Congenital EP Featured Articles of July 2017

Congenital Pediatric Cardiac EP Reviews of July/August 2017 Manuscripts

 

A Multicenter Review of Ablation in the Aortic Cusps in Young People

Nguyen MB, Ceresnak SR, Janson CM, Fishberger SB, Love BA, Blaufox AD, Motonaga KS, Dubin AM, Nappo L, Pass RH.

Pacing and Clinical Electrophysiology. 2017; 40:798-802.

 

Take Home Points:

 

  • Catheter ablation in the aortic cusps for the treatment of arrhythmias in young patients is infrequently pursued, but can be performed safely and with high success.
  • Overall experience with aortic cusp ablation in young patients remains limited, but highly supports the following: comprehensive mapping of arrhythmia targets endocardially before pursuing the aortic cusp sites; appropriate imaging of the aortic cusps and coronary arterial origins before, during, and after ablation; and post-procedural assessments to evaluate aortic valve and ventricular function.

 

Philip-Chang_Headshot (small)Comment from Dr. Philip Chang (Gainesville, FL), section editor of Congenital Electrophysiology Journal Watch:  Though rarely done in young patients, overall experience with catheter ablation within the aortic cusps has been growing, primarily within the adult electrophysiology community, and published results have generally been favorable across a variety of arrhythmia types.  Experience with this ablation approach in young patients is limited and far less published.  In this study, Pass et al. sought to provide a multicenter review of the experience with this ablation approach from 5 medium-large sized pediatric EP centers.

 

Following IRB approval at all centers, retrospective review was performed and all cases in patients <21yo and involving aortic cusp ablation were included.  The authors collected general demographic data along with cardiac-specific data, procedural details, and any reported complications for descriptive analysis.

 

A total of 13 patients/cases were found and included with the cohort’s median age being 16yo (range 10-20.5yo), median weight 57.5 kg (range 31-108 kg), median BSA 1.58 m2 (range 1.12-2.33 m2), and fairly even gender distribution (54% male).  All subjects had structurally normal hearts, preserved ventricular function, and no aortic valve pathology.  Standard and conventional diagnostic EPS and conventional intracardiac mapping and attempted arrhythmia treatment were incorporated before pursuing aortic cusp mapping and ablation in all cases.  Electroanatomic mapping (EAM) was incorporated in the majority of cases (12/13).  When aortic cusp mapping and ablation was pursued, aortic root angiography was performed first in RAO and LAO projections.  Selective coronary angiography was not performed based on the procedural description and details provided by the authors.  A standard 4-mm tip RF ablation catheter was used in 11/13 cases (85%) while irrigated-tip RF ablation was performed in the remaining 2 cases.  For standard RF delivery, powers were limited to 30-50 W and maximum temperature limit was 60o C.  Power was <35 W for irrigated RF deliveries.  Cryoenergy was used in 1 case before changing to RF due to lack of success.  Acute success with aortic cusp ablation using RF energy was achieved in all cases.  Post-ablation aortic root angiography was performed in all cases.  Intracardiac echo (ICE) was utilized in the most recent 3 cases included in the cohort.  Post-procedural echo and ECG assessments were performed within 14 days after the procedure.  Of note, the authors did not report the exact percentage of cases that aortic cusp ablation accounted for at individual centers or collectively.  The approach to anticoagulation and target ACT values were not reported.

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Table 1 outlines the variables collected from the 13 cases included.  Ventricular arrhythmias (VT or PVCs) accounted for 69% of the arrhythmias treated (9/13 cases) and most of these were ablated within the noncoronary cusp (NCC; 8/9 cases).  The authors noted the unusually high proportion of NCC targets for ventricular arrhythmias in this young cohort in comparison to what is more typically noted in adult patients.  A total of 3 cases involved anteroseptal pathways (2 concealed, 1 WPW) and the last case involved ectopic atrial tachycardia.  Pseudoaneurysm involving the femoral arterial access site occurred in 1 patient.  Otherwise, no other complications were reported either acutely during the procedure or on post-procedural assessment.  Recurrence of PVCs occurred in 1 case over a median follow-up time of 20 months (range 7-86 months), which was described by the authors as involving very minimal intra-procedural PVC burden that prevented detailed mapping.  Table 2 outlines the respective cusps where ablation was performed in each arrhythmia subtype.  The authors did not report the total number of ablation lesions or duration of RF deliveries that were performed in each case or collectively.

 

Though limited by very small sample size despite its multicenter design, the study provides some important insights for consideration.  First, acute success with low complication rate can be achieved in the majority of patients where the target for ablation is mapped to the aortic cusps.  The authors acknowledged the sample size limitation, but also highlight this as a demonstration of how infrequent aortic cusp mapping and ablation appears to be of necessity (13 cases across 5 institutions over a 6-7 year period).  The exact percentages that these cases accounted for was not reported but would be expected to be extremely low.  While the low percentage likely reflects the low incidence of arrhythmia targets from the cusp locations, the authors also noted that the rarity may also reflect lack of performance secondary to concerns over potential complications.  The small sample size, along with relative paucity of published experience and data also call for more experience and pooling of data to determine best practices in terms of procedural setup, angiography, integration of ICE, necessity of EAM, and post-procedural monitoring and follow-up.

 

The Utility of Exercise Testing in Risk Stratification of Asymptomatic Patients with Type 1 Brugada Pattern

Subramanian M, Prabhu MA, Harikrishnan MS, Shekhar SS, Pai PG, Natarjan K.

J Cardiovasc Electrophysiol 2017; 28:677-683.

 

Take Home Points:

 

  • Treadmill exercise testing may provide data that can aid in the identification of higher risk groups of asymptomatic patients with type 1 Brugada pattern on ECG
  • Treadmill exercise testing in patients with type 1 Brugada pattern on ECG demonstrates ECG changes at peak exercise and recovery that reflect Na channel dysfunction as well as autonomic imbalances that may contribute, in part, to the basis of developing ventricular arrhythmias.

 

Comment from Dr. Philip Chang (Gainesville, FL), section editor of Congenital Electrophysiology Journal Watch:  Risk stratification of asymptomatic patients who are found to have type 1 Brugada patterns of standard 12-lead ECG remains challenging.  While ICD therapy can provide survival benefit, the low true sudden death event rate and relatively high complication rate with ICDs further complicates the determination of who warrants primary prevention ICD implantation in this patient population.  Subramanian et al. sought to evaluate the utility of standard treadmill exercise testing in uncovering ECG changes that could help identify higher risk patients among a group of subjects with type 1 Brugada patterns on ECG.

