Congenital EP Featured Articles of April 2017

 

Pediatric Cardiac and EP Reviews of April 2017 Manuscripts

 

Pacemaker and Defibrillator Implantation in Patients with Transposition of the Great Arteries

Grubb AF, Shah G, Aziz PF, Krasuski RA

The Journal of Innovations in Cardiac Rhythm Management, 8 (2017), 2658-2664

 

Take Home Points:

 

  • Cardiac implantable device implantation in TGA substrates presents unique challenges both at implant and throughout follow-up.
  • This study does not apply to arterial switch patients.
  • Despite differences in indications for implantable devices and age at device implant between d- and l-TGA patients, both TGA groups unfortunately appear to have similar progressions in the development of heart failure with its associated morbidity and mortality.
  • Utilization of current published guidelines for primary prevention ICD implantation has shortcomings when applied to TGA substrates and would benefit from updated evidence and experience with ICDs in TGA patients to guide and individualize this treatment option in this unique patient population.

 

chang-philip-1780821827Commentary from Dr. Philip Chang (Los Angeles), section editor of Congenital Heart Surgery Journal Watch: Article summary:

Grubb et al presented a retrospective review of their single-center experience with cardiac implantable electronic devices (CIEDs) in d- and l-TGA patients with systemic RVs.  The study looked at all TGA patients with CIEDs cared for at their institution over an 18-year period.  All patients had systemic right ventricles and all patients had undergone biventricular repair approaches, with single-ventricle variants and those with repairs to restore systemic morphologic left ventricular circulation excluded from analysis.  In total, 63 patients were identified (34 d-TGA, 29 l-TGA).  The authors performed detailed chart review for each subject to determine initial device implant timing/age, development of heart failure, and variables associated with ICD follow-up including defibrillation thresholds and shocks.

 

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EP3

 

EP4

 

Study limitations include single-center inclusivity and retrospective design.  The authors did not provide more in-depth detail pertaining to implant approaches, CIED pocket site (left vs. right chest for transvenous devices, which could have implications in ICD defibrillation function), or CIED-related complications.   Several patients reportedly had CRT devices, but no detail was provided in terms of the indications for CRT or implant approach.

 

Reviewer perspective and thoughts for pediatric/CHD EP:

While it is commonly recognized and even expected that TGA patients will require CIED implantation, primarily for pacing indications, it has been quite some time since the topic of CIED utilization and benefit in TGA patients has been studied.  As such, the authors are commended for reviewing their data and presenting their experience, which is quite valuable for our community.  The study definitely highlights the importance of considering multi-institutional pooling of data and experience.  Furthermore, while the growth of the atrial switched d-TGA population is diminishing, there is still a considerable population of these patients, along with a growing number of adult l-TGA patients that should motivate greater awareness of the use of CIEDs in these very unique ACHD subgroups.

There is little debate in the indications for and benefits of pacing in TGA substrates and the authors’ findings of more predominant pacing for sinus node disease in d-TGA and AV block in l-TGA isn’t novel.  It remains unclear as to the role of CRT for wide QRS, heart block, or dyssynchrony in TGA, and more specifically its feasibility and effectiveness in primary systemic RV resynchronization.  With the advent of and interest in His-bundle pacing, the feasibility of this method of pacing in d- vs. l-TGA would be interesting to explore in terms of technical considerations and long-term benefit.  The finding that no primary prevention ICD patients received appropriate shocks was interesting, but not entirely surprising.  This certainly raises the question of the appropriateness of applying standard guidelines for ICD implantation to the TGA population and while some published data exists for risk stratification in d-TGA, greater evidence is needed to guide ICD therapy, particularly for primary prevention indications, in these patients.   Finally, with the subcutaneous ICD as a contemporary implant option, it will also be interesting to see how the balance between implant indication and “ease” of device implant will change.

 

It is sobering to see that both d- and l-TGA patients with systemic RVs progress in very similar fashions to develop heart failure and its associated clinical sequelae and mortality.  This shared finding between d- and l-TGA patients highlights the unique and highly complex substrate in which CIEDs are being applied, as well as the multifactorial process involved in the near-universal fate of the systemic RV in these TGA patients.

 

 

 

Lone Pediatric Atrial Fibrillation in the United States: Analysis of Over 1500 Cases

El-Assaad I, Al-Kindi SG, Saarel EV, Aziz PF

Pediatr Cardiol April 2017 (DOI: 10.1007/s00246-017-1608-7)

 

Take home points:

  • Lone atrial fibrillation is a rare arrhythmia condition among pediatric patients.
  • Increasing age and obesity appear associated with higher incidence of lone atrial fibrillation though evidence indicating direct causality is lacking.
  • While complications such as thromboembolic events are rare in pediatric lone atrial fibrillation, their incidence is not ignorable. However, treatment recommendations and practice patterns are inconsistent.

 

Commentary from Dr. Philip Chang (Los Angeles, CA) section editor of Congenital Heart Surgery Journal WatchThere has been much recent interest in atrial fibrillation in pediatric and young adult patients, particularly as it pertains to risk factors for its development as well as treatment in patients with significant or symptomatic recurrences and in the setting of CHD.  Despite this interest, studies with large sample sizes are lacking in order to better understand its incidence, clinical sequelae, treatment options and response, and long-term outcomes, particularly in otherwise healthy individuals.  The current study from El-Assaad et al provides a descriptive analysis of the largest cohort of young patients with lone atrial fibrillation in the United States.

The authors sought to evaluate risk factors and short-term outcomes of pediatric lone atrial fibrillation, simply defined as atrial fibrillation occurring in the absence of cardiac and systemic diseases.  They utilized a privately managed national healthcare database, Explorys, from which they were able to query and determine an incidence of pediatric lone atrial fibrillation, other variables that may be associated with its diagnosis, and clinical sequelae over a 17-year period (1999-2016).  The database was created from de-identified data provided by 360 hospitals in all 50 US states and over 300,000 providers.

