynov IA, Ivantsov SM, Gorbatykh AV, Ponomarev DN, Bogachev-Prokophiev AV, Karaskov AM.

Interact Cardiovasc Thorac Surg. 2017 Jan 15. pii: ivw429. doi: 10.1093/icvts/ivw429. [Epub ahead of print]

10. The “basic” approach: a single-centre experience with a cost-reducing model for paediatric cardiac extracorporeal membrane oxygenation.

Padalino MA, Tessari C, Guariento A, Frigo AC, Vida VL, Marcolongo A, Zanella F, Harvey MJ, Thiagarajan RR, Stellin G.

Interact Cardiovasc Thorac Surg. 2017 Jan 10. pii: ivw381. doi: 10.1093/icvts/ivw381. [Epub ahead of print]

11. Le-Compte maneuver in surgical correction of absent pulmonary valve. Does it improve severe bronchial compression?

Sarıtaş B, Özker E, Sarısoy Ö, Şahin M, Gümüş B, Ayabakan C.

Int J Cardiol. 2017 Jan 6. pii: S0167-5273(16)32123-4. doi: 10.1016/j.ijcard.2017.01.061. [Epub ahead of print] No abstract available.

12. Evaluation of left pulmonary artery sling, associated cardiovascular anomalies, and surgical outcomes using cardiovascular computed tomography angiography.

Xie J, Juan YH, Wang Q, Chen J, Zhuang J, Xie Z, Liang C, Zhu Y, Yu Z, Li J, Saboo SS, Liu H.

Sci Rep. 2017 Jan 5;7:40042. doi: 10.1038/srep40042.


13. Extubation Failure after Neonatal Cardiac Surgery: A Multicenter Analysis.

Mastropietro CW, Cashen K, Grimaldi LM, Narayana Gowda KM, Piggott KD, Wilhelm M, Gradidge E, Moser EA, Benneyworth BD, Costello JM.

J Pediatr. 2017 Jan 4. pii: S0022-3476(16)31427-5. doi: 10.1016/j.jpeds.2016.12.028. [Epub ahead of print]

14. A Post-operative Feeding Protocol to Improve Outcomes for Neonates With Critical Congenital Heart Disease.

Newcombe J, Fry-Bowers E.

J Pediatr Nurs. 2017 Jan 4. pii: S0882-5963(16)30097-5. doi: 10.1016/j.pedn.2016.12.010. [Epub ahead of print]

15. Total Anomalous Pulmonary Venous Connection: The Current Management Strategies in a Pediatric Cohort of 768 Patients.

Shi G, Zhu Z, Chen J, Ou Y, Hong H, Nie Z, Zhang H, Liu X, Zheng J, Sun Q, Liu J, Chen H, Zhuang J.

Circulation. 2017 Jan 3;135(1):48-58. doi: 10.1161/CIRCULATIONAHA.116.023889.

16. Minimally Invasive Esophagectomy in a Patient With Tetralogy of Fallot and Right-Sided Aortic Arch.

Thomas MJ, Bartlett HL, Bassetti MF, Lubner SJ, Kirvassilis G, Anagnostopoulos PV, Maloney JD, Macke RA.

Ann Thorac Surg. 2017 Jan;103(1):e77-e79. doi: 10.1016/j.athoracsur.2016.06.092.

17. HeartWare Ventricular Assist Device Implantation in Patients With Fontan Physiology.

Imielski BR, Niebler RA, Kindel SJ, Woods RK.

Artif Organs. 2017 Jan;41(1):40-46. doi: 10.1111/aor.12852.

18. Extracorporeal Membrane Oxygenation Outcomes After the Comprehensive Stage II Procedure in Patients With Single Ventricles.

Gomez D, Duffy V, Hersey D, Backes C, Rycus P, McConnell P, Voss J, Galantowicz M, Cua CL.

Artif Organs. 2017 Jan;41(1):66-70. doi: 10.1111/aor.12810.

19. Mechanical Circulatory Support Devices for Pediatric Patients With Congenital Heart Disease.

Chopski SG, Moskowitz WB, Stevens RM, Throckmorton AL.

Artif Organs. 2017 Jan;41(1):E1-E14. doi: 10.1111/aor.12760. Review.

20. Topical and low-dose intravenous tranexamic acid in cyanotic cardiac surgery.

Patel J, Prajapati M, Patel H, Gandhi H, Deodhar S, Pandya H.

Asian Cardiovasc Thorac Ann. 2017 Jan 1:218492316688416. doi: 10.1177/0218492316688416. [Epub ahead of print]

21. Five decades of pediatric heart transplantation: challenges overcome, challenges remaining.

Zangwill S.

Curr Opin Cardiol. 2017 Jan;32(1):69-77.

22. Management of Opioid and Sedative Weaning in Pediatric Congenital Heart Disease Patients: Assessing the State of Practice.

O’Connell C, Ziniel S, Hartwell L, Connor J.

Dimens Crit Care Nurs. 2017 Mar/Apr;36(2):116-124. doi: 10.1097/DCC.0000000000000229.

23. Orthotropic heart transplantation for adult congenital heart disease: a case with heterotaxy and dextrocardia.

Matsuda H, Fukushima N, Ichikawa H, Sawa Y.

Gen Thorac Cardiovasc Surg. 2017 Jan;65(1):47-51. doi: 10.1007/s11748-015-0573-4.

24. Single center experience of aortic bypass graft for aortic arch obstruction in children.

Shinkawa T, Chipman C, Holloway J, Tang X, Gossett JM, Imamura M.

Heart Vessels. 2017 Jan;32(1):76-82. doi: 10.1007/s00380-016-0842-x.

25. A novel procedure for reconstructing an extensive hypoplastic aortic arch in older children.

Wu S, Yang Y, Hu S, Zhao T.

Interact Cardiovasc Thorac Surg. 2017 Jan;24(1):132-134. doi: 10.1093/icvts/ivw320.

26. Is a hybrid strategy a lower-risk alternative to stage 1 Norwood operation?

Wilder TJ, McCrindle BW, Hickey EJ, Ziemer G, Tchervenkov CI, Jacobs ML, Gruber PJ, Blackstone EH, Williams WG, DeCampli WM, Caldarone CA, Pizarro C; Congenital Heart Surgeons’ Society..

J Thorac Cardiovasc Surg. 2017 Jan;153(1):163-172.e6. doi: 10.1016/j.jtcvs.2016.08.021.

27. A derived and validated score to predict prolonged mechanical ventilation in patients undergoing cardiac surgery.

Sharma V, Rao V, Manlhiot C, Boruvka A, Fremes S, Wąsowicz M.

J Thorac Cardiovasc Surg. 2017 Jan;153(1):108-115. doi: 10.1016/j.jtcvs.2016.08.020.

28. Prevention preferable to treatment: 3 case reports of patients experiencing right-sided heart failure after Ebstein anomaly correction.

Luo M, Lin J, Qin Z, Du L.

Medicine (Baltimore). 2017 Jan;96(1):e5627. doi: 10.1097/MD.0000000000005627.

29. Reversal of angiographic findings of moyamoya syndrome after congenital cyanotic heart disease repair.

Pande AC, Kesav P, Sreedharan SE, Sylaja PN.

Neurol India. 2017 Jan-Feb;65(1):190-191. doi: 10.4103/0028-3886.198202. No abstract available.


30. Adrenal insufficiency in neonates after cardiac surgery with cardiopulmonary bypass.

Crawford JH, Hull MS, Borasino S, Steenwyk BL, Hock KM, Wall K, Alten JA.

Paediatr Anaesth. 2017 Jan;27(1):77-84. doi: 10.1111/pan.13013.

31. Clinical Associations of Early Dysnatremias in Critically Ill Neonates and Infants Undergoing Cardiac Surgery.

Kaufman J, Phadke D, Tong S, Eshelman J, Newman S, Ruzas C, da Cruz EM, Osorio S.

Pediatr Cardiol. 2017 Jan;38(1):149-154. doi: 10.1007/s00246-016-1495-3.

32. Oncotic pressure and paediatric cardiopulmonary bypass: establishing baseline data for complex congenital cardiac surgery and its relation to risk stratification.

Crook R, Issitt R.

Perfusion. 2017 Jan 1:267659117690251. doi: 10.1177/0267659117690251. [Epub ahead of print]

33. Anaesthetic management of breast surgery in a patient with Eisenmenger syndrome.

Galán Gutiérrez JC, Fernández Suárez FE, Miranda García P, Sopena Zubiria LA.

Rev Esp Anestesiol Reanim. 2017 Jan;64(1):41-45. doi: 10.1016/j.redar.2016.07.007. English, Spanish.

34. Surgical Considerations in Total Anomalous Pulmonary Venous Connection.

Shaw FR, Chen JM.

Semin Cardiothorac Vasc Anesth. 2017 Jan 1:1089253216688535. doi: 10.1177/1089253216688535. [Epub ahead of print]

35. Valve-Sparing Aortic Root Replacement in Pediatric Patients: Lessons Learned Over Two Decades.

Vricella LA, Cameron DE.

Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2017 Jan;20:56-62. doi: 10.1053/j.pcsu.2016.10.001. Review.

36. The 50/50 cc Total Artificial Heart Trial: Extending the Benefits of the Total Artificial Heart to Underserved Populations.

Wells D, Villa CR, Simón Morales DL.

Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2017 Jan;20:16-19. doi: 10.1053/j.pcsu.2016.09.004. Review.

37. Surgical Site Infection After Pediatric Cardiothoracic Surgery.

Sochet AA, Cartron AM, Nyhan A, Spaeder MC, Song X, Brown AT, Klugman D.

World J Pediatr Congenit Heart Surg. 2017 Jan;8(1):7-12. doi: 10.1177/2150135116674467.

38. Clinical Databases and Registries in Congenital and Pediatric Cardiac Surgery, Cardiology, Critical Care, and Anesthesiology Worldwide.