 

Following IRB approval, the authors performed a retrospective, case controlled analysis of their experience with exercise testing in otherwise asymptomatic patients >18yo with a spontaneous or pharmacologically induced type 1 Brugada pattern on ECG and no structural cardiac abnormalities over the time period January 2007-December 2015.  A total of 75 patients were included with the majority (72.2%) noted to have spontaneous type 1 patterns while the remaining subjects had an induced type 1 pattern with flecainide.  An ICD was implanted in 22/75 study patients with the majority (20/22) for inducible VT/VF during EPS and 1 patient each for family history of Brugada with sudden death (1/22) and history of spontaneous nonsustained VT (1/22).  The control group was comprised of age- and sex-matched healthy subjects.  Standard symptom-limited exercise testing with Bruce protocol was performed.  All subjects were not on any antiarrhythmic medications.  Testing involved 12-lead ECG collections at rest, at the end of each exercise stage, at peak exercise, and at 1-minute intervals during a 6-minute recovery phase (further divided into early and late recovery segments).  Clinical follow-up was conducted every 6 months or sooner if syncope or ICD therapy occurred (in patients implanted with ICDs).

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Tables 1 and 2 show baseline comparisons between study and control subjects (table 1) and comparisons between study subjects with and without major arrhythmic events (MAEs; table 2).  Subjects with type 1 Brugada patterns had significantly longer PR intervals and slower resting heart rates compared to controls.  Among type 1 Brugada patients with and without MAEs, there were no significant differences in variables.

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Exercise testing resulted in measurable changes in S wave upslope, J point, and maximum ST segment elevation during exercise and recovery in patients with type 1 Brugada pattern, and particularly in those who experienced subsequent MAEs. (Figure 2)  Changes were noted in the precordial leads but interestingly, also in lead aVR as well.  The authors noted longer QRS complex durations at peak exercise in type 1 Brugada patients compared to controls (p <0.001).  They also noted significantly slower and delayed heart rate recovery during the recovery period among type 1 Brugada patients compared to controls (p <0.05).  Nonsustained VT occurred in early recovery in 1 patients with a type 1 Brugada pattern.  Over a mean follow-up duration of 77.9 ± 28.9 months, 8/72 study patients (11.1%) had MAEs (5 VT/VF on ICD and 3 sudden deaths) and 3 patients were lost to follow-up.

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Regression analysis showed precordial S wave upslope duration ratio ≥ 30% at peak exercise, aVR augmented J point elevation ≥ 0.3 mV in late recovery, and delayed heart rate recovery ≤ 40% of maximum heart rate in late recovery as being independent predictors of MAEs in type 1 Brugada patients. (Table 3)  Furthermore, they noted a cumulative risk with increasing number or risk factors in the same patient. (Figure 5)

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This study offers several interesting insights with regards to the management of the asymptomatic type 1 Brugada pattern patient.  First, it offers insights into measurable changes on a noninvasive testing modality that is commonly done and widely available that could help to determine which asymptomatic patients may be at higher risk of arrhythmic events.  Second, the changes that occur during exercise testing also appear to highlight a potential autonomic influence, which may vary from patient to patient and may contribute to higher SCD risk in some compared to others.  This offers new avenues to pursue in terms of therapies to modulate autonomics and perhaps new functional analyses in certain genetic mutations which may be more affected by autonomic factors.  The notable ECG changes in lead aVR are also a novel insight, particularly as most attention is focused on the right precordial leads in Brugada syndrome.  Finally, the presence of measurable ECG changes with standard exercise testing also provides insights into ways to monitor treatment response and titrate medical therapy in Brugada syndrome (though pharmacotherapy to treat Brugada syndrome is very limited to begin with).

There are several limitations noted in this study, including some limitations to the applicability and extrapolation to pediatric patients since this was an adult cohort that excluded patients <18yo.  The overall sample size of the study cohort was small in this single institution study and there may be regional and international variations in disease phenotype and practice composition.  This may be 1 explanation as to the rather high MAE incidence of 11.1%.  Nearly a quarter of study patients also had pharmacologic testing done, though the authors did not disclose the reasons for testing and therefore whether these patients were truly asymptomatic is in question.  The integration of genetic testing was also not discussed.

 

 

CHD EP July 2017

 

  1. Management of postoperative junctional ectopic tachycardia in pediatric patients: a survey of 30 centers in Germany, Austria, and Switzerland.

Entenmann A, Michel M, Herberg U, Haas N, Kumpf M, Gass M, Egender F, Gebauer R.

Eur J Pediatr. 2017 Jul 21. doi: 10.1007/s00431-017-2969-x. [Epub ahead of print]

PMID: 28730319

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  1. Genotype Positive Long QT Syndrome in Patients With Coexisting Congenital Heart Disease.

Ebrahim MA, Williams MR, Shepard S, Perry JC.

Am J Cardiol. 2017 Jul 15;120(2):256-261. doi: 10.1016/j.amjcard.2017.04.018. Epub 2017 Apr 27.

PMID: 28532774

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  1. Increased risk of thromboembolic events in adult congenital heart disease patients with atrial tachyarrhythmias: Bias due to the data sparsity.

Ayubi E, Safiri S, Mansournia MA.

Int J Cardiol. 2017 Jul 15;239:20. doi: 10.1016/j.ijcard.2017.02.133. No abstract available.

PMID: 28560967

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  1. How to Perform Transconduit and Transbaffle Puncture in Patients who have previously undergone the Fontan or Mustard Operation.

Uhm JS, Kim NK, Kim TH, Joung B, Pak HN, Lee MH.

Heart Rhythm. 2017 Jul 14. pii: S1547-5271(17)30877-9. doi: 10.1016/j.hrthm.2017.07.020. [Epub ahead of print] No abstract available.

PMID: 28716702

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  1. A focus on pharmacological management of catecholaminergic polymorphic ventricular tachycardia.

Barbanti C, Maltret A, Sidi D.

Mini Rev Med Chem. 2017 Jul 7. doi: 10.2174/1389557517666170707100923. [Epub ahead of print]

PMID: 28685702

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  1. Does pharmacological therapy still play a role in preventing sudden death in surgically treated Tetralogy of Fallot?