Results of querying the Explorys database yielded nearly 8 million children, with 1910 children linked to an atrial fibrillation diagnosis.  Of these patients, 1750 children met the definition of lone atrial fibrillation.  This resulted in a calculated pediatric lone AF incidence of 7.5 in 100,000 persons at risk.  Nearly 10% of these patients also had concomitant diagnosis of SVT but further characterization of this could not be determined from the dataset variables.  The authors found relatively low percentages of antiarrhythmic use (5%), aspirin or warfarin for anticoagulation (7% and 5%, respectively), and electrical cardioversion (3%).  Older patients tended to be prescribed aspirin or warfarin more frequently compared to younger aged cohorts.

EP5

Multivariate analysis showed increasing age, male gender, and obesity to be associated with risk of lone AF.  Increasing age was also associated with increased risk of AF recurrence within 1 month of initial episode.  Interestingly, among patients 15-19 years of age, nearly 20% experienced AF recurrence.  The authors also found that 2% of patients experienced a stroke within 1 year of lone AF diagnosis.  Following exclusion of other concomitant diagnoses including sepsis, hypertension, hypertensive crisis, drug abuse, stimulant use, cancer, renal impairment, sleep apena, respiratory failure, and bone marrow transplant, a total of 1580 patient remained.  Among these patients, a male predominance was noted (61%), and most episodes of lone AF occurred in the 15-19 years age group (58%).  Those in the oldest age cohort had recurrence rates spanning 15-22% from 1 month up to 1 year after the initial event.  In this lone AF subset, 1% of patients had a stroke within 1 year of lone AF diagnosis.

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EP7

Study limitations include limited available variables in the database and inability to control for the quality and accuracy of the data provided.  For example, the authors noted that they were unable to review ECGs to confirm AF diagnosis or echocardiograms to evaluate chamber size and that some patients could have been misclassified as having AF in the first place.  Recurrence rates could have been overestimated if a single AF diagnosis reappeared on more than 1 occasion.  They also noted that the dataset itself has not been directly validated for pediatric studies, though adult studies have demonstrated its validity.  It is unclear as to why nearly 10% of the originally classified “lone AF” patients were excluded given other systemic diseases to come to the subgroup of 1580 patients that were further analyzed for lone AF incidence and recurrence.  By the authors’ definition, these patients would not have met a pure lone AF diagnosis given these concomitant conditions, many of which can certainly increase the risk of arrhythmias (AF being one of them).  If the subgroup patients were used to calculate incidence, the lone AF incidence would be even lower.  Finally, the authors did not have further details relating to concomitant SVT in the 10% of patients identified and there was no long-term data regarding treatment efficacy (antiarrhythmics or catheter ablation), prognosis, and outcomes.

 

Reviewer perspective and thoughts for pediatric/CHD EP:

This study is an excellent example of the use of large scale databases to evaluate otherwise rare conditions.  As such, it provides a faster, and perhaps more accurate, assessment of pediatric lone AF incidence, as well as general approaches to management, complications, and recurrence.  The study is obviously limited by the quality and accuracy of data entered into the database itself.

This study’s finding of the association between lone AF and obesity, male gender, and increasing age is helpful and consistent with previous publications noting these potential associations that had substantially smaller patient cohorts.  The association with obesity is likely multifactorial.  Obesity is frequently associated with other cardiovascular conditions including hypertension, diastolic dysfunction, and left atrial distension and pressure loading, though interestingly, the association between obesity and lone AF was apparently made in the study after excluding for these other systemic diseases.  It is possible that these other systemic diseases were under-reported in the dataset and echo data was not available to assess for evidence of diastolic dysfunction or left atrial abnormalities.  Obesity is also frequently associated with obstructive sleep apnea, which is increasingly recognized as having a strong association with arrhythmia risk including AF (though again, the study largely excluded patients with concomitant diagnoses of AF and sleep apnea).  The authors noted familial lone AF as a possible important contributor, and the genetics of AF remains an important area of ongoing research.

Several important findings from this study that are important for the pediatric EP community to be aware of is the low incidence of pediatric lone AF that is found and which is likely more reflective of its true incidence in the general pediatric population.  Furthermore, based on the study’s results, there appears to be a fairly high recurrence rate (up to 20% in the oldest age cohort) as well as a low but very concerning incidence of stroke.  Both of these appeared to be present in the setting of low utilization of antiarrhythmic agents and anticoagulation.  In fact, the stroke incidence that was noted in this study corresponded to an equivalent CHADS2VASc score that would qualify patients to receive anticoagulation with either warfarin or NOACs.  This study’s findings show that pediatric lone AF may not be as benign of a condition as some may think and that these patients require aggressive and close follow-up to address or prevent recurrences and to reduce AF-related complications.

CHD EP April 2017

 

  1. Atrial undersensing secondary to quiet timer blanking in pediatric and congenital heart disease patients.

von Alvensleben JC, Schaffer M, Brateng C, Collins KK.

Pacing Clin Electrophysiol. 2017 Apr 24. doi: 10.1111/pace.13101. [Epub ahead of print]

PMID: 28436549

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  1. Percutaneous Ventricular Assist Device for Circulatory Support During Ablation of Atrial Tachycardias in Patients With Fontan Circulation.

Hendriks A, De Vries L, Witsenburg M, Yap SC, Van Mieghem N, Szili-Torok T.

Rev Esp Cardiol (Engl Ed). 2017 Apr 18. pii: S1885-5857(17)30170-6. doi: 10.1016/j.rec.2017.03.010. [Epub ahead of print] English, Spanish. No abstract available.

PMID: 28431884

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  1. The electrical heart axis and ST events in fetal monitoring: A post-hoc analysis following a multicentre randomised controlled trial.