Vener DF, Gaies M, Jacobs JP, Pasquali SK.

World J Pediatr Congenit Heart Surg. 2017 Jan;8(1):77-87. doi: 10.1177/2150135116681730.

39. Common Arterial Trunk Repair by Means of a Handmade Bovine Pericardial-Valved Woven Dacron Conduit.

Ramírez-Marroquín S, Curi-Curi PJ, Calderón-Colmenero J, García-Montes JA, Cervantes-Salazar JL.

World J Pediatr Congenit Heart Surg. 2017 Jan;8(1):69-76. doi: 10.1177/2150135116674439.

40. The Accuracy of Noninvasive Peripheral Pulse Oximetry After Palliative Cardiac Surgery in Patients With Cyanotic Congenital Heart Disease.

Scrimgeour GE, Griksaitis MJ, Pappachan JV, Baldock AJ.

World J Pediatr Congenit Heart Surg. 2017 Jan;8(1):32-38. doi: 10.1177/2150135116673016.

41. Virtual 3-Dimensional Computed Tomographic Surgical Planning for Severe Aortic Coarctation/Aneurysm in the Setting of Bicuspid Aortic Valve.

Shinbane JS, Ghanshani S, Starnes V.

World J Pediatr Congenit Heart Surg. 2017 Jan;8(1):99-100. doi: 10.1177/2150135116668336. No abstract available.

42. Short-Term Results of Sinus of Valsalva Aneurysm Repair.

Abralov K, Alimov A.

World J Pediatr Congenit Heart Surg. 2017 Jan;8(1):13-17. doi: 10.1177/2150135116673809.

43. Preoperative Feeding Neonates With Cardiac Disease.

Scahill CJ, Graham EM, Atz AM, Bradley SM, Kavarana MN, Zyblewski SC.

World J Pediatr Congenit Heart Surg. 2017 Jan;8(1):62-68. doi: 10.1177/2150135116668833.

44. Impact of Heart Transplantation on the Functional Status of U.S. Children with End-Stage Heart Failure. Analysis of Data from the Organ Procurement and Transplantation Network.

Peng DM, Zhang Y, Rosenthal DN, Palmon M, Chen S, Kaufman BD, Maeda K, Hollander S, McDonald N, Smoot LB, Bernstein D, Almond CS.

Circulation. 2017 Jan 24. pii: CIRCULATIONAHA.115.016520. doi: 10.1161/CIRCULATIONAHA.115.016520. [Epub ahead of print]

45. Central Venous to Arterial CO2 Difference After Cardiac Surgery in Infants and Neonates.

Rhodes LA, Erwin WC, Borasino S, Cleveland DC, Alten JA.

Pediatr Crit Care Med. 2017 Jan 24. doi: 10.1097/PCC.0000000000001085. [Epub ahead of print]

46. Prolonged circulatory support with short-term continuous-flow pump in an infant with end-stage heart failure.

Knezevic I, Vrtovec B, Ksela J.

Transpl Int. 2017 Jan 21. doi: 10.1111/tri.12922. [Epub ahead of print]

47. Giant saccular aneurysm of the descending aorta in an infant.

Onan IS, Ozturk E, Akyol MB, Onan B.

J Card Surg. 2017 Jan 19. doi: 10.1111/jocs.13095. [Epub ahead of print] No abstract available.

48. Early and late outcomes after surgical management of congenital vascular rings.

François K, Panzer J, De Groote K, Vandekerckhove K, De Wolf D, De Wilde H, Marchau F, De Caluwe W, Benatar A, Bové T.

Eur J Pediatr. 2017 Jan 13. doi: 10.1007/s00431-017-2850-y. [Epub ahead of print]

49. Idiopathic infantile arterial calcification: a case report of successful extracorporeal membrane oxygenation support.

Deshpande SR, Kasniya G, Cuadrado AR, Maher KO.

Int J Artif Organs. 2017 Jan 12:0. doi: 10.5301/ijao.5000544. [Epub ahead of print]

50. The End of Life Experience of Pediatric Heart Transplant Recipients.

Hollander SA, Dykes J, Chen S, Barkoff L, Sourkes B, Cohen H, Rosenthal DN, Bernstein D, Kaufman BD.

J Pain Symptom Manage. 2017 Jan 4. pii: S0885-3924(16)31235-0. doi: 10.1016/j.jpainsymman.2016.12.334. [Epub ahead of print]

51. The Surgical Repair of a Hyperimmunoglobulin E Syndrome Associated Ascending Aortic Aneurysm.

Battaloglu B, Colak C, Disli OM, Akca B, Erdil N, Karakurt C.

Ann Thorac Surg. 2017 Jan;103(1):e65-e67. doi: 10.1016/j.athoracsur.2016.06.021.

52. Comparable Cerebral Blood Flow in Both Hemispheres During Regional Cerebral Perfusion in Infant Aortic Arch Surgery.

Rüffer A, Tischer P, Münch F, Purbojo A, Toka O, Rascher W, Cesnjevar RA, Jüngert J.

Ann Thorac Surg. 2017 Jan;103(1):178-185. doi: 10.1016/j.athoracsur.2016.05.088.

53. Mechanical Circulatory Support as Bridge to Transplantation for the Failing Single Ventricle.

Arnaoutakis GJ, Blitzer D, Fuller S, Eckhauser AW, Montenegro LM, Rossano JW, Gaynor JW.

Ann Thorac Surg. 2017 Jan;103(1):193-197. doi: 10.1016/j.athoracsur.2016.05.015.

54. Routine Use of Distal Arterial Perfusion in Pediatric Femoral Venoarterial Extracorporeal Membrane Oxygenation.

Schad CA, Fallon BP, Monteagudo J, Okochi S, Cheung EW, Morrissey NJ, Kadenhe-Chiweshe AV, Aspelund G, Stylianos S, Middlesworth W.

Artif Organs. 2017 Jan;41(1):11-16. doi: 10.1111/aor.12861.

55. Successful Bridge-to-Transplant of Functionally Univentricular Patients With a Modified Continuous-Flow Ventricular Assist Device.

Mongé MC, Kulat BT, Eltayeb O, Zingle NR, Moss ST, Gossett JG, Pahl E, Costello JM, Backer CL.

Artif Organs. 2017 Jan;41(1):25-31. doi: 10.1111/aor.12881.

56. Concurrent use of continuous and pulsatile flow Ventricular Assist Device on a fontan patient: A simulation study.

Di Molfetta A, Ferrari G, Iacobelli R, Filippelli S, Amodeo A.

Artif Organs. 2017 Jan;41(1):32-39. doi: 10.1111/aor.12859.

57. Sources of Circuit Thrombosis in Pediatric Extracorporeal Membrane Oxygenation.

Hastings SM, Ku DN, Wagoner S, Maher KO, Deshpande S.

ASAIO J. 2017 Jan/Feb;63(1):86-92. doi: 10.1097/MAT.0000000000000444.

58. Transplant Survival After Berlin Heart EXCOR Support.

Bryant R 3rd, Zafar F, Castleberry C, Jefferies JL, Lorts A, Chin C, Morales DL.

ASAIO J. 2017 Jan/Feb;63(1):80-85. doi: 10.1097/MAT.0000000000000439.

59. Intraoperative diagnosis of a quadricuspid aortic valve.

Tatari H, Dehaki MG, Omrani G, Ghaheri H, Al-Dairy A, Mortezaeian H.

Asian Cardiovasc Thorac Ann. 2017 Jan 1:218492317692467. doi: 10.1177/0218492317692467. [Epub ahead of print]

60. Total anomalous pulmonary venous connection with ventricular septal defects.

Fuchigami T, Nishioka M, Akashige T, Nabeshima T, Nagata N.

Asian Cardiovasc Thorac Ann. 2017 Jan;25(1):62-64. doi: 10.1177/0218492315622102.

61. Development and Validation of a Score to Predict Mortality in Children Undergoing Extracorporeal Membrane Oxygenation for Respiratory Failure: Pediatric Pulmonary Rescue With Extracorporeal Membrane Oxygenation Prediction Score.

Bailly DK, Reeder RW, Zabrocki LA, Hubbard AM, Wilkes J, Bratton SL, Thiagarajan RR; Extracorporeal Life Support Organization Member Centers..

Crit Care Med. 2017 Jan;45(1):e58-e66.

62. Aortic valve repair in the paediatric population: insights from a 38-year single-centre experience.

Poncelet AJ, El Khoury G, De Kerchove L, Sluysmans T, Moniotte S, Momeni M, Detaille T, Rubay JE.

Eur J Cardiothorac Surg. 2017 Jan;51(1):43-49. doi: 10.1093/ejcts/ezw259.

63. Scan, plan, print, practice, perform: Development and use of a patient-specific 3-dimensional printed model in adult cardiac surgery.

Hermsen JL, Burke TM, Seslar SP, Owens DS, Ripley BA, Mokadam NA, Verrier ED.

J Thorac Cardiovasc Surg. 2017 Jan;153(1):132-140. doi: 10.1016/j.jtcvs.2016.08.007.

64. Preoperative Staphylococcus aureus Carriage and Risk of Surgical Site Infection After Cardiac Surgery in Children Younger than 1 year: A Pilot Cohort Study.

Macher J, Gras Le Guen C, Chenouard A, Liet JM, Gaillard Le Roux B, Legrand A, Mahuet J, Launay E, Gournay V, Joram N.

Pediatr Cardiol. 2017 Jan;38(1):176-183. doi: 10.1007/s00246-016-1499-z.

65. Prediction of Fluid Responsiveness Using Pulse Pressure Variation in Infants Undergoing Ventricular Septal Defect Repair with Median Sternotomy or Minimally Invasive Right Thoracotomy.

Han D, Liu YG, Luo Y, Li J, Ou-Yang C.