Bronzetti G, Brighenti M, Bonvicini M.

Mini Rev Med Chem. 2017 Jul 7. doi: 10.2174/1389557517666170707101411. [Epub ahead of print]

PMID: 28685697

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  1. Major adverse events and atrial tachycardia in Ebstein’s anomaly predicted by cardiovascular magnetic resonance.

Rydman R, Shiina Y, Diller GP, Niwa K, Li W, Uemura H, Uebing A, Barbero U, Bouzas B, Ernst S, Wong T, Pennell DJ, Gatzoulis MA, Babu-Narayan SV.

Heart. 2017 Jul 6. pii: heartjnl-2017-311274. doi: 10.1136/heartjnl-2017-311274. [Epub ahead of print]

PMID: 28684436 Free Article

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  1. Prevention of Sudden Cardiac Death in Adults With Congenital Heart Disease: Do the Guidelines Fall Short?

Vehmeijer JT, Koyak Z, Budts W, Harris L, Silversides CK, Oechslin EN, Bouma BJ, Zwinderman AH, Mulder BJM, de Groot JR.

Circ Arrhythm Electrophysiol. 2017 Jul;10(7). pii: e005093. doi: 10.1161/CIRCEP.116.005093.

PMID: 28696220

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  1. Where to Throw That Shoe? Catheter Ablation of Atrioventricular Nodal Reentrant Tachycardia in Congenital Heart Disease.

Sherwin ED, Berul CI.

Circ Arrhythm Electrophysiol. 2017 Jul;10(7). pii: e005525. doi: 10.1161/CIRCEP.117.005525. No abstract available.

PMID: 28687672

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  1. Atrioventricular Nodal Reentrant Tachycardia in Patients With Congenital Heart Disease: Outcome After Catheter Ablation.

Papagiannis J, Beissel DJ, Krause U, Cabrera M, Telishevska M, Seslar S, Johnsrude C, Anderson C, Tisma-Dupanovic S, Connelly D, Avramidis D, Carter C, Kornyei L, Law I, Von Bergen N, Janusek J, Silva J, Rosenthal E, Willcox M, Kubus P, Hessling G, Paul T; Pediatric and Congenital Electrophysiology Society.

Circ Arrhythm Electrophysiol. 2017 Jul;10(7). pii: e004869. doi: 10.1161/CIRCEP.116.004869. Epub 2017 Jul 7.

PMID: 28687669

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  1. Appearance of QRS fragmentation late after Mustard/Senning repair is associated with adverse outcome.

Helsen F, Vandenberk B, De Meester P, Van De Bruaene A, Gabriels C, Troost E, Gewillig M, Meyns B, Willems R, Budts W; FH and BV contributed equally to this study.

Heart. 2017 Jul;103(13):1036-1042. doi: 10.1136/heartjnl-2016-310512. Epub 2017 Feb 9.

PMID: 28183791

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  1. Localized atrial reentrant tachycardia in tetralogy of Fallot: Ultra-high-resolution mapping and termination by nonpropagated atrial pacing stimulus.

Lee A, Kite J, Davison O, Haqqani HM.

Heart Rhythm. 2017 Jul;14(7):1102-1103. doi: 10.1016/j.hrthm.2017.03.007. Epub 2017 Mar 18. No abstract available.

PMID: 28323170

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  1. Congenital heart block and immune mediated sensorineural hearing loss: possible cross reactivity of immune response.

Bason C, Pagnini I, Brucato A, Maestroni S, Puccetti A, Lunardi C, Cimaz R.

Lupus. 2017 Jul;26(8):835-840. doi: 10.1177/0961203316682099. Epub 2016 Dec 5.

PMID: 27913750

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  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 Jul;40(7):843-849. doi: 10.1111/pace.13101. Epub 2017 Jun 14.

PMID: 28436549

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  1. Discrimination between QRS and T Waves Using a Right Parasternal Lead for S-ICD in a Patient with a Single Ventricle.

Nishiyama T, Kimura T, Nishiyama N, Aizawa Y, Fukuda K, Takatsuki S.

Pacing Clin Electrophysiol. 2017 Jul;40(7):904-907. doi: 10.1111/pace.13046. Epub 2017 Mar 3.

PMID: 28185283

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  1. Pediatric Dosing of Intravenous Sotalol Based on Body Surface Area in Patients with Arrhythmia.

Li X, Zhang Y, Liu H, Jiang H, Ge H, Zhang Y.

Pediatr Cardiol. 2017 Jul 28. doi: 10.1007/s00246-017-1683-9. [Epub ahead of print]

PMID: 28755092

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  1. Radiofrequency ablation of fast ventricular tachycardia causing an ICD storm in an infant with hypertrophic cardiomyopathy.

Ergul Y, Ozyilmaz I, Bilici M, Ozturk E, Haydin S, Guzeltas A.

Pacing Clin Electrophysiol. 2017 Jul 27. doi: 10.1111/pace.13154. [Epub ahead of print]

PMID: 28749010

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  1. NS1643 enhances ionic currents in a G604S-WT hERG co-expression system associated with long QT syndrome 2.

Huo J, Guo X, Lu Q, Qiang H, Liu P, Bai L, Huang CLH, Zhang Y, Ma A.

Clin Exp Pharmacol Physiol. 2017 Jul 25. doi: 10.1111/1440-1681.12820. [Epub ahead of print]

PMID: 28741726

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  1. Contemporary Outcomes in Patients With Long QT Syndrome.

Rohatgi RK, Sugrue A, Bos JM, Cannon BC, Asirvatham SJ, Moir C, Owen HJ, Bos KM, Kruisselbrink T, Ackerman MJ.

J Am Coll Cardiol. 2017 Jul 25;70(4):453-462. doi: 10.1016/j.jacc.2017.05.046.

PMID: 28728690

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  1. What endocardial right ventricular pacing site shows better contractility and synchrony in children and adolescents?

Silvetti MS, Ammirati A, Palmieri R, Pazzano V, Placidi S, Ravà L, Remoli R, Saputo FA, Verticelli L, Drago F.