Vullings R, Verdurmen KMJ, Hulsenboom ADJ, Scheffer S, de Lau H, Kwee A, Wijn PFF, Amer-Wåhlin I, van Laar JOEH, Oei SG.

PLoS One. 2017 Apr 14;12(4):e0175823. doi: 10.1371/journal.pone.0175823. eCollection 2017.

PMID: 28410419 Free PMC Article

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  1. Sudden cardiac death in adults with congenital heart disease: does QRS-complex fragmentation discriminate in structurally abnormal hearts?

Vehmeijer JT, Koyak Z, Bokma JP, Budts W, Harris L, Mulder BJ, de Groot JR.

Europace. 2017 Apr 10. doi: 10.1093/europace/eux044. [Epub ahead of print]

PMID: 28402450

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  1. Post mortem therapy from a subcutaneous ICD: What is the mechanism?

Wiles BM, Fitzsimmons SJ, Roberts PR.

Pacing Clin Electrophysiol. 2017 Apr 4. doi: 10.1111/pace.13089. [Epub ahead of print] No abstract available.

PMID: 28374449

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  1. Lone Pediatric Atrial Fibrillation in the United States: Analysis of Over 1500 Cases.

El-Assaad I, Al-Kindi SG, Saarel EV, Aziz PF.

Pediatr Cardiol. 2017 Apr 3. doi: 10.1007/s00246-017-1608-7. [Epub ahead of print]

PMID: 28374048

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  1. [Clinical features and outcomes of radiofrequency catheter ablation of atrial flutter in children].

Jiang H, Li XM, Zhang Y, Liu HJ, Li MT, Ge HY.

Zhonghua Er Ke Za Zhi. 2017 Apr 2;55(4):267-271. doi: 10.3760/cma.j.issn.0578-1310.2017.04.007. Chinese.

PMID: 28441822

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  1. [Brief interpretation of “the Pediatric and Congenital Electrophysiology Society (PACES)and the Heart Rhythm Society (HRS) expert consensus statement on the use of catheter ablation in children and patients with congenital heart disease].

Wu JJ, Li F.

Zhonghua Er Ke Za Zhi. 2017 Apr 2;55(4):256-259. doi: 10.3760/cma.j.issn.0578-1310.2017.04.004. Chinese. No abstract available.

PMID: 28441820

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  1. Non-fluoroscopic cardiac ablation of neonates with CHD.

Bigelow AM, Arnold BS, Padrutt GC, Clark JM.

Cardiol Young. 2017 Apr;27(3):592-596. doi: 10.1017/S1047951116001554. Epub 2016 Oct 21.

PMID: 27766996

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  1. What Is the Best Age for Diagnostic Prediction of Pediatric Long-QT Syndrome With a Borderline QT Interval?

Miyazaki A, Doi H.

Circ Arrhythm Electrophysiol. 2017 Apr;10(4). pii: e005119. doi: 10.1161/CIRCEP.117.005119. No abstract available.

PMID: 28356308

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  1. Left ventricular dysfunction is associated with frequent premature ventricular complexes and asymptomatic ventricular tachycardia in children.

Bertels RA, Harteveld LM, Filippini LH, Clur SA, Blom NA.

Europace. 2017 Apr 1;19(4):617-621. doi: 10.1093/europace/euw075.

PMID: 28431063

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  1. Outcomes of lead extraction in young adults.

El-Chami MF, Sayegh MN, Patel A, El-Khalil J, Desai Y, Leon AR, Merchant FM.

Heart Rhythm. 2017 Apr;14(4):537-540. doi: 10.1016/j.hrthm.2017.01.030. Epub 2017 Feb 16.

PMID: 28189822

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  1. Avoiding sports-related sudden cardiac death in children with congenital channelopathy : Recommendations for sports activities.

Lang CN, Steinfurt J, Odening KE.

Herz. 2017 Apr;42(2):162-170. doi: 10.1007/s00059-017-4549-2.

PMID: 28233036

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  1. 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 Apr 1;232:348. doi: 10.1016/j.ijcard.2017.01.077. Epub 2017 Jan 27. No abstract available.

PMID: 28139301 Free Article

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  1. 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 Apr;48(3):369-374. doi: 10.1007/s10840-017-0224-z. Epub 2017 Jan 14.

PMID: 28091832

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  1. Evaluation of Prolonged QT Interval: Structural Heart Disease Mimicking Long QT Syndrome.

Weissler-Snir A, Gollob MH, Chauhan V, Care M, Spears DA.

Pacing Clin Electrophysiol. 2017 Apr;40(4):417-424. doi: 10.1111/pace.13040. Epub 2017 Mar 16.

PMID: 28155223

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  1. Arrhythmias in Adults with Congenital Heart Disease: What Are Risk Factors for Specific Arrhythmias?

Loomba RS, Buelow MW, Aggarwal S, Arora RR, Kovach J, Ginde S.

Pacing Clin Electrophysiol. 2017 Apr;40(4):353-361. doi: 10.1111/pace.12983. Epub 2017 Feb 27.

PMID: 27987225

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  1. Clinical Application of the QRS-T Angle for the Prediction of Ventricular Arrhythmias in Patients with the Fontan Palliation.

Tran TV, Cortez D.

Pediatr Cardiol. 2017 Apr 29. doi: 10.1007/s00246-017-1618-5. [Epub ahead of print]

PMID: 28456831

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  1. Fetal cardiac time intervals in healthy pregnancies – an observational study by fetal ECG (Monica Healthcare System).

Wacker-Gussmann A, Plankl C, Sewald M, Schneider KM, Oberhoffer R, Lobmaier SM.

J Perinat Med. 2017 Apr 28. pii: /j/jpme.ahead-of-print/jpm-2017-0003/jpm-2017-0003.xml. doi: 10.1515/jpm-2017-0003. [Epub ahead of print]

PMID: 28453441

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  1. Electrophysiological effects of anthracyclines in adult survivors of pediatric malignancy.