Pediatr Cardiol. 2017 Jan;38(1):184-190. doi: 10.1007/s00246-016-1500-x.

66. Predicting the Need for Neoaortic Arch Intervention in Infants with Hypoplastic Left Heart Syndrome Through the Glenn Procedure.

Eagam M, Loomba RS, Pelech AN, Tweddell JS, Kirkpatrick E.

Pediatr Cardiol. 2017 Jan;38(1):70-76. doi: 10.1007/s00246-016-1485-5.

67. Interstage Survival for Patients with Hypoplastic Left Heart Syndrome After ECMO.

Fernandez RP, Joy BF, Allen R, Stewart J, Miller-Tate H, Miao Y, Nicholson L, Cua CL.

Pediatr Cardiol. 2017 Jan;38(1):50-55. doi: 10.1007/s00246-016-1483-7.

68. Improved Survival While Waiting and Risk Factors for Death in Pediatric Patients Listed for Cardiac Transplantation.

Zakaria D, Frazier E, Imamura M, Garcia X, Pye S, Knecht KR, Prodhan P, Gossett JR, Swearingen CJ, Morrow WR.

Pediatr Cardiol. 2017 Jan;38(1):77-85. doi: 10.1007/s00246-016-1486-4.

69. Coronary Artery Bypass Grafting and Percutaneous Coronary Intervention after Kawasaki Disease: The Pediatric Canadian Series.

Dionne A, Bakloul M, Manlhiot C, McCrindle BW, Hosking M, Houde C, Pepelassis D, Dahdah N.

Pediatr Cardiol. 2017 Jan;38(1):36-43. doi: 10.1007/s00246-016-1480-x.

70. Acute Kidney Injury in Patients Undergoing the Extracardiac Fontan Operation With and Without the Use of Cardiopulmonary Bypass.

Algaze CA, Koth AM, Faberowski LW, Hanley FL, Krawczeski CD, Axelrod DM.

Pediatr Crit Care Med. 2017 Jan;18(1):34-43. doi: 10.1097/PCC.0000000000000984.

71. Off-Label Use of Medical Devices in Children.


Pediatrics. 2017 Jan;139(1). pii: e20163439. doi: 10.1542/peds.2016-3439.

72. Cardiac output: a central issue in patients with respiratory extracorporeal support.

Romagnoli S, Zagli G, Ricci Z, Villa G, Barbani F, Pinelli F, De Gaudio R, Chelazzi C.

Perfusion. 2017 Jan;32(1):44-49. doi: 10.1177/0267659116658112.

73. Closing in on the PumpKIN Trial of the Jarvik 2015 Ventricular Assist Device.

Baldwin JT, Adachi I, Teal J, Almond CA, Jaquiss RD, Massicotte MP, Dasse K, Siami FS, Zak V, Kaltman JR, Mahle WT, Jarvik R.

Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2017 Jan;20:9-15. doi: 10.1053/j.pcsu.2016.09.003. Review.

74. The Evolution of an Adult Congenital Heart Surgery Program: The Emory System.

Kogon B, Rosenblum J, Alsoufi B, Shashidharan S, Book W.

Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2017 Jan;20:28-32. doi: 10.1053/j.pcsu.2016.09.011. Review.

75. Mechanical Circulatory Support of the Fontan Patient.

Woods RK, Ghanayem NS, Mitchell ME, Kindel S, Niebler RA.

Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2017 Jan;20:20-27. doi: 10.1053/j.pcsu.2016.09.009. Review.

76. Surgical Site Infection After Pediatric Cardiothoracic Surgery.

Sochet AA, Cartron AM, Nyhan A, Spaeder MC, Song X, Brown AT, Klugman D.

World J Pediatr Congenit Heart Surg. 2017 Jan;8(1):7-12. doi: 10.1177/2150135116674467.

77. Severe Pulmonary Vascular Obstructive Disease After Neonatal Arterial Switch Operation for Simple Transposition of the Great Arteries.

Kimura D, Briceno-Medina M, Kumar TK, Knott-Craig CJ.

World J Pediatr Congenit Heart Surg. 2017 Jan;8(1):114-116. doi: 10.1177/2150135115623288.

78. Intramyocardial Hematoma After Ebstein Anomaly Repair.

Lim JK, Lee JH, Mok YH, Chen CK, Loh YJ.

World J Pediatr Congenit Heart Surg. 2017 Jan;8(1):117-120. doi: 10.1177/2150135115623528.


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Pediatric Cardiology Featured Articles of December 2016

Neuropsychiatric aspects of 22q11.2 deletion syndrome: considerations in the prenatal setting.

Bassett AS, Costain G, Marshall CR.

Prenat Diagn. 2017 Jan;37(1):61-69. doi: 10.1002/pd.4935.

Take Home Points:

  • This is excellent review of the neuropsychiatric aspects of 22q11.2 deletion syndrome and a useful resource for prenatally counselling.
  • Neuropsychiatric conditions associated with 22q11.2 deletions are on a spectrum ranging for intellectual disability, schizophrenia, epilepsy and early onset Parkinson disease.
  • There is no specific test or clinical finding currently known to predict the severity of neuropsychiatric conditions associated with 22q11.2 deletion.

Abarbanell picture smallComment from Dr. Ginnie Abarbanell (Atlanta), section editor of Pediatric Cardiology Journal Watch: It is well known that certain congenital heart defects (CHD) are associated with 22q11.2 deletion (DiGeorge) syndrome.  However, many pediatric cardiologists may not be as educated regarding the neuropsychiatric aspects of 22q11.2 deletion syndrome.  This article is an excellent review of the neuropsychiatric aspects of 22q11.2 deletion syndrome written specifically to help medical practitioners in counselling families with a new diagnosis in the prenatal period.  22q11.2 deletion occurs in about 1 in 1000 pregnancies and occurs de novo in about 90% of cases.  Studies have found that up to 1 in 10 newly diagnosed 22q11.2 deletion cases a parent will also be found to have the deletion.  The prevalence of 22q11.2 deletion with CHD ranges from 5-50%.

  • VSD 5-10%
  • Tetralogy of Fallot ~15%
  • Truncus arteriosus ~33%
  • Interrupted aortic arch type B ~50%

It has been estimated that 40-75% of patients with 22q11.2 deletion syndrome have CHD with a spectrum of severity.  Without the presence of CHD, 22q11.2 deletion syndrome can be difficult to diagnose in utero.  Neuropsychiatric conditions associated with 22q11.2 deletions are on a spectrum ranging for intellectual disability, schizophrenia, epilepsy and early onset Parkinson disease.  Unfortunately, there is no current testing or clinical findings that accurately predict the severity of neuropsychiatric conditions associated with 22q11.2 deletion.  This review provides very relevant information for prenatal counselling which is summarized below.

  • 30-40% of patients with 22q11.2 deletion will have mild intellectual disability and most will have an IQ in the borderline range.
  • Schizophrenia occurs in 1 in 4 patients with 22q11.2 deletion syndrome which is over a 20-fold greater risk than the general population.
  • Autism spectrum disorders and attention deficit disorders are common presentations of 22q11.2 deletion syndrome in childhood.
  • There is an increased prevalence of epilepsy in 22q11.2 deletion syndrome of 5-7% compared to 0.5-1% in the general population.
  • 2 deletion syndrome has been associated with an increased risk for early onset Parkinson’s disease.

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Proportion of selected congenital heart defects attributable to recognized risk factors.

Simeone RM, Tinker SC, Gilboa SM, Agopian AJ, Oster ME, Devine OJ, Honein MA; National Birth Defects Prevention Study..

Ann Epidemiol. 2016 Dec;26(12):838-845. doi: 10.1016/j.annepidem.2016.10.003.

Take Home Points:

  • There may be modifiable risk factors to prevent congenital heart disease.
  • This population based study found that modifiable risk factors may have contributed to 10% of cases with hypoplastic left heart syndrome and 18.6% of Tetralogy of Fallot cases.
  • Potential modifiable risk factors identified in this study included prepregnancy overweight-obesity, opioid use, and pregestational diabetes.

Comment from Dr. Ginnie Abarbanell (Atlanta), section editor of Pediatric Cardiology Journal Watch: This study sought to evaluate the contribution of multiple risk factors on hypoplastic left heart syndrome (HLHS) and Tetralogy of Fallot (TOF) using data from the National Birth Defects Prevention Study.  The National Birth Defect Prevention study is a population based case-control study including birth defect cases from Arkansas, Iowa, New Jersey, Utah, California, Georgia, Massachusetts, North Carolina, New York and Texas.  Researchers in this study estimated the average adjusted population attributable fractions (aaPAF) for several recognized risk factors for HLHS and TOF.  As the authors describe “The aaPAF can therefore be thought of as the expected impact of removing each risk factor from the population across all possible sequences of doing so relative to the other risk factors”.  594 HLHS cases, 971 TOF cases and 11,829 controls were included in the analysis.  Approximately 40% of HLHS cases and 60% of TOF cases could not be explained by any of the risk factors evaluated.  However, 57% of HLHS cases were estimated to be attributable to studied risk factors of which 45.6% were secondary to nonmodifiable risk facts of nonHispanic White race, male sex and family history of congenital heart disease.  Modifiable risk factors were identified in slightly over 10% of HLHS cases which included modifiable risk factors of maternal pre-pregnancy overweight-obesity (6.5%), opioid use (1.5%) and maternal report of fever (1.4%).  Similarly, in TOF cases, 37% of cases were estimated to be attributable to studied risk factors of which 18.6% were secondary to nonmodifiable risk factors of male sex, family history of CHD and nulliparity.  Modifiable risk factors were identified in almost half (18.6%) of TOF cases including risk factors of pre-pregnancy overweight/obesity, advanced maternal age and pregestational diabetes.  These findings may guide public health interventions regarding congenital heart disease, with specific focus on HLHS and TOF.