Pacing Clin Electrophysiol. 2017 Jul 25. doi: 10.1111/pace.13153. [Epub ahead of print]

PMID: 28744930

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  1. Potential utility of pulsed Doppler for prenatal diagnosis of fetal ventricular tachycardia secondary to long QT syndrome.

Miyoshi T, Sakaguchi H, Shiraishi I, Yoshimatsu J, Ikeda T.

Ultrasound Obstet Gynecol. 2017 Jul 25. doi: 10.1002/uog.18819. [Epub ahead of print]

PMID: 28741754

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  1. Plakophilin-2 is required for transcription of genes that control calcium cycling and cardiac rhythm.

Cerrone M, Montnach J, Lin X, Zhao YT, Zhang M, Agullo-Pascual E, Leo-Macias A, Alvarado FJ, Dolgalev I, Karathanos TV, Malkani K, Van Opbergen CJM, van Bavel JJA, Yang HQ, Vasquez C, Tester D, Fowler S, Liang F, Rothenberg E, Heguy A, Morley GE, Coetzee WA, Trayanova NA, Ackerman MJ, van Veen TAB, Valdivia HH, Delmar M.

Nat Commun. 2017 Jul 24;8(1):106. doi: 10.1038/s41467-017-00127-0.

PMID: 28740174 Free PMC Article

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  1. The impact of intrauterine treatment on fetal tachycardia: a nationwide survey in Japan.

Ueda K, Maeno Y, Miyoshi T, Inamura N, Kawataki M, Taketazu M, Nii M, Hagiwara A, Horigome H, Shozu M, Shimizu W, Yasukochi S, Yoda H, Shiraishi I, Sakaguchi H, Katsuragi S, Sago H, Ikeda T; ; on behalf of Japan Fetal Arrhythmia Group.

J Matern Fetal Neonatal Med. 2017 Jul 19:1-6. doi: 10.1080/14767058.2017.1350159. [Epub ahead of print]

PMID: 28720014

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  1. Utility of Echocardiography in Detecting Silent Complications After Pediatric Catheter Ablations.

Amdani SM, Sallaam S, Karpawich PP, Aggarwal S.

Pediatr Cardiol. 2017 Jul 15. doi: 10.1007/s00246-017-1680-z. [Epub ahead of print]

PMID: 28711964

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  1. Bridge to Success: A Better Method of Cryoablation for Atrioventricular Nodal Reentrant Tachycardia in Children.

Reddy CD, Ceresnak SR, Motonaga KS, Avasarala K, Feller C, Trela A, Hanisch D, Dubin AM.

Heart Rhythm. 2017 Jul 14. pii: S1547-5271(17)30875-5. doi: 10.1016/j.hrthm.2017.07.018. [Epub ahead of print]

PMID: 28716699

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  1. Uptake of Predictive Genetic Testing and Cardiac Evaluation for Children at Risk for an Inherited Arrhythmia or Cardiomyopathy.

Christian S, Atallah J, Clegg R, Giuffre M, Huculak C, Dzwiniel T, Parboosingh J, Taylor S, Somerville M.

J Genet Couns. 2017 Jul 11. doi: 10.1007/s10897-017-0129-0. [Epub ahead of print]

PMID: 28699125

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  1. Does pharmacological therapy still play a role in preventing sudden death in surgically treated Tetralogy of Fallot?

Bronzetti G, Brighenti M, Bonvicini M.

Mini Rev Med Chem. 2017 Jul 7. doi: 10.2174/1389557517666170707101411. [Epub ahead of print]

PMID: 28685697

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  1. Therapy Of Cardiac Arrhythmias In Children: An Emerging Role Of Electroanatomical Mapping Systems.

Matteo C, Maurizio M, Viviana T, Scarano M, Paolo B, Giuseppe D.

Curr Vasc Pharmacol. 2017 Jul 5. doi: 10.2174/1570161115666170705155542. [Epub ahead of print]

PMID: 28677509

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  1. [Clinical analysis of pacing therapy and treatment of complications during follow-up in children].

Zhao PJ, Chen YW, Li F, Li Y, Yang JP, Wu JJ.

Zhonghua Er Ke Za Zhi. 2017 Jul 2;55(7):514-518. doi: 10.3760/cma.j.issn.0578-1310.2017.07.009. Chinese.

PMID: 28728260

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  1. Successful use of sirolimus for refractory atrial ectopic tachycardia in a child with cardiac rhabdomyoma.

Ninic S, Kalaba M, Jovicic B, Vukomanovic V, Prijic S, Vucetic B, Kravljanac R, Vujic A, Kosutic J.

Ann Noninvasive Electrocardiol. 2017 Jul;22(4). doi: 10.1111/anec.12435. Epub 2017 Feb 19.

PMID: 28217909

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  1. Effects of Acute Carbon Monoxide Poisoning on ECG and Echocardiographic Parameters in Children.

Ozyurt A, Karpuz D, Yucel A, Tosun MD, Kibar AE, Hallioglu O.

Cardiovasc Toxicol. 2017 Jul;17(3):326-334. doi: 10.1007/s12012-016-9389-4.

PMID: 27778147

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Select item 27509882

 

  1. Risk factors for sudden cardiac death in childhood hypertrophic cardiomyopathy: A systematic review and meta-analysis.

Norrish G, Cantarutti N, Pissaridou E, Ridout DA, Limongelli G, Elliott PM, Kaski JP.

Eur J Prev Cardiol. 2017 Jul;24(11):1220-1230. doi: 10.1177/2047487317702519. Epub 2017 May 9.

PMID: 28482693

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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 MD.

Heart Rhythm. 2017 Jul;14(7):e53. doi: 10.1016/j.hrthm.2017.03.021. Epub 2017 Apr 5. No abstract available.

PMID: 28389305

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  1. Skin sympathetic nerve activity precedes the onset and termination of paroxysmal atrial tachycardia and fibrillation.

Uradu A, Wan J, Doytchinova A, Wright KC, Lin AYT, Chen LS, Shen C, Lin SF, Everett TH 4th, Chen PS.

Heart Rhythm. 2017 Jul;14(7):964-971. doi: 10.1016/j.hrthm.2017.03.030. Epub 2017 Mar 24.

PMID: 28347833

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  1. Efficacy of Flecainide in the Treatment of Catecholaminergic Polymorphic Ventricular Tachycardia: A Randomized Clinical Trial.