Markman TM, Ruble K, Loeb D, Chen A, Zhang Y, Beasley GS, Thompson WR, Nazarian S.

Pediatr Blood Cancer. 2017 Apr 28. doi: 10.1002/pbc.26556. [Epub ahead of print]

PMID: 28453898

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  1. Prospective Study of Adenosine on Atrioventricular Nodal Conduction in Pediatric and Young Adult Patients After Heart Transplant.

Flyer JN, Zuckerman WA, Richmond ME, Anderson BR, Mendelsberg TG, McAllister JM, Liberman L, Addonizio LJ, Silver ES.

Circulation. 2017 Apr 27. pii: CIRCULATIONAHA.117.028087. doi: 10.1161/CIRCULATIONAHA.117.028087. [Epub ahead of print]

PMID: 28450351

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  1. Mid-Term Follow-up of School-Aged Children With Borderline Long QT Interval.

Miyazaki A, Sakaguchi H, Matsumura Y, Hayama Y, Noritake K, Negishi J, Tsuda E, Miyamoto Y, Aiba T, Shimizu W, Kusano K, Shiraishi I, Ohuchi H.

Circ J. 2017 Apr 25;81(5):726-732. doi: 10.1253/circj.CJ-16-0991. Epub 2017 Feb 18.

PMID: 28216547 Free Article

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  1. Transesophageal and invasive electrophysiologic evaluation in children with Wolff-Parkinson-White pattern.

Koca S, Pac FA, Kavurt AV, Cay S, Mihcioglu A, Aras D, Topaloglu S.

Pacing Clin Electrophysiol. 2017 Apr 24. doi: 10.1111/pace.13100. [Epub ahead of print]

PMID: 28436586

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  1. The role of echocardiography in fetal tachyarrhythmia diagnosis. A burden for the pediatric cardiologist and a review of the literature.

Gozar L, Marginean C, Toganel R, Muntean I.

Med Ultrason. 2017 Apr 22;19(2):232-235. doi: 10.11152/mu-892.

PMID: 28440361 Free Article

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  1. Automated T-wave analysis can differentiate acquired QT prolongation from congenital long QT syndrome.

Sugrue A, Noseworthy PA, Kremen V, Bos JM, Qiang B, Rohatgi RK, Sapir Y, Attia ZI, Brady P, Caraballo PJ, Asirvatham SJ, Friedman PA, Ackerman MJ.

Ann Noninvasive Electrocardiol. 2017 Apr 21. doi: 10.1111/anec.12455. [Epub ahead of print]

PMID: 28429460

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  1. Cardiac Transplantation in Children and Adolescents with Long QT Syndrome.

Kelle AM, Bos JM, Etheridge SP, Cannon BC, Bryant RM, Johnson JN, Ackerman MJ.

Heart Rhythm. 2017 Apr 14. pii: S1547-5271(17)30451-4. doi: 10.1016/j.hrthm.2017.04.023. [Epub ahead of print]

PMID: 28416468

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  1. Lidocaine Attenuation Testing: An In Vivo Investigation of Putative LQT3-Associated Variants in the SCN5A-Encoded Sodium Channel.

Heather N Anderson MD, Bos JM, Kapplinger JD, Meskill JM, Ye D, Ackerman MJ.

Heart Rhythm. 2017 Apr 12. pii: S1547-5271(17)30448-4. doi: 10.1016/j.hrthm.2017.04.020. [Epub ahead of print]

PMID: 28412158

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  1. The KCNH2-IVS9-28A/G mutation causes aberrant isoform expression and hERG trafficking defect in cardiomyocytes derived from patients affected by Long QT Syndrome type 2.

Mura M, Mehta A, Ramachandra CJ, Zappatore R, Pisano F, Ciuffreda MC, Barbaccia V, Crotti L, Schwartz PJ, Shim W, Gnecchi M.

Int J Cardiol. 2017 Apr 12. pii: S0167-5273(17)30298-X. doi: 10.1016/j.ijcard.2017.04.038. [Epub ahead of print]

PMID: 28433559

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  1. Evaluation of QTc in Rett syndrome: Correlation with age, severity, and genotype.

Crosson J, Srivastava S, Bibat GM, Gupta S, Kantipuly A, Smith-Hicks C, Myers SM, Sanyal A, Yenokyan G, Brenner J, Naidu SR.

Am J Med Genet A. 2017 Apr 10. doi: 10.1002/ajmg.a.38191. [Epub ahead of print]

PMID: 28394409

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  1. Noninvasive predictors of perioperative atrial arrhythmias in patients with tetralogy of Fallot undergoing pulmonary valve replacement.

Cortez D, Barham W, Ruckdeschel E, Sharma N, McCanta AC, von Alvensleben J, Sauer WH, Collins KK, Kay J, Patel S, Nguyen DT.

Clin Cardiol. 2017 Apr 10. doi: 10.1002/clc.22707. [Epub ahead of print]

PMID: 28394443 Free Article

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  1. Tachycardia cycle and atrioventricular nodal conduction properties in children with supraventricular tachycardia.

Mills M, Dubin AM, Motonaga KS, Ceresnak SR.

Pacing Clin Electrophysiol. 2017 Apr 6. doi: 10.1111/pace.13083. [Epub ahead of print] No abstract available.

PMID: 28383202

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  1. To the Editors-Risk factors for complications in the implantation of epicardial pacemakers in neonates and infants.

Kean AC, Rodefeld M.

Heart Rhythm. 2017 Apr 5. pii: S1547-5271(17)30314-4. doi: 10.1016/j.hrthm.2017.03.021. [Epub ahead of print] No abstract available.

PMID: 28389305

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  1. Interpreting Incidentally Identified Variants in Genes Associated With Catecholaminergic Polymorphic Ventricular Tachycardia in a Large Cohort of Clinical Whole-Exome Genetic Test Referrals.