Perioperative Feeding Approaches in Single Ventricle Infants: A Survey of 46 Centers.

Slicker J, Sables-Baus S, Lambert LM, Peterson LE, Woodard FK, Ocampo EC; National Pediatric Cardiology-Quality Improvement Collaborative Feeding Work Group..

Congenit Heart Dis. 2016 Dec;11(6):707-715. doi: 10.1111/chd.12390.

Take Home Points:

  • In this survey of 46 pediatric cardiac surgery centers, perioperative feeding practices in infants with single ventricle was variable and often center dependent.
  • 65% of the centers fed single ventricle infants preoperatively.
  • Over 50% of centers reported sending patients home postoperatively with a nasogastric tube feeds or gastric tube feeds.

Comment from Dr. Ginnie Abarbanell (Atlanta), section editor of Pediatric Cardiology Journal Watch: A survey was conducted of the centers participating in the National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC) regarding perioperative feeding practices in infants with single ventricle.  Results of the survey were variable and often center dependent.  65% fed single ventricle infants preoperatively.  63% of centers allowed infants to breast feed and 73% used tube feedings preoperatively.  Postoperatively, over 50% followed a written feeding evaluation guideline prior to feeding infants orally after stage 1 palliation.  Over 50% of center survey responders reported sending patients home with nasogastric tube (NGT) or gastric tube (GT) feedings.  There was no significant difference in the use of NGT vs GT at discharge.  The authors in the discussion review the concerns of perioperative complications with feeding practices verses possible benefits.  As the authors conclude further investigation in a larger cohort is needed to determine the best feeding practices in this population.

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Implementation of a Quality Improvement Bundle Improves Echocardiographic Imaging after Congenital Heart Surgery in Children.

Parthiban A, Levine JC, Nathan M, Marshall JA, Shirali GS, Simon SD, Colan SD, Newburger JW, Raghuveer G.

J Am Soc Echocardiogr. 2016 Dec;29(12):1163-1170.e3. doi: 10.1016/j.echo.2016.09.002.

Take Home Points: 

  • This study demonstrates improved post-operative imaging after implementation of a quality improvement initiative which included specific targeting imaging protocols, sonographer education and use of sedation when needed.

Comment from Dr. Ginnie Abarbanell (Atlanta), section editor of Pediatric Cardiology Journal Watch: Echocardiograms can be difficult to perform in the post-operative period secondary to poor image windows, agitation, pain as well as the presence of bandages and drains. This quality improvement study demonstrates that through the use of focused imaging protocols, procedural sedation and sonographer education the rate of optimal, postoperative echocardiographic imaging can improve.  Researchers found a 3 to 4 fold increase in optimal imaging after initiating a quality improvement bundle consisting of prespecified, targeting imaging protocol for 4 surgical repairs (Tetralogy of Fallot, arterial switch operation, bidirectional Glenn and Fontan procedures), use of sedation and sonographer education.  The optimal imaging rates improved from 69% to 85% for intracardiac structures and from 35% to 67% for extracardiac structures.  Researchers report the following “important lessons” learned from this quality improvement project.

  • A quality improvement program can be successfully implanted in high volume programs.
  • Certain structures even under optimal conditions were difficult to image (for example coronary arteries after arterial switch operation and Fontan conduit anastomoses).
  • Procedural sedation was used without adverse events and improved the image quality.

ped 3


Pediatric Cardiology Articles – December 2016


  1. Platelet Inhibition in Shunted Infants on Aspirin at Short and Midterm Follow-Up.

Truong DT, Johnson JT, Bailly DK, Clawson JR, Sheng X, Burch PT, Witte MK, LuAnn Minich L.

Pediatr Cardiol. 2016 Dec 30. doi: 10.1007/s00246-016-1529-x. [Epub ahead of print]

  1. [Ebstein’s “like” anomaly ventricular double inlet. A rare association].

Muñoz Castellanos L, Kuri Nivon M.

Arch Cardiol Mex. 2016 Dec 30. pii: S1405-9940(16)30117-3. doi: 10.1016/j.acmx.2016.11.012. [Epub ahead of print] Spanish.

  1. Clinical presentation and echocardiographic characteristics of Uhl’s anomaly.

Mihos CG, Larrauri-Reyes M, Yucel E, Santana O.

Echocardiography. 2016 Dec 29. doi: 10.1111/echo.13430. [Epub ahead of print]

  1. Microduplication of 7q36.3 encompassing the SHH longrange regulator (ZRS) in a patient with triphalangeal thumbpolysyndactyly syndrome and congenital heart disease.

Liu Z, Yin N, Gong L, Tan Z, Yin B, Yang Y, Luo C.

Mol Med Rep. 2016 Dec 29. doi: 10.3892/mmr.2016.6092. [Epub ahead of print]

  1. Myocardial stress perfusion magnetic resonance: initial experience in a pediatric and young adult population using regadenoson.

Noel CV, Krishnamurthy R, Moffett B, Krishnamurthy R.

Pediatr Radiol. 2016 Dec 29. doi: 10.1007/s00247-016-3762-0. [Epub ahead of print]

  1. Down syndrome and the complexity of genome dosage imbalance.

Antonarakis SE.

Nat Rev Genet. 2016 Dec 28. doi: 10.1038/nrg.2016.154. [Epub ahead of print]

  1. Two-dimensional right ventricular strain by speckle tracking for assessment of longitudinal right ventricular function after paediatric congenital heart disease surgery.

Karsenty C, Hadeed K, Dulac Y, Semet F, Alacoque X, Breinig S, Leobon B, Acar P, Hascoet S.

Arch Cardiovasc Dis. 2016 Dec 26. pii: S1875-2136(16)30204-2. doi: 10.1016/j.acvd.2016.09.003. [Epub ahead of print]

  1. Transposition of the great arteries: Rationale for tailored preoperative management.

Séguéla PE, Roubertie F, Kreitmann B, Mauriat P, Tafer N, Jalal Z, Thambo JB.

Arch Cardiovasc Dis. 2016 Dec 23. pii: S1875-2136(16)30208-X. doi: 10.1016/j.acvd.2016.11.002. [Epub ahead of print] Review.

  1. Structural cerebral abnormalities and neurodevelopmental status in single ventricle congenital heart disease before Fontan procedure.

Knirsch W, Mayer KN, Scheer I, Tuura R, Schranz D, Hahn A, Wetterling K, Beck I, Latal B, Reich B.

Eur J Cardiothorac Surg. 2016 Dec 23. pii: ezw399. doi: 10.1093/ejcts/ezw399. [Epub ahead of print]

  1. Multi-Organ Involvement Confounding the Diagnosis of Bartonella henselae Infective Endocarditis in Children with Congenital Heart Disease.

Ouellette CP, Joshi S, Texter K, Jaggi P.

Pediatr Infect Dis J. 2016 Dec 23. doi: 10.1097/INF.0000000000001510. [Epub ahead of print]

  1. Risk for Congenital Malformation With H1N1 Influenza Vaccine: A Cohort Study With Sibling Analysis.

Ludvigsson JF, Ström P, Lundholm C, Cnattingius S, Ekbom A, Örtqvist Å, Feltelius N, Granath F, Stephansson O.

Ann Intern Med. 2016 Dec 20;165(12):848-855. doi: 10.7326/M16-0139.

  1. [Neonatal arterial ischemic stroke: Review of the current guidelines].

Saliba E, Debillon T; Recommandations accident vasculaire cérébral (AVC) néonatal., Auvin S, Baud O, Biran V, Chabernaud JL, Chabrier S, Cneude F, Cordier AG, Darmency-Stamboul V, Diependaele JF, Debillon T, Dinomais M, Durand C, Ego A, Favrais G, Gruel Y, Hertz-Pannier L, Husson B, Marret S, N’Guyen The Tich S, Perez T, Saliba E, Valentin JB, Vuillerot C.

Arch Pediatr. 2016 Dec 20. pii: S0929-693X(16)30537-1. doi: 10.1016/j.arcped.2016.11.005. [Epub ahead of print] French.

  1. Pathology and molecular mechanisms of coarctation of the aorta and its association with the ductus arteriosus.

Yokoyama U, Ichikawa Y, Minamisawa S, Ishikawa Y.

J Physiol Sci. 2016 Dec 20. doi: 10.1007/s12576-016-0512-x. [Epub ahead of print] Review.

  1. Transposition of Great Arteries with Complex Coronary Artery Variants: Time-Related Events Following Arterial Switch Operation.

Al Anani S, Fughhi I, Taqatqa A, Elzein C, Ilbawi MN, Polimenakos AC.

Pediatr Cardiol. 2016 Dec 19. [Epub ahead of print]

  1. Frequency of malformed infants in a tertiary center in Hokkaido, Japan over a period of 10 years.

Hayasaka I, Cho K, Uzuki Y, Morioka K, Akimoto T, Ishikawa S, Takei K, Yamada T, Morikawa M, Yamada T, Ariga T, Minakami H.

J Obstet Gynaecol Res. 2016 Dec 17. doi: 10.1111/jog.13229. [Epub ahead of print]

  1. Ethnic and socioeconomic variation in incidence of congenital heart defects.

Knowles RL, Ridout D, Crowe S, Bull C, Wray J, Tregay J, Franklin RC, Barron DJ, Cunningham D, Parslow RC, Brown KL.

Arch Dis Child. 2016 Dec 16. pii: archdischild-2016-311143. doi: 10.1136/archdischild-2016-311143. [Epub ahead of print]

  1. Serum C-reactive protein levels and body mass index in children and adolescents with CHD.

Goulart MR, Schuh DS, Moraes DW, Barbiero SM, Pellanda LC.

Cardiol Young. 2016 Dec 16:1-7. [Epub ahead of print]

  1. Factors Influencing Neurodevelopment after Cardiac Surgery during Infancy.

Hövels-Gürich HH.