Kannankeril PJ, Moore JP, Cerrone M, Priori SG, Kertesz NJ, Ro PS, Batra AS, Kaufman ES, Fairbrother DL, Saarel EV, Etheridge SP, Kanter RJ, Carboni MP, Dzurik MV, Fountain D, Chen H, Ely EW, Roden DM, Knollmann BC.

JAMA Cardiol. 2017 Jul 1;2(7):759-766. doi: 10.1001/jamacardio.2017.1320.

PMID: 28492868

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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 Jul;40(7):808-814. doi: 10.1111/pace.13100. Epub 2017 Jun 1.

PMID: 28436586

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Congenital EP Featured Articles of June 2017

 

Congenital and Pediatric Cardiac EP Reviews of June 2017 Manuscripts

 

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

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

Circulation. 2017 Jun 20;135(25):2485-2493. doi: 10.1161/CIRCULATIONAHA.117.028087. Epub 2017 Apr 27.

PMID: 28450351

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Take Home Points:

  • Supraventricular tachycardia can be seen in up to 50% of children and adults after heart transplantation.
  • Adenosine, a endogenous nucleoside that results in sinus slowing and AV block, has been considered relatively contraindicated in heart transplant recipients due to post-transplant denervation
  • Adenosine use at low doses of 25 μg/kg or 1.5 mg were found to be effective in causing AV block in healthy heart transplant patients in sinus rhythm
  • AV block was seen in 96% of patients with escalating adenosine administration up to maximal dose of 200 μg/kg or 12 mg
  • There were no adverse events seen after adenosine administration including no clinically significant asystole (≥ 12 seconds) requiring rescue ventricular pacing
  • The use of adenosine during supraventricular tachycardia in post-heart transplants patients was not evaluated and therefore, the dosing, response and safety of adenosine in that scenario cannot be commented on based on this study.

 

A PatelComment from Dr. Akash Patel (San Francisco), section editor of Congenital Electrophysiology Journal Watch:  Previous limited data has suggested heart transplant patients are at adverse risk to adenosine in the setting of cardiac denervation after transplantation. In particular, adenosine exposure in this setting has been thought to lead to excessive AV block and bradycardia. Due to this concern, adenosine is relatively contraindicated in post-heart transplant patients and thus uncommonly used. However, up to 50% of children and adults with heart transplantation can be affected by supraventricular tachycardia with adenosine potentially providing a therapeutic and/or diagnostic option.  This single center prospective study from New York Presbyterian/Morgan Stanley Children’s Hospital, Columbia University Medical Center aimed to improve our understanding of the safety and efficacy of adenosine in pediatric and young adult patients who have undergone heart transplantation with hopes to reconsider its use in this population.

 

All healthy patients ≥ 2 weeks post-cardiac transplantation were included if they agreed to consent and were excluded if there was evidence of rejection (clinical/humeral/cellular), graft vasculopathy, concurrent use of intravenous inotropic medication, concurrent treatment for rejection, conduction disease (first-, second-, or third-degree AV block, preexisting sinus node dysfunction), or systolic ventricular dysfunction. At the time of routine post-cardiac transplant catheterization, eligible patients underwent adenosine testing.

The study group included 80 patients (55% male) with a median age of 13.6 years (Range: 1.1 – 24 year). The median weight was 50.4 kg (Range: 7.8 – 120 kg). The indications for transplantation included cardiomyopathy (60%), congenital heart disease (30%), retransplantation (7.5%), and other (2.5%). The most common type of transplantation was bicaval (71%) followed by biatrial (28%). Prior history of rejection was seen in 49%. There was use of dipyridamole in 53% which is a medication known to effect adenosine metabolism. This medication was stopped 3 days before catheterization in all patients. There was use of antiarrhythmic drugs – beta-blocker (24%) and digoxin (2.5%)- but no data on indications or prior arrhythmia status. The cardiac catheterization procedures were done under general anesthesia in 64%.

After catheterization and biopsy were complete but before the removal of central access, a 4F quadripolar pacing catheter was introduced into the right ventricle with pacing thresholds tested and outputs set appropriately. Adenosine was given via central access in serial escalating doses based on weight until AV block or clinically significant asystole (either a sinus pause or AV block ≥ 12 seconds which were the predetermined criterion to initiate ventricular pacing) were seen. If patients weighed <60 kg, patients were given 12.5, 25, 50, 100, and 200 μg/kg of adenosine. If patients weighed ≥ 60 kg, patients were given 0.8, 1.5, 3, 6, and 12 mg.  See below.  The median maximal dose of adenosine given was 3.6 mg (IQR: 2.0 – 6.0 mg).

June EP v1

The primary outcome of the study was to assess the safety of adenosine.  There were no patients (0%) who had clinically significant asystole requiring rescue ventricular pacing after adenosine administration during the escalating adenosine protocol. See below.

 June EP V2

 

The secondary objective was to assess the efficacy of adenosine in the post-heart transplant patient. There was AV block seen in 77/80 (96%) of the cohort (see above).  There was sinus pause > 2 seconds seen in 4 (5%). The effect of AV nodal blockade was seen at doses as low as 25 μg/kg (< 60 kg) or 1.5 mg (≥ 60kg) in 12%. As expected, incremental escalations in dosing increased the percent of patients demonstrating AV nodal block. At up to initial standard dosing of 100 μg/kg (< 60 kg) or 6 mg (≥ 60kg), there was AV nodal blockade seen in 72%.  See Below.

June EP V3The AV nodal effects of adenosine varied in duration. The median longest R-R interval during AV block was 1.9 seconds (IQR: 1.4 – 3.2 seconds) (Example See Figure Below (A)) The mean total adenosine effect was 4.3 seconds (SD: ±2.0 seconds)  (Example: See Figure Below (B)). The absolute longest R-R interval and total adenosine effect in any patient was 8.4 seconds. The median consecutive non-conducted P waves during the longest pause were 2 (IQR, 1–3). The authors stated no variable predicted adenosine effect including prior rejection, maximal dose, weight, or time from transplant.

June EP v6

The use of adenosine was well tolerated with typical symptoms reported in the non-anesthetized group including shortness of breath, discomfort, chest pain, headache, and flushing. There were no adverse symptoms that resulted in premature termination of the protocol.