Landstrom AP, Dailey-Schwartz AL, Rosenfeld JA, Yang Y, McLean MJ, Miyake CY, Valdes SO, Fan Y, Allen HD, Penny DJ, Kim JJ.

Circ Arrhythm Electrophysiol. 2017 Apr;10(4). pii: e004742. doi: 10.1161/CIRCEP.116.004742.

PMID: 28404607

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  1. Effect of Age and Sex on the QTc Interval in Children and Adolescents With Type 1 and 2 Long-QT Syndrome.

Vink AS, Clur SB, Geskus RB, Blank AC, De Kezel CC, Yoshinaga M, Hofman N, Wilde AA, Blom NA.

Circ Arrhythm Electrophysiol. 2017 Apr;10(4). pii: e004645. doi: 10.1161/CIRCEP.116.004645.

PMID: 28356306

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  1. J-Wave syndromes expert consensus conference report: Emerging concepts and gaps in knowledge.

Antzelevitch C, Yan GX, Ackerman MJ, Borggrefe M, Corrado D, Guo J, Gussak I, Hasdemir C, Horie M, Huikuri H, Ma C, Morita H, Nam GB, Sacher F, Shimizu W, Viskin S, Wilde AAM.

Europace. 2017 Apr 1;19(4):665-694. doi: 10.1093/europace/euw235. No abstract available.

PMID: 28431071

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  1. 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 Apr 1;232:348. doi: 10.1016/j.ijcard.2017.01.077. Epub 2017 Jan 27. No abstract available.

PMID: 28139301 Free Article

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  1. Precision Cardiovascular Medicine: State of Genetic Testing.

Giudicessi JR, Kullo IJ, Ackerman MJ.

Mayo Clin Proc. 2017 Apr;92(4):642-662. doi: 10.1016/j.mayocp.2017.01.015. Review.

PMID: 28385198

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  1. Early Repolarization in Normal Adolescents is Common.

Ahmed H, Czosek RJ, Spar DS, Knilans TK, Anderson JB.

Pediatr Cardiol. 2017 Apr;38(4):864-872. doi: 10.1007/s00246-017-1594-9. Epub 2017 Apr 3.

PMID: 28367598

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  1. Arrhythmias After Fontan Operation with Intra-atrial Lateral Tunnel Versus Extra-cardiac Conduit: A Systematic Review and Meta-analysis.

Li D, Fan Q, Hirata Y, Ono M, An Q.

Pediatr Cardiol. 2017 Apr;38(4):873-880. doi: 10.1007/s00246-017-1595-8. Epub 2017 Mar 7.

PMID: 28271152

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  1. Lateral Atrial Tunnel Fontan Operation Predisposes to the Junctional Rhythm.

Januszewska K, Schuh A, Lehner A, Dalla-Pozza R, Malec E.

Pediatr Cardiol. 2017 Apr;38(4):712-718. doi: 10.1007/s00246-017-1571-3. Epub 2017 Feb 10.

PMID: 28184977

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  1. 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 Apr;38(4):663-668. doi: 10.1007/s00246-016-1564-7. Epub 2017 Jan 11.

PMID: 28078383

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  1. ECGs in the ED.

Tanel RE.

Pediatr Emerg Care. 2017 Apr;33(4):309-310. doi: 10.1097/PEC.0000000000001149. No abstract available.

PMID: 28353534

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  1. [Our experience in the diagnosis and treatment of postural orthostatic tachycardia syndrome, vasovagal syncope, and inappropriate sinus tachycardia in children].

Ugan Atik S, Dedeoğlu R, Koka A, Öztunç F.

Turk Kardiyol Dern Ars. 2017 Apr;45(3):227-234. doi: 10.5543/tkda.2017.36517. Turkish.

PMID: 28429690 Free Article

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CHD Intervention Featured Articles of April 2017

Cardiac Catheterization Reviews of April 2017 Manuscripts

 

Tricuspid Regurgitation Does Not Impact Right Ventricular Remodeling After Percutaneous Pulmonary Valve Implantation.

Tanase D, Ewert P, Georgiev S, Meierhofer C, Pabst von Ohain J, McElhinney DB, Hager A, Kühn A, Eicken A.

JACC Cardiovasc Interv. 2017 Apr 10;10(7):701-708. doi: 10.1016/j.jcin.2017.01.036.

 

Take Home Points:

 

  • Percutaneous pulmonary valve implantation (PPVI) improves right ventricular (RV) size and exercise performance regardless of the severity of tricuspid regurgitation (TR).
  • Severity of TR improves following PPVI, and therefore the presence of significant TR should not preclude attempts at PPVI.

 

Whiteside, WendyComment from Dr. Wendy Whiteside (Cincinnati), section editor of Congenital Heart Disease Interventions Journal Watch:  Percutaneous pulmonary valve implantation (PPVI) has become the therapy of choice for treatment of RV outflow tract (RVOT) conduit dysfunction.  In patients presenting for PPVI, TR is a frequent associated finding, occurring in as many as 1/3 of patients.  There are multiple etiologies and mechanisms for TR in these patients, including annular dilation in the setting of a dilated right ventricle, valvar dysplasia, or surgical or pacemaker lead disruption, however the presence of significant TR may impact the decision to perform pulmonary valve replacement via a percutaneous or surgical approach and may affect the ability for the RV to remodel following pulmonary valve replacement.  If significant TR is present and the tricuspid valve is felt to require surgical intervention at the time of valve replacement, these patients may be referred exclusively for surgical intervention without consideration for a less invasive approach.  However, if the TR can be improved just by improving the RVPA conduit dysfunction and subsequent RV remodeling, these patients may be able to have a less invasive percutaneous procedure rather than a surgical one.