Front Pediatr. 2016 Dec 15;4:137. doi: 10.3389/fped.2016.00137. Review.

  1. MicroRNA-Based Therapy of GATA2-Deficient Vascular Disease.

Hartmann D, Fiedler J, Sonnenschein K, Just A, Pfanne A, Zimmer K, Remke J, Foinquinos A, Butzlaff M, Schimmel K, Maegdefessel L, Hilfiker-Kleiner D, Lachmann N, Schober A, Froese N, Heineke J, Bauersachs J, Batkai S, Thum T.

Circulation. 2016 Dec 13;134(24):1973-1990.

  1. The promises and challenges of exome sequencing in familial, non-syndromic congenital heart disease.

Blue GM, Humphreys D, Szot J, Major J, Chapman G, Bosman A, Kirk EP, Sholler GF, Harvey RP, Dunwoodie SL, Winlaw DS.

Int J Cardiol. 2016 Dec 13. pii: S0167-5273(16)34400-X. doi: 10.1016/j.ijcard.2016.12.024. [Epub ahead of print]

  1. Making the Quick Diagnosis: A Case of Neonatal Shock.

Gardiner M, Ruttan TK, Kienstra AJ, Wilkinson M.

J Emerg Med. 2016 Dec 13. pii: S0736-4679(16)30991-X. doi: 10.1016/j.jemermed.2016.11.003. [Epub ahead of print]

  1. Magnetic resonance tissue phase mapping demonstrates altered left ventricular diastolic function in children with chronic kidney disease.

Gimpel C, Jung BA, Jung S, Brado J, Schwendinger D, Burkhardt B, Pohl M, Odening KE, Geiger J, Arnold R.

Pediatr Radiol. 2016 Dec 13. [Epub ahead of print]

  1. Cardiac manifestations of congenital LMNA-related muscular dystrophy in children: three case reports and recommendations for care.

Heller F, Dabaj I, Mah JK, Bergounioux J, Essid A, Bönnemann CG, Rutkowski A, Bonne G, Quijano-Roy S, Wahbi K.

Cardiol Young. 2016 Dec 12:1-7. [Epub ahead of print]

  1. Bicuspid aortic valve outcomes.

Rodrigues I, Agapito AF, de Sousa L, Oliveira JA, Branco LM, Galrinho A, Abreu J, Timóteo AT, Rosa SA, Ferreira RC.

Cardiol Young. 2016 Dec 12:1-12. [Epub ahead of print]

  1. Regional Differences in End-Diastolic Volumes between 3D Echo and CMR in HLHS Patients.

Gomez A, Oktay O, Rueckert D, Penney GP, Schnabel JA, Simpson JM, Pushparajah K.

Front Pediatr. 2016 Dec 12;4:133. doi: 10.3389/fped.2016.00133.

  1. [Report of a pedigree affected with congenital heart disease].

Ding W, Zeng F, Qin Y, Liu M.

Zhonghua Yi Xue Yi Chuan Xue Za Zhi. 2016 Dec 10;33(6):890. Chinese. No abstract available.

  1. A Prospective Evaluation of Contrast and Radiation Dose and Image Quality in Cardiac CT in Children with Complex Congenital Heart Disease using Low-concentration Iodinated Contrast Agent and Low Tube Voltage and Current.

Hou QR, Gao W, Zhong YM, Sun AM, Wang Q, Qiu HS, Wang F, Li JY, Hu L.

Br J Radiol. 2016 Dec 7:20160669. [Epub ahead of print]

  1. The Diagnostic Value of (1 → 3)-Beta-D-glucans and Galactomannan Assays in Children Suffering from Bacteremia in Pediatric Intensive Care Unit.

Zheng F, Gu Y, Zha H, Deng J, Zhang Z.

Mycopathologia. 2016 Dec 7. [Epub ahead of print]

  1. Prevalence of non-cardiovascular findings on CT angiography in children with congenital heart disease.

Malik A, Hellinger JC, Servaes S, Schwartz MC, Keller MS, Epelman M.

Pediatr Radiol. 2016 Dec 6. [Epub ahead of print]

  1. A de novo 2q35-q36.1 deletion incorporating IHH in a Chinese boy (47,XYY) with syndactyly, type III Waardenburg syndrome, and congenital heart disease.

Wang D, Ren GF, Zhang HZ, Yi CY, Peng ZJ.

Genet Mol Res. 2016 Dec 2;15(4). doi: 10.4238/gmr15049060.

  1. [Diagnosis of a case with Williams-Beuren syndrome with nephrocalcinosis using chromosome microarray analysis].

Jin SJ, Liu M, Long WJ, Luo XP.

Zhonghua Er Ke Za Zhi. 2016 Dec 2;54(12):941-945. doi: 10.3760/cma.j.issn.0578-1310.2016.12.014. Chinese.

  1. Feasibility and utility of portable ultrasound during retrieval of sick term and late preterm infants.

Browning Carmo K, Lutz T, Berry A, Kluckow M, Evans N.

Acta Paediatr. 2016 Dec;105(12):e549-e554. doi: 10.1111/apa.13589.

  1. Utility of genetic evaluation in infants with congenital heart defects admitted to the cardiac intensive care unit.

Ahrens-Nicklas RC, Khan S, Garbarini J, Woyciechowski S, D’Alessandro L, Zackai EH, Deardorff MA, Goldmuntz E.

Am J Med Genet A. 2016 Dec;170(12):3090-3097. doi: 10.1002/ajmg.a.37891.

  1. Autosomal and X chromosome structural variants are associated with congenital heart defects in Turner syndrome: The NHLBI GenTAC registry.

Prakash SK, Bondy CA, Maslen CL, Silberbach M, Lin AE, Perrone L, Limongelli G, Michelena HI, Bossone E, Citro R; BAVCon Investigators, GenTAC Registry Investigators., Lemaire SA, Body SC, Milewicz DM.

Am J Med Genet A. 2016 Dec;170(12):3157-3164. doi: 10.1002/ajmg.a.37953.

  1. [Sex differences in congenital heart disease].

Aubry P, Demian H.

Ann Cardiol Angeiol (Paris). 2016 Dec;65(6):440-445. doi: 10.1016/j.ancard.2016.10.006. French.

  1. Proportion of selected congenital heart defects attributable to recognized risk factors.

Simeone RM, Tinker SC, Gilboa SM, Agopian AJ, Oster ME, Devine OJ, Honein MA; National Birth Defects Prevention Study..

Ann Epidemiol. 2016 Dec;26(12):838-845. doi: 10.1016/j.annepidem.2016.10.003.

  1. [Progressive moderate mitral regurgitation in a children with Axenfeld-Rieger syndrome. The importance of cardiologic follow up].

Sánchez Ferrer F, Grima Murcia MD.

Arch Argent Pediatr. 2016 Dec 1;114(6):e417-e420. doi: 10.5546/aap.2016.e417. Spanish.

  1. Stem cell therapies for congenital heart disease.

Ghafarzadeh M, Namdari M, Eatemadi A.

Biomed Pharmacother. 2016 Dec;84:1163-1171. doi: 10.1016/j.biopha.2016.10.055. Review.

  1. [Prevalence of birth defects in Risaralda, 2010-2013].

Porras-Hurtado GL, León-Castañeda OM, Molano-Hurtado J, Quiceno SL, Pachajoa H, Montoya JJ.

Biomedica. 2016 Dec 1;36(4):556-563. doi: 10.7705/biomedica.v36i4.2771. Spanish.

  1. Expanding the phenotype of CRB2 mutations – A new ciliopathy syndrome?

Jaron R, Rosenfeld N, Zahdeh F, Carmi S, Beni-Adani L, Doviner V, Picard E, Segel R, Zeligson S, Carmel L, Renbaum P, Levy-Lahad E.

Clin Genet. 2016 Dec;90(6):540-544. doi: 10.1111/cge.12764.

  1. A year in pediatric cardiology and the future.

Moodie DS.

Congenit Heart Dis. 2016 Dec;11(6):535-536. doi: 10.1111/chd.12431. No abstract available.

  1. Bacteremia in Patients with Heterotaxy: A Review and Implications for Management.

Loomba RS, Geddes GC, Basel D, Benson DW, Leuthner SR, Hehir DA, Ghanayem N, Shillingford AJ.

Congenit Heart Dis. 2016 Dec;11(6):537-547. doi: 10.1111/chd.12395. Review.

  1. Perioperative Feeding Approaches in Single Ventricle Infants: A Survey of 46 Centers.

Slicker J, Sables-Baus S, Lambert LM, Peterson LE, Woodard FK, Ocampo EC; National Pediatric Cardiology-Quality Improvement Collaborative Feeding Work Group..

Congenit Heart Dis. 2016 Dec;11(6):707-715. doi: 10.1111/chd.12390.

  1. Vitamin D Kinetics and Parathyroid Gland Function in Patients with Congenital Heart Disease.

Izumi G, Inai K, Shimada E, Nakanishi T.

Congenit Heart Dis. 2016 Dec;11(6):700-706. doi: 10.1111/chd.12389.

  1. Anxiety Scores in Caregivers of Children with Hypoplastic Left Heart Syndrome.

Caris EC, Dempster N, Wernovsky G, Butz C, Neely T, Allen R, Stewart J, Miller-Tate H, Fonseca R, Texter K, Nicholson L, Cua CL.

Congenit Heart Dis. 2016 Dec;11(6):727-732. doi: 10.1111/chd.12387.

  1. Appropriate Use and Clinical Impact of Echocardiographic “Evaluation of Murmur” in Pediatric Patients.

Rose-Felker K, Kelleman MS, Campbell RM, Oster ME, Sachdeva R.

Congenit Heart Dis. 2016 Dec;11(6):721-726. doi: 10.1111/chd.12379.