This study demonstrates the safety of adenosine use in a large healthy pediatric and young adult post-cardiac transplant cohort with no adverse effects including no clinically significant asystole requiring rescue ventricular pacing.  The authors conclude that adenosine is effective in causing AV node blockade at much lower doses – 25 μg/kg (< 60kg) or 1.5mg (≥ 60 kg) –  than PALS or ACLS standard dosing. As the effect is dose-dependent, gradual dose escalation can be considered up to maximal doses of 200 μg/kg (< the 60kg) or 12 mg (≥60kg) until AV blockade is achieved.

This study however does not address if and why lower doses are required and if there is an exaggerated response with adenosine use in post-transplant patients. In particular, it is unclear if the same lower doses given centrally in healthy age-based controls would have resulted in the same effect. In addition, in 1990 Ellenbogen et al (Circulation 1990;81: 821-828  PMID: 2306833) demonstrated there was evidence of a supersensitivity effect in adult patients. Since the dose escalation was terminated once AV block was seen, it is unclear if maximal dosing in all 80 patients would have resulted in an exaggerated response. The study design did not allow for answering these important questions but did show safety with their adenosine dose escalation protocol.  The authors discuss the roles of cardiac denervation and reinnervation as it may play a factor in these issues, but interesting the study showed safe adenosine effect as early as 25 days post-transplant and overall no correlation between time of transplant and effective adenosine dose suggesting other factors may play a role.

Clearly there are limitations to this study with the most notable being that patients are known to be healthy, were not in supraventricular tachycardia at the time of adenosine administration, and the route of adenosine administration was via central access. From a practical standpoint, adenosine is often given peripherally and used in an acute setting. Therefore, the effect of adenosine in the “healthy patient” is a less likely scenario as their rejection status and function may not be known and they may be on antiarrhythmics or other medications that may affect the response to adenosine. Nevertheless, this study demonstrates that adenosine may be used safely in this population but a cautious approach using low doses should be undertaken.

 

Additional EP Articles Worth Reading.

 

Intraoperative arrhythmias in children with congenital heart disease: transient, innocent events? Houck CA, Ramdjan TTTK, Yaksh A, Teuwen CP, Lanters EAH, Bogers AJJC, de Groot NMS.Europace. 2017 Jun 28. doi: 10.1093/europace/eux072. [Epub ahead of print]

PMID: 28666343

 

Cardiac Fibroblast Transcriptome Analyses Support a Role for Interferogenic, Profibrotic and Inflammatory Genes in Anti-SSA/Ro-Associated Congenital Heart Block.

Clancy RM, Markham AJ, Jackson T, Rasmussen SE, Blumenberg M, Buyon JP.

Am J Physiol Heart Circ Physiol. 2017 Jun 16:ajpheart.00256.2017. doi: 10.1152/ajpheart.00256.2017. [Epub ahead of print]

PMID: 28626076

 

Cardiac Arrhythmias in Adults with Congenital Heart Disease.

Balaji S, Mandapati R, Shivkumar K.

Card Electrophysiol Clin. 2017 Jun;9(2):xv-xvi. doi: 10.1016/j.ccep.2017.04.001. No abstract available.

PMID: 28457248

 

Genotype-Phenotype Correlation of SCN5A Mutation for the Clinical and Electrocardiographic Characteristics of Probands With Brugada Syndrome: A Japanese Multicenter Registry.

Yamagata K, Horie M, Aiba T, Ogawa S, Aizawa Y, Ohe T, Yamagishi M, Makita N, Sakurada H, Tanaka T, Shimizu A, Hagiwara N, Kishi R, Nakano Y, Takagi M, Makiyama T, Ohno S, Fukuda K, Watanabe H, Morita H, Hayashi K, Kusano K, Kamakura S, Yasuda S, Ogawa H, Miyamoto Y, Kapplinger JD, Ackerman MJ, Shimizu W.

Circulation. 2017 Jun 6;135(23):2255-2270. doi: 10.1161/CIRCULATIONAHA.117.027983. Epub 2017 Mar 24.

PMID: 28341781

 

 

CHD EP June 2017

 

  1. Intraoperative arrhythmias in children with congenital heart disease: transient, innocent events?

Houck CA, Ramdjan TTTK, Yaksh A, Teuwen CP, Lanters EAH, Bogers AJJC, de Groot NMS.

Europace. 2017 Jun 28. doi: 10.1093/europace/eux072. [Epub ahead of print]

PMID: 28666343

 

Select item 28660303

 

  1. Cardiac Fibroblast Transcriptome Analyses Support a Role for Interferogenic, Profibrotic and Inflammatory Genes in Anti-SSA/Ro-Associated Congenital Heart Block.

Clancy RM, Markham AJ, Jackson T, Rasmussen SE, Blumenberg M, Buyon JP.

Am J Physiol Heart Circ Physiol. 2017 Jun 16:ajpheart.00256.2017. doi: 10.1152/ajpheart.00256.2017. [Epub ahead of print]

PMID: 28626076

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  1. Sudden cardiac death in the young: Epidemiology and overview.

Link MS.

Congenit Heart Dis. 2017 Jun 15. doi: 10.1111/chd.12494. [Epub ahead of print]

PMID: 28618149

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  1. Sudden death in athletes.

Corrado D, Zorzi A.

Int J Cardiol. 2017 Jun 15;237:67-70. doi: 10.1016/j.ijcard.2017.03.034. Epub 2017 Mar 10.

PMID: 28318658

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  1. <i>Nkx2.5</i> is Essential to Establish Normal Heart Rate Variability in the Zebrafish Embryo.

Harrington JK, Sorabella R, Tercek A, Isler JR, Targoff KL.

Am J Physiol Regul Integr Comp Physiol. 2017 Jun 14:ajpregu.00223.2016. doi: 10.1152/ajpregu.00223.2016. [Epub ahead of print]

PMID: 28615160

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  1. Calcium Signaling and Cardiac Arrhythmias.

Landstrom AP, Dobrev D, Wehrens XHT.

Circ Res. 2017 Jun 9;120(12):1969-1993. doi: 10.1161/CIRCRESAHA.117.310083. Review.

PMID: 28596175

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  1. Permanent chronotropic impairment after closure of atrial or ventricular septal defect.

Heiberg J, Nyboe C, Hjortdal VE.

Scand Cardiovasc J. 2017 Jun 8:1-6. doi: 10.1080/14017431.2017.1337216. [Epub ahead of print]

PMID: 28592193

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  1. Cardiac Arrhythmias in Adults with Congenital Heart Disease.