 

To investigate the impact of moderate or severe TR on RV remodeling after PPVI, Tanase et al conducted a matched cohort study comparing patients who underwent percutaneous pulmonary valve implantation (PPVI) either with significant TR (moderate or severe) or without significant TR, assessing the outcomes of TR severity by echocardiogram, RV size by cardiac MRI, and exercise performance by objective measures from cardiopulmonary exercise testing.

 

Among the 173 patients at their center who underwent PPVI over an 8-year period, patients with moderate to severe TR by echocardiographic criteria were identified, and represented 13% of their PPVI population.  Ultimately, the study included 18 patients with RVOT dysfunction and significant TR and 18 matched control subjects with RVOT dysfunction but no TR.  Patients were matched to have the same pulmonary valve pathology (primary stenosis, insufficiency, mixed), similar indexed RV end-diastolic volume, and similar NYHA functional classification).  Data were obtained at baseline, 6 months post-PPVI, and at latest follow-up (median of 6.5 years post-PPVI, range 8 months to 9.3 years).  All patients with significant TR were identified by serial echocardiography (looking back at least 8 years prior to PPVI) and found to have gradually increasing TR over time with no patients having a sudden increase in TR after surgery to suggest a primary anatomic valve issue.

 

Median patient age was 22±8 years, majority had NYHA functional class II symptoms, median peak oxygen uptake (VO2 peak) was 28.5 mlO2/kg/min, and median RVEDVi was 100 ml/m2 (range 61-185 ml/m2).  After PPVI, the degree of TR improved in 15 of 18 patients (83%) and was unchanged in the remaining 3 patients at 6-month follow-up.  At latest follow-up, no patient had significant TR (15 trivial and 3 mild).  There was a significant decrease in RVEDVi from pre- to post-PPVI with no difference between TR groups.  Similarly, while VO2 increased following PPVI, there was no difference in this increase between TR groups.  Tanase et al therefore conclude that in most patients with RVOT conduit dysfunction and significant TR, PPVI leads to a reduction in TR.  PPVI also decreases RV volume and improves exercise tolerance without a difference between baseline TR severity groups

 

These findings help to provide insight into a clinically very meaningful question—whether the presence of significant TR in a patient with a dysfunctional RVOT conduit should play into the decision for type and timing of intervention.  While they provide convincing evidence to suggest that secondary TR will improve following PPVI, there some important limitations to consider. The mechanism of TR is an important variable in the ability to improve TR.  Tanase et al attempted to exclude patients with a primary tricuspid valve abnormality (by showing a slow progression of TR over time in the included study patients).  Generalizing these results, therefore, to patients with primary valve abnormalities (dysplastic or surgically manipulated valves) should be done with caution.  Secondly, the median RVEDVi of included patients is small (100 ml/m2) and may not be representative of most PPVI populations.  This may lessen the effect of the additional volume load of TR on RV remodeling following PPVI.  Despite these limitations and small patient size, this study is of interest and should be considered in decision making for patients with RVOT conduit dysfunction and concomitant TR.

 

 

 

 

 

 

Impacts of early cardiac catheterization for children with congenital heart disease supported by extracorporeal membrane oxygenation.

Kato A, Lo Rito M, Lee KJ, Haller C, Guerguerian AM, Sivarajan VB, Honjo O.

Catheter Cardiovasc Interv. 2017 Apr;89(5):898-905. doi: 10.1002/ccd.26632. Epub 2016 Jul 14.

PMID: 27416545

 

Take Home Points:

  • Early catheterization is associated with shorter duration of ECMO support and higher survival probability at 30 days after ECMO cannulation.
  • ECMO-related end-organ dysfunction is a significantly poor prognostic factor for successful decannulation.

 

Averin , KonstantinCommentary from Dr. Konstantin Averin (Edmonton), section editor of Congenital Heart Disease Interventions Journal Watch: Extracorporeal membrane oxygenation (ECMO) has been widely used in the pediatric population for cases of peri-operative hemodynamic instability, failure to wean from cardiopulmonary bypass, and cardiopulmonary resuscitation. Cardiac catheterization in patients on ECMO support poses significant challenges but can potentially be useful in improving clinical outcomes. This single center retrospective cohort study focused on patients with congenital heart disease (CHD) and hypothesized that timing of cardiac catheterization may be a predictor of clinical outcomes. The specific aims of the study were: (1) to analyze the institution’s experience with cardiac catheterization on pediatric patients with CHD supported by cardiac ECMO and (2) to determine factors associated with successful weaning from ECMO and short-term outcomes.

 

Three hundred and forty-two patients required ECMO support between 2000 and 2014. Of these, 47 underwent 49 cardiac catheterizations that met inclusion criteria with a median patient age of 65 days and median weight 4.2kg. ECMO was successfully weaned in 33 patients (70%) after a median support time of 4 days with 51% of patients surviving to hospital discharge. Cardiac catheterizations were performed a median of 1 day after ECMO initiation and during 27 of them an intervention was performed (balloon/stent angioplasty and balloon atrial septostomy). There was no procedure related mortality but 9 (18%) procedure-related serious complications with 8 occurring during interventional catheterization (pulmonary artery rupture, stent dislodgement, arrhythmia, pulmonary hemorrhage, and sheath tip migration). In a multivariate analysis, absence of renal or respiratory complications were prognostic of successful ECMO weaning. Kaplan-Meier analysis demonstrated that patient who underwent earlier catheterization (<48 hours after cannulation) had a higher survival probability at 30 days after ECMO cannulation compared with late catheterization.

 

The authors conclude that early cardiac catheterization in pediatric patients with CHD who require ECMO support may be associated with better short-term survival. Early cardiac catheterization should be considered in this cohort, especially if there are unresolved anatomic or physiologic questions. The absence of ECMO-related complications is a predictor for successful weaning off ECMO.

cath 1 april

 

 

Impact of imaging approach on radiation dose and associated cancer risk in children undergoing cardiac catheterization.