  1. Prevalence and Severity of Anemia in Children Hospitalized with Acute Heart Failure.

Goldberg JF, Shah MD, Kantor PF, Rossano JW, Shaddy RE, Chiou K, Hanna J, Hagan JL, Cabrera AG, Jeewa A, Price JF.

Congenit Heart Dis. 2016 Dec;11(6):622-629. doi: 10.1111/chd.12355.

  1. School and Community Screening Shows Malawi, Africa, to Have a High Prevalence of Latent Rheumatic Heart Disease.

Sims Sanyahumbi A, Sable CA, Beaton A, Chimalizeni Y, Guffey D, Hosseinipour M, Karlsten M, Kazembe PN, Kennedy N, Minard CG, Penny DJ.

Congenit Heart Dis. 2016 Dec;11(6):615-621. doi: 10.1111/chd.12353.

  1. Echocardiographic and Surgical Correlation of Coronary Artery Patterns in Transposition of the Great Arteries.

Fundora MP, Aregullin EO, Wernovsky G, Welch EM, Muniz JC, Sasaki N, Hannan RL, Burke RP, Lopez L.

Congenit Heart Dis. 2016 Dec;11(6):570-577. doi: 10.1111/chd.12338.

  1. Three-dimensional Echocardiography of Right Ventricular Function Correlates with Severity of Pediatric Pulmonary Hypertension.

Jone PN, Patel SS, Cassidy C, Ivy DD.

Congenit Heart Dis. 2016 Dec;11(6):562-569. doi: 10.1111/chd.12337.

  1. Cerebral oxygen saturation during the first 72h after birth in infants diagnosed prenatally with congenital heart disease.

Mebius MJ, van der Laan ME, Verhagen EA, Roofthooft MT, Bos AF, Kooi EM.

Early Hum Dev. 2016 Dec;103:199-203. doi: 10.1016/j.earlhumdev.2016.10.001.

  1. Congenital heart defects in newborns with apparently isolated single gastrointestinal malformation: A retrospective study.

Schierz IA, Pinello G, Giuffrè M, La Placa S, Piro E, Corsello G.

Early Hum Dev. 2016 Dec;103:43-47. doi: 10.1016/j.earlhumdev.2016.07.005.

  1. Bilateral congenital coronary ostial obstruction: Exertional syncope in an otherwise healthy 8-year-old.

Sharma K, Thankavel P.

Echocardiography. 2016 Dec;33(12):1919-1922. doi: 10.1111/echo.13393.

  1. Tricuspid annular plane systolic excursion correlates with exercise capacity in a cohort of patients with hypoplastic left heart syndrome after Fontan operation.

Goldberg DJ, French B, Szwast AL, McBride MG, Paridon SM, Rychik J, Mercer-Rosa L.

Echocardiography. 2016 Dec;33(12):1897-1902. doi: 10.1111/echo.13348.

  1. Constitutional 560.49 kb chromosome 2p24.3 duplication including the MYCN gene identified by SNP chromosome microarray analysis in a child with multiple congenital anomalies and bilateral Wilms tumor.

Micale MA, Embrey B 4th, Macknis JK, Harper CE, Aughton DJ.

Eur J Med Genet. 2016 Dec;59(12):618-623. doi: 10.1016/j.ejmg.2016.10.010.

  1. The Visible Heart® project and free-access website ‘Atlas of Human Cardiac Anatomy’.

Iaizzo PA.

Europace. 2016 Dec;18(suppl 4):iv163-iv172. doi: 10.1093/europace/euw359.

  1. Genotype and phenotype in patients with Noonan syndrome and a RIT1 mutation.

Kouz K, Lissewski C, Spranger S, Mitter D, Riess A, Lopez-Gonzalez V, Lüttgen S, Aydin H, von Deimling F, Evers C, Hahn A, Hempel M, Issa U, Kahlert AK, Lieb A, Villavicencio-Lorini P, Ballesta-Martinez MJ, Nampoothiri S, Ovens-Raeder A, Puchmajerová A, Satanovskij R, Seidel H, Unkelbach S, Zabel B, Kutsche K, Zenker M.

Genet Med. 2016 Dec;18(12):1226-1234. doi: 10.1038/gim.2016.32.

  1. Defining the Risk and Associated Morbidity and Mortality of Severe Respiratory Syncytial Virus Infection Among Preterm Infants Without Chronic Lung Disease or Congenital Heart Disease.

Figueras-Aloy J, Manzoni P, Paes B, Simões EA, Bont L, Checchia PA, Fauroux B, Carbonell-Estrany X.

Infect Dis Ther. 2016 Dec;5(4):417-452. Review.


  1. Implementation of a Quality Improvement Bundle Improves Echocardiographic Imaging after Congenital Heart Surgery in Children.

Parthiban A, Levine JC, Nathan M, Marshall JA, Shirali GS, Simon SD, Colan SD, Newburger JW, Raghuveer G.

J Am Soc Echocardiogr. 2016 Dec;29(12):1163-1170.e3. doi: 10.1016/j.echo.2016.09.002.

  1. Intracardiac Air Bubbles Originating From Pneumatosis Intestinalis in an Infant With Cyanotic Congenital Heart Disease.

Nishibe S, Kodama M, Morita Y, Sakai D, Yoshikawa H.

J Cardiothorac Vasc Anesth. 2016 Dec;30(6):1632-1635. doi: 10.1053/j.jvca.2015.12.028. No abstract available.

  1. Integration of Computed Tomography and Three-Dimensional Echocardiography for Hybrid Three-Dimensional Printing in Congenital Heart Disease.

Gosnell J, Pietila T, Samuel BP, Kurup HK, Haw MP, Vettukattil JJ.

J Digit Imaging. 2016 Dec;29(6):665-669.

  1. Early-Term Birth in Single-Ventricle Congenital Heart Disease After the Fontan Procedure: Neurodevelopmental and Psychiatric Outcomes.

Calderon J, Stopp C, Wypij D, DeMaso DR, Rivkin M, Newburger JW, Bellinger DC.

J Pediatr. 2016 Dec;179:96-103. doi: 10.1016/j.jpeds.2016.08.084.

  1. Congenital Anorectal Malformation Severity Does Not Predict Severity of Congenital Heart Defects.

Jonker JE, Liem ET, Elzenga NJ, Molenbuur B, Trzpis M, Broens PM.

J Pediatr. 2016 Dec;179:150-153.e1. doi: 10.1016/j.jpeds.2016.08.047.

  1. Pulse oximetry screening for critical congenital heart disease in planned out of hospital births and the incidence of critical congenital heart disease in the Plain community.

Miller KK, Vig KS, Goetz EM, Spicer G, Yang AJ, Hokanson JS.

J Perinatol. 2016 Dec;36(12):1088-1091. doi: 10.1038/jp.2016.135.

  1. Impact of Telemedicine in the Screening for Congenital Heart Disease in a Center from Northeast Brazil.

de Araújo JS, Regis CT, Gomes RG, Mourato FA, Mattos SD.

J Trop Pediatr. 2016 Dec;62(6):471-476.

  1. Application of array-comparative genomic hybridization in tetralogy of Fallot.

Liu L, Wang HD, Cui CY, Wu D, Li T, Fan TB, Peng BT, Zhang LZ, Wang CZ.

Medicine (Baltimore). 2016 Dec;95(49):e5552.

  1. Usefulness of Lung Ultrasound in Neonatal Congenital Heart Disease (LUSNEHDI): Lung Ultrasound to Assess Pulmonary Overflow in Neonatal Congenital Heart Disease.

Rodríguez-Fanjul J, Llop AS, Balaguer M, Bautista-Rodriguez C, Hernando JM, Jordan I.

Pediatr Cardiol. 2016 Dec;37(8):1482-1487.

  1. Diagnosis and Diagnostic Modalities in Pediatric Patients with Elevated Troponin.

Harris TH, Gossett JG.

Pediatr Cardiol. 2016 Dec;37(8):1469-1474.

  1. Incidence of Respiratory Disease During the First Two Years of Life in Children with Hemodynamically Significant Congenital Heart Disease in Italy: A Retrospective Study.

Pongiglione G, Possidoni A, di Luzio Paparatti U, Costanzo AM, Gualberti G, Bonvicini M, Rimini A, Agnoletti G, Calabrò MP, Pozzi M, Tumbarello R, Salice P, Fiorini P, Russo MG, Milanesi O.

Pediatr Cardiol. 2016 Dec;37(8):1581-1589.

  1. Association Between Single Nucleotide Polymorphisms in NFATC1 Signaling Pathway Genes and Susceptibility to Congenital Heart Disease in the Chinese Population.

Wang F, Wang H, Wang L, Zhou S, Chang M, Zhou J, Dou Y, Wang Y, Shi X.

Pediatr Cardiol. 2016 Dec;37(8):1548-1561.

  1. Differences by Altitude in the Frequency of Congenital Heart Defects in Colombia.

García A, Moreno K, Ronderos M, Sandoval N, Caicedo M, Dennis RJ.

Pediatr Cardiol. 2016 Dec;37(8):1507-1515.

  1. Evaluation of Impedance Cardiography for Measurement of Stroke Volume in Congenital Heart Disease.

Ebrahim M, Hegde S, Printz B, Abcede M, Proudfoot JA, Davis C.

Pediatr Cardiol. 2016 Dec;37(8):1453-1457.

  1. Association of A Dilated Coronary Sinus in the Fetus with Actual and Apparent Coarctation of the Aorta and Diminutive Left Heart Structures.

Ramaswamy P, Rafii D, Osmolovsky M, Agarwal A, Amirtharaj C.

Pediatr Cardiol. 2016 Dec;37(8):1562-1568.

  1. Cardiac Manifestations and Associations with Gene Mutations in Patients Diagnosed with RASopathies.

Jhang WK, Choi JH, Lee BH, Kim GH, Yoo HW.