Balaji S, Mandapati R, Shivkumar K.

Card Electrophysiol Clin. 2017 Jun;9(2):xv-xvi. doi: 10.1016/j.ccep.2017.04.001. No abstract available.

PMID: 28457248

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  1. Arrhythmia Surgery for Adults with Congenital Heart Disease.

Deal BJ, Mavroudis C.

Card Electrophysiol Clin. 2017 Jun;9(2):329-340. doi: 10.1016/j.ccep.2017.02.014. Review.

PMID: 28457246

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  1. Cardiac Arrhythmias in Adults with Congenital Heart Disease: Pacemakers, Implantable Cardiac Defibrillators, and Cardiac Resynchronization Therapy Devices.

Cecchin F, Halpern DG.

Card Electrophysiol Clin. 2017 Jun;9(2):319-328. doi: 10.1016/j.ccep.2017.02.013. Epub 2017 Mar 31. Review.

PMID: 28457245

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  1. Catheter Ablation: General Principles and Advances.

Ernst S.

Card Electrophysiol Clin. 2017 Jun;9(2):311-317. doi: 10.1016/j.ccep.2017.02.012. Epub 2017 Mar 14. Review.

PMID: 28457244

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  1. Drug Therapy in Adult Congenital Heart Disease.

Contractor T, Levin V, Mandapati R.

Card Electrophysiol Clin. 2017 Jun;9(2):295-309. doi: 10.1016/j.ccep.2017.02.011. Review.

PMID: 28457243

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  1. Sudden Cardiac Death in Adult Congenital Heart Disease.

Ávila P, Chaix MA, Mondésert B, Khairy P.

Card Electrophysiol Clin. 2017 Jun;9(2):225-234. doi: 10.1016/j.ccep.2017.02.003. Epub 2017 Mar 18. Review.

PMID: 28457237

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  1. Ventricular Arrhythmias in Adult Congenital Heart Disease: Mechanisms, Diagnosis, and Clinical Aspects.

Sathananthan G, Harris L, Nair K.

Card Electrophysiol Clin. 2017 Jun;9(2):213-223. doi: 10.1016/j.ccep.2017.02.004. Epub 2017 Mar 18. Review.

PMID: 28457236

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  1. Supraventricular Tachycardia in Adult Congenital Heart Disease: Mechanisms, Diagnosis, and Clinical Aspects.

Janson CM, Shah MJ.

Card Electrophysiol Clin. 2017 Jun;9(2):189-211. doi: 10.1016/j.ccep.2017.02.005. Review.

PMID: 28457235

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  1. Bradyarrhythmias in Congenital Heart Disease.

Carlson SK, Patel AR, Chang PM.

Card Electrophysiol Clin. 2017 Jun;9(2):177-187. doi: 10.1016/j.ccep.2017.02.002. Epub 2017 Mar 22. Review.

PMID: 28457234

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  1. Introduction to the Congenital Heart Defects: Anatomy of the Conduction System.

Moore JP, Aboulhosn JA.

Card Electrophysiol Clin. 2017 Jun;9(2):167-175. doi: 10.1016/j.ccep.2017.02.001. Epub 2017 Mar 14. Review.

PMID: 28457233

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  1. Ventricular pacing in single ventricles-A bad combination.

Bulic A, Zimmerman FJ, Ceresnak SR, Shetty I, Motonaga KS, Freter A, Trela AV, Hanisch D, Russo L, Avasarala K, Dubin AM.

Heart Rhythm. 2017 Jun;14(6):853-857. doi: 10.1016/j.hrthm.2017.03.035.

PMID: 28528723

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

Wiles BM, Fitzsimmons SJ, Roberts PR.

Pacing Clin Electrophysiol. 2017 Jun;40(6):735-737. doi: 10.1111/pace.13089. Epub 2017 May 16. 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 Jun;38(5):1004-1009. doi: 10.1007/s00246-017-1608-7. Epub 2017 Apr 3.

PMID: 28374048

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  1. Measures of and changes in heart rate variability in pediatric heart transplant recipients.

Williams T, Tang X, Gilmore G, Gossett J, Knecht KR.

Pediatr Transplant. 2017 Jun;21(4). doi: 10.1111/petr.12894. Epub 2017 Feb 8.

PMID: 28181355

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  1. [News techniques of pacing in congenital heart diseases].

Le Bloa M, Thambo JB.

Presse Med. 2017 Jun;46(6 Pt 1):594-605. doi: 10.1016/j.lpm.2017.05.015. Epub 2017 Jun 5. French.

PMID: 28595994

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  1. Postnatal Outcomes of Fetal Supraventricular Tachycardia: a Multicenter Study.

Hinkle KA, Peyvandi S, Stiver C, Killen SAS, Weng HY, Etheridge SP, Puchalski MD.

Pediatr Cardiol. 2017 Jun 29. doi: 10.1007/s00246-017-1662-1. [Epub ahead of print]

PMID: 28664446

 

Select item 28664445

 

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

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

Circulation. 2017 Jun 20;135(25):2485-2493. doi: 10.1161/CIRCULATIONAHA.117.028087. Epub 2017 Apr 27.

PMID: 28450351

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  1. Right Atrial Diverticulosis and Early-onset Arrhythmia: Rare Cause of Incessant Neonatal Arrhythmia.

Aggarwal N, Joshi R, Joshi RK, Agarwal M.

Indian Pediatr. 2017 Jun 15;54(6):503-504.

PMID: 28667725

 

Select item 28620681

 

  1. Variation in Pediatric Post-Ablation Care: A Survey of the Pediatric and Congenital Electrophysiology Society (PACES).

Dechert BE, Dick M 2nd, Bradley DJ, LaPage MJ.

Pediatr Cardiol. 2017 Jun 15. doi: 10.1007/s00246-017-1654-1. [Epub ahead of print]

PMID: 28620754

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  1. Comparison of 6-mm Versus 8-mm-Tip Cryoablation Catheter for the Treatment of Atrioventricular Nodal Reentrant Tachycardia in Children: A Prospective Study.

Tuzcu V, Gul EE, Karacan M, Kamali H, Celik N, Akdeniz C.