Hill KD, Wang C, Einstein AJ, Januzis N, Nguyen G, Li JS, Fleming GA, Yoshizumi TK.

Catheter Cardiovasc Interv. 2017 Apr;89(5):888-897. doi: 10.1002/ccd.26630. Epub 2016 Jun 17.

PMID: 27315598

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Take Home Points:

  • Congenital cardiac catheterizations utilize much higher doses of ionizing radiation than other imaging modalities, such as computed tomography, increasing the lifetime cancer risk for patients
  • Traditional measures of radiation exposure (air kerma or dose-area product) do not accurately measure the true tissue exposure to radiation
  • Altering the methods of image acquisition (collimation, magnification, minimizing source to image distance, removal of antiscatter grids, lower acquisition frame rates) lead to marked variation in effective radiation doses and are easily modifiable by the interventional cardiologist

 

Seckler, MikeCommentary from Dr. Michael Seckeler (Tucson), section editor of Congenital Heart Disease Interventions Journal Watch: The authors have undertaken a very important study for any practitioner who utilizes ionizing radiation for imaging pediatric patients. Using two patient phantoms representing a newborn and a 5-year-old child, they systematically compared the relative effects of different alterable parameters of fluoroscopic imaging used in standard congenital catheterization laboratories on effective radiation doses in the phantoms. In addition, they were able to estimate lifetime cancer risks based on the effective radiation doses. This is the first study to look at this in pediatric patients.

 

The modifiable factors included collimation, magnification, minimizing source to image distance, removal of antiscatter grids and lower acquisition frame rates. These were tested in various combinations and effective radiation doses measured. Optimizing the imaging for minimal radiation exposure (lower magnification, maximal collimation, lowest source to image distance, removal of antiscatter grids and lower acquisition frame rates) all led to significant reductions in the effective radiation doses and lower predicted lifetime cancer risks (Figure 1).

 

The authors do report several limitations to their conclusions. First, optimizing the radiation exposure does not always lead to the best image quality, which has the potential to compromise patient safety during a procedure. However, by highlighting how each of several different factors can improve radiation exposure, this data will allow interventional cardiologists to make as many modifications as possible to find the optimal balance between safety and image quality. Second, the radiation exposures were derived from phantoms and mathematical simulations; however, the intention was not to provide exact doses, but the relative changes in exposure with changes in imaging modality.

This is a very important paper for our field and provides tools to help interventional cardiologists minimize the radiation exposure for our patients.

cath 2 april

CHD Interventions April 2017

 

  1. Real-time three dimensional CT and MRI to guide interventions for congenital heart disease and acquired pulmonary vein stenosis.

Suntharos P, Setser RM, Bradley-Skelton S, Prieto LR.

Int J Cardiovasc Imaging. 2017 Apr 28. doi: 10.1007/s10554-017-1151-x. [Epub ahead of print]

PMID: 28455631

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  1. Clinical Trial Principles and Endpoint Definitions for Paravalvular Leaks in Surgical Prosthesis: An Expert Statement.

Ruiz CE, Hahn RT, Berrebi A, Borer JS, Cutlip DE, Fontana G, Gerosa G, Ibrahim R, Jelnin V, Jilaihawi H, Jolicoeur EM, Kliger C, Kronzon I, Leipsic J, Maisano F, Millan X, Nataf P, O’Gara PT, Pibarot P, Ramee SR, Rihal CS, Rodes-Cabau J, Sorajja P, Suri R, Swain JA, Turi ZG, Tuzcu EM, Weissman NJ, Zamorano JL, Serruys PW, Leon MB; Paravalvular Leak Academic Research Consortium..

J Am Coll Cardiol. 2017 Apr 25;69(16):2067-2087. doi: 10.1016/j.jacc.2017.02.038. Review.

PMID: 28427582

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  1. In vivo intracardiac vector velocity imaging using phased array transducers for pediatric cardiology.

Fadnes S, Wigen M, Nyrnes SA, Lovstakken L.

IEEE Trans Ultrason Ferroelectr Freq Control. 2017 Apr 24. doi: 10.1109/TUFFC.2017.2689799. [Epub ahead of print]

PMID: 28436859

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  1. Prediction of adverse events after catheter-based procedures in adolescents and adults with congenital heart disease in the IMPACT registry.

Stefanescu Schmidt AC, Armstrong A, Kennedy KF, Nykanen D, Aboulhosn J, Bhatt AB.

Eur Heart J. 2017 Apr 18. doi: 10.1093/eurheartj/ehx200. [Epub ahead of print]

PMID: 28430913

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  1. Clinical Trial Principles and Endpoint Definitions for Paravalvular Leaks in Surgical Prosthesis: An Expert Statement.

Ruiz CE, Hahn RT, Berrebi A, Borer JS, Cutlip DE, Fontana G, Gerosa G, Ibrahim R, Jelnin V, Jilaihawi H, Jolicoeur EM, Kliger C, Kronzon I, Leipsic J, Maisano F, Millan X, Nataf P, O’Gara PT, Pibarot P, Ramee SR, Rihal CS, Rodes-Cabau J, Sorajja P, Suri R, Swain JA, Turi ZG, Tuzcu EM, Weissman NJ, Zamorano JL, Serruys PW, Leon MB; of the Paravalvular Leak Academic Research Consortium..

Eur Heart J. 2017 Apr 18. doi: 10.1093/eurheartj/ehx211. [Epub ahead of print]

PMID: 28430909

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  1. Cell Therapy Trials in Congenital Heart Disease.

Oh H.

Circ Res. 2017 Apr 14;120(8):1353-1366. doi: 10.1161/CIRCRESAHA.117.309697. Review.

PMID: 28408455

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  1. A systematic review of 3-D printing in cardiovascular and cerebrovascular diseases.

Sun Z, Lee SY.