Pediatr Cardiol. 2016 Dec;37(8):1539-1547.

  1. The Use of Speckle Tracking Echocardiography for Early Detection of Myocardial Dysfunction in Patients with Duchenne Muscular Dystrophy.

Taqatqa A, Bokowski J, Al-Kubaisi M, Khalil A, Miranda C, Alaksham H, Fughhi I, Kenny D, Diab KA.

Pediatr Cardiol. 2016 Dec;37(8):1422-1428.

  1. Radiation Dose and Image Quality in Pediatric Cardiac Computed Tomography: A Comparison Between Sequential and Third-Generation Dual-Source High-Pitch Spiral Techniques.

Koh H, Ong CC, Choo YS, Liang CR, Tan GH, Lim TC, Quek SC, Sriram S, Teo LL.

Pediatr Cardiol. 2016 Dec;37(8):1397-1403.

  1. Biomarkers of Myocardial Injury in Congenital Heart Disease: More Questions than Answers.

Chen CA.

Pediatr Neonatol. 2016 Dec;57(6):451-452. doi: 10.1016/j.pedneo.2016.10.001. No abstract available.

  1. Myocardial Injury Biomarkers in Newborns with Congenital Heart Disease.

Neves AL, Cabral M, Leite-Moreira A, Monterroso J, Ramalho C, Guimarães H, Barros H, Guimarães JT, Henriques-Coelho T, Areias JC.

Pediatr Neonatol. 2016 Dec;57(6):488-495. doi: 10.1016/j.pedneo.2015.11.004.

  1. Cardiopulmonary exercise performance is reduced in congenital diaphragmatic hernia survivors.

Bojanić K, Grizelj R, Dilber D, Šarić D, Vuković J, Pianosi PT, Driscoll DJ, Weingarten TN, Pritišanac E, Schroeder DR, Sprung J.

Pediatr Pulmonol. 2016 Dec;51(12):1320-1329. doi: 10.1002/ppul.23481.

  1. Short-length ligamentum arteriosum as a cause of congenital narrowing of the left main stem bronchus.

Sacco O, Santoro F, Ribera E, Magnano GM, Rossi GA.

Pediatr Pulmonol. 2016 Dec;51(12):1356-1361. doi: 10.1002/ppul.23460.

  1. Combined blood pool and extracellular contrast agents for pediatric and young adult cardiovascular magnetic resonance imaging.

Johnson JT, Robinson JD, Deng J, Rigsby CK.

Pediatr Radiol. 2016 Dec;46(13):1822-1830.

  1. Impact of MYH6 variants in hypoplastic left heart syndrome.

Tomita-Mitchell A, Stamm KD, Mahnke DK, Kim MS, Hidestrand PM, Liang HL, Goetsch MA, Hidestrand M, Simpson P, Pelech AN, Tweddell JS, Benson DW, Lough JW, Mitchell ME.

Physiol Genomics. 2016 Dec 1;48(12):912-921. doi: 10.1152/physiolgenomics.00091.2016.

  1. High Mid-Flow to Vital Capacity Ratio and the Response to Exercise in Children With Congenital Heart Disease.

Vilozni D, Alcaneses-Ofek MR, Reuveny R, Rosenblum O, Inbar O, Katz U, Ziv-Baran T, Dubnov-Raz G.

Respir Care. 2016 Dec;61(12):1629-1635.

  1. Pericarditis in a Child with an Intrapericardial Bronchogenic Cyst.

Attmann T, Grothusen C, Rickers C, Dütschke P, Kramer HH, Scheewe J.

Thorac Cardiovasc Surg Rep. 2016 Dec;5(1):47-49. doi: 10.1055/s-0035-1571139.

  1. Regenerative Medicine Strategies for Hypoplastic Left Heart Syndrome.

Chery J, Wong J, Huang S, Wang S, Si MS.

Tissue Eng Part B Rev. 2016 Dec;22(6):459-469.

  1. Editorial: Biomarkers in neonatology.

Meyer S, Zemlin M, Poryo M.

Early Hum Dev. 2016 Dec 29. pii: S0378-3782(16)30563-1. doi: 10.1016/j.earlhumdev.2016.12.001. [Epub ahead of print] No abstract available.

  1. D-Transposition of the Great Arteries and ductal dependent pulmonary circulation.

Ghimire LV.

Early Hum Dev. 2016 Dec 29. pii: S0378-3782(16)30547-3. doi: 10.1016/j.earlhumdev.2016.12.010. [Epub ahead of print] No abstract available.

  1. Transcriptional Analysis of Intravenous Immunoglobulin Resistance in Kawasaki Disease Using an Induced Pluripotent Stem Cell Disease Model.

Ikeda K, Mizoro Y, Ameku T, Nomiya Y, Mae SI, Matsui S, Kuchitsu Y, Suzuki C, Hamaoka-Okamoto A, Yahata T, Sone M, Okita K, Watanabe A, Osafune K, Hamaoka K.

Circ J. 2016 Dec 22;81(1):110-118. doi: 10.1253/circj.CJ-16-0541.

  1. Development of the atrial septum in relation to postnatal anatomy and interatrial communications.

Jensen B, Spicer DE, Sheppard MN, Anderson RH.

Heart. 2016 Dec 21. pii: heartjnl-2016-310660. doi: 10.1136/heartjnl-2016-310660. [Epub ahead of print] Review.

  1. Psychosocial impact on families with an infant with a hypoplastic left heart syndrome during and after the interstage monitoring period – a prospective mixed-method study.

Stoffel G, Spirig R, Stiasny B, Bernet V, Dave H, Knirsch W.

J Clin Nurs. 2016 Dec 21. doi: 10.1111/jocn.13694. [Epub ahead of print]

  1. Treatment initiation in paediatric pulmonary hypertension: insights from a multinational registry.

Humpl T, Berger RM, Austin ED, Fasnacht Boillat MS, Bonnet D, Ivy DD, Zuk M, Beghetti M, Schulze-Neick I.

Cardiol Young. 2016 Dec 20:1-10. [Epub ahead of print]

  1. Treatable mitochondrial diseases: cofactor metabolism and beyond.

Distelmaier F, Haack TB, Wortmann SB, Mayr JA, Prokisch H.

Brain. 2016 Dec 19. pii: aww303. [Epub ahead of print] No abstract available.

  1. Assessing Myocardial Function in Infants with Pulmonary Hypertension: The Role of Tissue Doppler Imaging and Tricuspid Annular Plane Systolic Excursion.

Richardson C, Amirtharaj C, Gruber D, Hayes DA.

Pediatr Cardiol. 2016 Dec 19. [Epub ahead of print]

  1. Factors Influencing Neurodevelopment after Cardiac Surgery during Infancy.

Hövels-Gürich HH.

Front Pediatr. 2016 Dec 15;4:137. doi: 10.3389/fped.2016.00137. Review.

  1. Ventricular-vascular dynamics in pediatric patients with heart failure and preserved ejection fraction.

Masutani S, Kuwata S, Kurishima C, Iwamoto Y, Saiki H, Sugimoto M, Ishido H, Senzaki H.

Int J Cardiol. 2016 Dec 15;225:306-312. doi: 10.1016/j.ijcard.2016.09.118.

  1. Former Very Preterm Infants Show an Unfavorable Cardiovascular Risk Profile at a Preschool Age.

Posod A, Odri Komazec I, Kager K, Pupp Peglow U, Griesmaier E, Schermer E, Würtinger P, Baumgartner D, Kiechl-Kohlendorfer U.

PLoS One. 2016 Dec 13;11(12):e0168162. doi: 10.1371/journal.pone.0168162.

  1. Discrepancies Between Clinical Diagnoses and Autopsy Findings in Critically Ill Children: A Prospective Study.

Carlotti AP, Bachette LG, Carmona F, Manso PH, Vicente WV, Ramalho FS.

Am J Clin Pathol. 2016 Dec 10. pii: aqw187. [Epub ahead of print]

  1. Tricuspid annular plane systolic excursion as a marker of right ventricular dysfunction in pediatric patients with dilated cardiomyopathy.

McLaughlin ES, Travers C, Border WL, Deshpande S, Sachdeva R.

Echocardiography. 2016 Dec 9. doi: 10.1111/echo.13416. [Epub ahead of print]

  1. Myocardial dysfunction in children with intrauterine growth restriction: an echocardiographic study.

Niewiadomska-Jarosik K, Zamojska J, Zamecznik A, Wosiak A, Jarosik P, Stańczyk J.

Cardiovasc J Afr. 2016 Dec 7;27:1-4. doi: 10.5830/CVJA-2016-053. [Epub ahead of print]

  1. Comparing the Efficacy of Tadalafil Versus Placebo on Pulmonary Artery Systolic Pressure and Right Ventricular Function in Patients with Beta-Thalassaemia Intermedia.

Jalalian R, Moghadamnia AA, Tamaddoni A, Khafri S, Iranian M.

Heart Lung Circ. 2016 Dec 2. pii: S1443-9506(16)31695-X. doi: 10.1016/j.hlc.2016.11.003. [Epub ahead of print]

  1. Usefulness of Serial N-terminal Pro-B-type Natriuretic Peptide Measurements to Predict Cardiac Death in Acute and Chronic Dilated Cardiomyopathy in Children.

den Boer SL, Rizopoulos D, du Marchie Sarvaas GJ, Backx AP, Ten Harkel AD, van Iperen GG, Rammeloo LA, Tanke RB, Boersma E, Helbing WA, Dalinghaus M.

Am J Cardiol. 2016 Dec 1;118(11):1723-1729. doi: 10.1016/j.amjcard.2016.08.053.

  1. Factors Associated With Resource Utilization and Coronary Artery Dilation in Refractory Kawasaki Disease (from the Pediatric Health Information System Database).

Lo JY, Minich LL, Tani LY, Wilkes J, Ding Q, Menon SC.

Am J Cardiol. 2016 Dec 1;118(11):1636-1640. doi: 10.1016/j.amjcard.2016.08.039.

  1. Evidence for troponin C (TNNC1) as a gene for autosomal recessive restrictive cardiomyopathy with fatal outcome in infancy.

Ploski R, Rydzanicz M, Ksiazczyk TM, Franaszczyk M, Pollak A, Kosinska J, Michalak E, Stawinski P, Ziolkowska L, Bilinska ZT, Werner B.

Am J Med Genet A. 2016 Dec;170(12):3241-3248. doi: 10.1002/ajmg.a.37860.

  1. Experts’ recommendations for the management of cardiogenic shock in children.

Brissaud O, Botte A, Cambonie G, Dauger S, de Saint Blanquat L, Durand P, Gournay V, Guillet E, Laux D, Leclerc F, Mauriat P, Boulain T, Kuteifan K.

Ann Intensive Care. 2016 Dec;6(1):14. doi: 10.1186/s13613-016-0111-2.

  1. Brain diffusion changes in Eisenmenger syndrome.

Dogan F, Sen Dokumaci D, Yildirim A, Bozdogan E, Boyaci FN, Koca B, Karakas E.

Br J Radiol. 2016 Dec;89(1068):20151007.

  1. Myocardial Dimensions in Children With Hypertrophic Cardiomyopathy: A Comparison Between Echocardiography and Cardiac Magnetic Resonance Imaging.

Windram JD, Dragelescu A, Benson L, Forsey J, Shariat M, Yoo SJ, Mertens L, Wong D, Grosse-Wortmann L.

Can J Cardiol. 2016 Dec;32(12):1507-1512. doi: 10.1016/j.cjca.2016.06.014.

  1. Cardiovascular alterations do exist in children with stage-2 chronic kidney disease.

Taşdemir M, Eroğlu AG, Canpolat N, Konukoğlu D, Ağbaş A, Sevim MD, Çalışkan S, Sever L.

Clin Exp Nephrol. 2016 Dec;20(6):926-933.

  1. Foramen ovale (FO) – The underestimated sibling of ductus arteriosus (DA): Relevance during neonatal transition.

Baik N, Urlesberger B, Schwaberger B, Schmölzer GM, Köstenberger M, Avian A, Pichler G.

Early Hum Dev. 2016 Dec;103:137-140. doi: 10.1016/j.earlhumdev.2016.08.009. No abstract available.

  1. Characterizing cerebral and locomotor muscle oxygenation to incremental ramp exercise in healthy children: relationship with pulmonary gas exchange.

Vandekerckhove K, Coomans I, Moerman A, De Wolf D, Boone J.

Eur J Appl Physiol. 2016 Dec;116(11-12):2345-2355.

  1. Congenital heart defects in Noonan syndrome and RIT1 mutation.

Calcagni G, Baban A, Lepri FR, Marino B, Tartaglia M, Digilio MC.

Genet Med. 2016 Dec;18(12):1320. doi: 10.1038/gim.2016.137. No abstract available.

  1. Echocardiographic diagnosis of double-chambered left ventricle.

Bilici M, Demir F, Akın A, Güzel A, Akdeniz O, Tan İ.

J Echocardiogr. 2016 Dec;14(4):176-178.

  1. Anomalous mitral and tricuspid arcade in a patient with stenotic double-orifice tricuspid valve.

Nitta M, Takigiku K, Yasukochi S, Takei K, Tazawa S, Utsumi M, Okamura T.

J Echocardiogr. 2016 Dec;14(4):173-175. No abstract available.

  1. Cardiac dysfunction in children and young adults with heart transplantation: A comprehensive echocardiography study.

Chinali M, Esposito C, Grutter G, Iacobelli R, Toscano A, D’Asaro MG, Pasqua AD, Brancaccio G, Parisi F, Drago F, Rinelli G.

J Heart Lung Transplant. 2016 Dec 1. pii: S1053-2498(16)30436-3. doi: 10.1016/j.healun.2016.11.007. [Epub ahead of print]

  1. Pediatric quality of life in long-term survivors of childhood cancer treated with anthracyclines.

Ryerson AB, Wasilewski-Masker K, Border WL, Goodman M, Meacham L, Austin H, Marchak JG, Mertens AC.

Pediatr Blood Cancer. 2016 Dec;63(12):2205-2211. doi: 10.1002/pbc.26149.

  1. Six-Minute Walk Test as a Predictor for Outcome in Children with Dilated Cardiomyopathy and Chronic Stable Heart Failure.

den Boer SL, Flipse DH, van der Meulen MH, Backx AP, du Marchie Sarvaas GJ, Ten Harkel AD, van Iperen GG, Rammeloo LA, Tanke RB, Helbing WA, Takken T, Dalinghaus M.

Pediatr Cardiol. 2016 Dec 1. [Epub ahead of print]

  1. Erratum to: A Literature Review of the Pharmacokinetics and Pharmacodynamics of Dobutamine in Neonates.

Mahoney L, Shah G, Crook D, Rojas-Anaya H, Rabe H.

Pediatr Cardiol. 2016 Dec 1. [Epub ahead of print] No abstract available.

  1. Right Heart Function of Fetuses and Infants with Large Ventricular Septal Defect: A Longitudinal Case-Control Study.

Chen J, Xie L, Dai L, Yu L, Liu L, Zhou Y, Wu G, Qin F, Liu H.

Pediatr Cardiol. 2016 Dec;37(8):1488-1497.

  1. Doppler Flow Pattern and Arterial Stiffness in Patients with Aortic Coarctation.

Cordeiro S, Gomes J, Mendes IC, Martins DS, Sousa A, Anjos R.

Pediatr Cardiol. 2016 Dec;37(8):1465-1468.

  1. Home Monitoring Program Reduces Mortality in High-Risk Sociodemographic Single-Ventricle Patients.

Castellanos DA, Herrington C, Adler S, Haas K, Ram Kumar S, Kung GC.

Pediatr Cardiol. 2016 Dec;37(8):1575-1580.

  1. Myocardial Layers Specific Strain Analysis for the Acute Phase of Infant Kawasaki Disease.

Eun LY, Kim JH, Jung JW, Choi JY.

Pediatr Cardiol. 2016 Dec;37(8):1404-1408.

  1. Characteristics of Hospitalizations for the Glenn Procedure in Those With Isomerism Compared to Those Without.

Loomba RS, Kouretas PC, Anderson RH.

Pediatr Cardiol. 2016 Dec;37(8):1409-1415.

  1. Fatal cytomegalovirus infection following cardiac surgery in a neonate with asplenia.

Hasegawa T, Oshima Y, Yamamoto M, Sato Y.

Pediatr Int. 2016 Dec;58(12):1367-1368. doi: 10.1111/ped.13078. No abstract available.

  1. Severe Hemolysis and Pulmonary Hypertension in a Neonate With Upshaw-Schulman Syndrome.

Tsujii N, Shiraishi I, Kokame K, Shima M, Fujimura Y, Takahashi Y, Matsumoto M.

Pediatrics. 2016 Dec;138(6). pii: e20161565.

  1. A case of tracheal bronchus associated with right aortic arch and partial anomalous pulmonary venous connection.

Doğan V, Ertuğrul İ, Taşcı Yıldız Y, Örün UA, Karademir S.

Turk Kardiyol Dern Ars. 2016 Dec;44(8):703-705. doi: 10.5543/tkda.2016.83903.

  1. Multiple pericardial abscesses in a child with known chronic granulomatous disease.

Kalyoncu AU, Habibi HA, Aslan M, Alis D, Aygun DF, Camcioglu Y, Adaletli I.

Ann Pediatr Cardiol. 2016 Sep-Dec;9(3):272-3. doi: 10.4103/0974-2069.189120. No abstract available.

  1. Acute poststreptococcal glomerulonephritis and acute rheumatic fever: An uncommon coincidence.

Vilija C, Paulius K, Karolis A, Augustina J.

Ann Pediatr Cardiol. 2016 Sep-Dec;9(3):268-9. doi: 10.4103/0974-2069.189115. No abstract available.

  1. Acquired Fontan paradox in isolated right ventricular cardiomyopathy.

Saran M, Sivasubramonian S, Abhilash SP, Tharakan JA.

Ann Pediatr Cardiol. 2016 Sep-Dec;9(3):251-3. doi: 10.4103/0974-2069.189117.

  1. Thromboembolism and anticoagulation after Fontan surgery.

Viswanathan S.

Ann Pediatr Cardiol. 2016 Sep-Dec;9(3):236-40. doi: 10.4103/0974-2069.189109. Review.

  1. Profile and risk factors for congenital heart defects: A study in a tertiary care hospital.

Abqari S, Gupta A, Shahab T, Rabbani MU, Ali SM, Firdaus U.

Ann Pediatr Cardiol. 2016 Sep-Dec;9(3):216-21. doi: 10.4103/0974-2069.189119.

  1. Pattern of congenital heart disease in a developing country tertiary care center: Factors associated with delayed diagnosis.

Rashid U, Qureshi AU, Hyder SN, Sadiq M.

Ann Pediatr Cardiol. 2016 Sep-Dec;9(3):210-5. doi: 10.4103/0974-2069.189125.

  1. Birth prevalence of congenital heart disease: A cross-sectional observational study from North India.

Saxena A, Mehta A, Sharma M, Salhan S, Kalaivani M, Ramakrishnan S, Juneja R.

Ann Pediatr Cardiol. 2016 Sep-Dec;9(3):205-9. doi: 10.4103/0974-2069.189122.

  1. Congenital heart disease profile: Four perspectives.

Kumar RK.

Ann Pediatr Cardiol. 2016 Sep-Dec;9(3):203-4. doi: 10.4103/0974-2069.189110. No abstract available.

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