Anatol J Cardiol. 2017 Apr 10. doi: 10.14744/AnatolJCardiol.2017.7464. [Epub ahead of print]

PMID: 28430115 Free Article

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  1. Avoidable costs of stenting for aortic coarctation in the United Kingdom: an economic model.

Salcher M, Mcguire A, Muthurangu V, Kelm M, Kuehne T, Naci H; CARDIOPROOF..

BMC Health Serv Res. 2017 Apr 10;17(1):258. doi: 10.1186/s12913-017-2215-2.

PMID: 28395657 Free PMC Article

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  1. Coincidental Significant Tricuspid Regurgitation at the Time of Right Ventricle-to-Pulmonary Artery Conduit Intervention: Should We Address it, Ignore it, or Take a More Nuanced Approach?

Hebson CL, Ephrem G, Rodriguez FH 3rd.

JACC Cardiovasc Interv. 2017 Apr 10;10(7):709-711. doi: 10.1016/j.jcin.2017.02.032. No abstract available.

PMID: 28385409

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  1. Coronary Sinus Defect Following Transcatheter Closure of ASD Using Amplatzer Septal Occluder: Potential Erosion by the Device.

Mohammad Nijres B, Al-Kubaisi M, Bokowski J, Abdulla RI, Awad S.

Pediatr Cardiol. 2017 Apr 10. doi: 10.1007/s00246-017-1613-x. [Epub ahead of print]

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  1. Impacts of early cardiac catheterization for children with congenital heart disease supported by extracorporeal membrane oxygenation.

Kato A, Lo Rito M, Lee KJ, Haller C, Guerguerian AM, Sivarajan VB, Honjo O.

Catheter Cardiovasc Interv. 2017 Apr;89(5):898-905. doi: 10.1002/ccd.26632. Epub 2016 Jul 14.

PMID: 27416545

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  1. Patent foramen ovale with right atrial septal pouch.

Kijima Y, Bokhoor P, Tobis JM.

Catheter Cardiovasc Interv. 2017 Apr;89(5):E169-E171. doi: 10.1002/ccd.26357. Epub 2015 Dec 29.

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  1. In-hospital cost comparison between percutaneous pulmonary valve implantation and surgery.

Andresen B, Mishra V, Lewandowska M, Andersen JG, Andersen MH, Lindberg H, Døhlen G, Fosse E.

Eur J Cardiothorac Surg. 2017 Apr 1;51(4):747-753. doi: 10.1093/ejcts/ezw378.

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  1. Evolution of hybrid interventions for congenital heart disease.

Agrawal H, Alkashkari W, Kenny D.

Expert Rev Cardiovasc Ther. 2017 Apr;15(4):257-266. doi: 10.1080/14779072.2017.1307733. Epub 2017 Mar 23. Review.

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  1. Minimally invasive endoscopic surgery versus catheter-based device occlusion for atrial septal defects in adults: reconsideration of the standard of care.

Schneeberger Y, Schaefer A, Conradi L, Brickwedel J, Reichenspurner H, Kozlik-Feldmann R, Detter C.

Interact Cardiovasc Thorac Surg. 2017 Apr 1;24(4):603-608. doi: 10.1093/icvts/ivw366.

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  1. The Medtronic Micro Vascular Plug™ for Vascular Embolization in Children With Congenital Heart Diseases.

Sathanandam S, Justino H, Waller BR 3rd, Gowda ST, Radtke W, Qureshi AM.

J Interv Cardiol. 2017 Apr;30(2):177-184. doi: 10.1111/joic.12369. Epub 2017 Feb 16.

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  1. Pulmonary artery dissection following balloon valvuloplasty in a dog with pulmonic stenosis.

Grint KA, Kellihan HB.

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  1. Bronchial artery embolization for the treatment of haemoptysis in pulmonary hypertension.

Rasciti E, Sverzellati N, Silva M, Casadei A, Attinà D, Palazzini M, Galiè N, Zompatori M.

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  1. Seven Coils in 1 Heart: Therapeutic Option for Multiple VSD.

Sabiniewicz R, Weryński P.

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Fiszer R, Chojnicki M, Szkutnik M, Haponiuk I, Chodór B, Białkowski J.

EuroIntervention. 2017 Apr 20;12(17):2100-2103. doi: 10.4244/EIJ-D-15-00238.

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  1. Targeted endomyocardial biopsy guided by real-time cardiovascular magnetic resonance.

Unterberg-Buchwald C, Ritter CO, Reupke V, Wilke RN, Stadelmann C, Steinmetz M, Schuster A, Hasenfuß G, Lotz J, Uecker M.

J Cardiovasc Magn Reson. 2017 Apr 19;19(1):45. doi: 10.1186/s12968-017-0357-3.

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Djukic M, Djordjevic SA, Dähnert I.

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den Harder AM, Suchá D, van Doormaal PJ, Budde RPJ, de Jong PA, Schilham AMR, Breur JMPJ, Leiner T.

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  1. Clinical Impact of Stent Implantation for Coarctation of the Aorta with Associated Hypoplasia of the Transverse Aortic Arch.

Lu WH, Fan CS, Chaturvedi R, Lee KJ, Manlhiot C, Benson L.

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  1. Intractable Back Pain After Coil Embolization of Giant Veno-Venous Collaterals in a Patient With Fontan Circulation.

Okada S, Kamada M, Nakagawa N, Ishiguchi Y, Moritoh Y, Shohi M, Okamoto K, Hasegawa S, Ohga S.

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  1. Impact of imaging approach on radiation dose and associated cancer risk in children undergoing cardiac catheterization.

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  1. Early Cardiac Catheterization Leads to Shortened Pediatric Extracorporeal Membrane Oxygenation Run Duration.

Burke CR, Chan T, Rubio AE, McMullan DM.

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Nazif TM, Kalra S, Ali ZA, Karmpaliotis D, Turner ME, Starc TJ, Cao Y, Marboe CC, Collins MB, Leon MB, Kirtane AJ.

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