Pediatr Cardiol. 2017 Jun 13. doi: 10.1007/s00246-017-1648-z. [Epub ahead of print]

PMID: 28612086

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Select item 28659645

 

  1. Genotype-Phenotype Correlation of SCN5A Mutation for the Clinical and Electrocardiographic Characteristics of Probands With Brugada Syndrome: A Japanese Multicenter Registry.

Yamagata K, Horie M, Aiba T, Ogawa S, Aizawa Y, Ohe T, Yamagishi M, Makita N, Sakurada H, Tanaka T, Shimizu A, Hagiwara N, Kishi R, Nakano Y, Takagi M, Makiyama T, Ohno S, Fukuda K, Watanabe H, Morita H, Hayashi K, Kusano K, Kamakura S, Yasuda S, Ogawa H, Miyamoto Y, Kapplinger JD, Ackerman MJ, Shimizu W.

Circulation. 2017 Jun 6;135(23):2255-2270. doi: 10.1161/CIRCULATIONAHA.117.027983. Epub 2017 Mar 24.

PMID: 28341781

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  1. “Chaotic Arrhythmia” During Successful Resuscitation After Ingestion of Yew (Taxus baccata) Needles.

Zutter A, Hauri K, Evers KS, Uhde S, Fassl J, Reuthebuch OT, Berset A, Kühne M, Donner BC.

Pediatr Emerg Care. 2017 Jun 6. doi: 10.1097/PEC.0000000000001196. [Epub ahead of print]

PMID: 28590987

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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 Jun;173(6):1495-1501. doi: 10.1002/ajmg.a.38191. Epub 2017 Apr 10.

PMID: 28394409

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  1. Cost Analysis of Patients Referred for Inherited Heart Rhythm Disorder Evaluation.

Janzen ML, Cheung C, Sanatani S, Cunningham T, Kerr C, Steinberg C, Sherwin E, Arbour L, Deyell MW, Andrade JG, Lehman AM, Gula LJ, Krahn AD.

Can J Cardiol. 2017 Jun;33(6):814-821. doi: 10.1016/j.cjca.2016.12.009. Epub 2016 Dec 20.

PMID: 28347582

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  1. Exome sequencing identifies primary carnitine deficiency in a family with cardiomyopathy and sudden death.

Lahrouchi N, Lodder EM, Mansouri M, Tadros R, Zniber L, Adadi N, Clur SB, van Spaendonck-Zwarts KY, Postma AV, Sefiani A, Ratbi I, Bezzina CR.

Eur J Hum Genet. 2017 Jun;25(6):783-787. doi: 10.1038/ejhg.2017.22. Epub 2017 Mar 15.

PMID: 28295041

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  1. Vectorcardiography identifies patients with electrocardiographically concealed long QT syndrome.

Cortez D, Bos JM, Ackerman MJ.

Heart Rhythm. 2017 Jun;14(6):894-899. doi: 10.1016/j.hrthm.2017.03.003. Epub 2017 Mar 6.

PMID: 28279743

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  1. Single-incision percutaneous pericardial ICD lead placement in a piglet model.

Clark BC, Opfermann JD, Davis TD, Krieger A, Berul CI.

J Cardiovasc Electrophysiol. 2017 Jun 1. doi: 10.1111/jce.13263. [Epub ahead of print]

PMID: 28569424

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  1. KCNQ1 p.L353L affects splicing and modifies the phenotype in a founder population with long QT syndrome type 1.

Kapplinger JD, Erickson A, Asuri S, Tester DJ, McIntosh S, Kerr CR, Morrison J, Tang A, Sanatani S, Arbour L, Ackerman MJ.

J Med Genet. 2017 Jun;54(6):390-398. doi: 10.1136/jmedgenet-2016-104153. Epub 2017 Mar 6.

PMID: 28264985 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 Jun;40(6):745-747. doi: 10.1111/pace.13083. Epub 2017 May 16. No abstract available.

PMID: 28383202

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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 Jun;38(5):1049-1056. doi: 10.1007/s00246-017-1618-5. Epub 2017 Apr 29.

PMID: 28456831

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  1. Low Iron Stores in Otherwise Healthy Children Affect Electrocardiographic Markers of Important Cardiac Events.

Karadeniz C, Özdemir R, Demirol M, Katipoğlu N, Yozgat Y, Meşe T, Ünal N.

Pediatr Cardiol. 2017 Jun;38(5):909-914. doi: 10.1007/s00246-017-1596-7. Epub 2017 Mar 7.

PMID: 28271153

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  1. Catecholaminergic Polymorphic Ventricular Tachycardia.

Wall JJ, Iyer RV.

Pediatr Emerg Care. 2017 Jun;33(6):427-431. doi: 10.1097/PEC.0000000000001156.

PMID: 28570361

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  1. High School Cardiac Emergency Response Plans and Sudden Cardiac Death in the Young.

White MJ, Loccoh EC, Goble MM, Yu S, Odetola FO, Russell MW.

Prehosp Disaster Med. 2017 Jun;32(3):269-272. doi: 10.1017/S1049023X17000048. Epub 2017 Feb 20.

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Congenital EP Featured Articles of May 2017

Congenital and Pediatric Cardiac EP Reviews of May 2017 Manuscripts

 

Ventricular pacing in single ventricles-A bad combination.

Bulic A, Zimmerman FJ, Ceresnak SR, Shetty I, Motonaga KS, Freter A, Trela AV, Hanisch D, Russo L, Avasarala K, Dubin AM.

Heart Rhythm. 2017 Jun;14(6):853-857. doi: 10.1016/j.hrthm.2017.03.035.

PMID: 28528723

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Select item 27885498

 

Take Home Points:

 

  • For single ventricle patients with ≥50% ventricular pacing, there was a high rate of moderate-to-severe ventricular systolic dysfunction (68%), AV valve regurgitation (55%), and death/transplantation (23%) in this study.
  • Compared to controls that were matched for age, similar ventricular morphology, and follow up duration, all 3 of the above endpoints were much more frequently observed in those with chronic ventricular pacing vs non-paced controls

 

J MooreCommentary by Dr. Jeremy Moore (Los Angeles) Congenital and Pediatric Cardiac EP section editor:  This was a single center study from 2 institutions over a 25-year period (1990 – 2015) that included patients with single ventricle congenital heart disease and ≥ 50% ventricular pacing. Cases with ventricular pacing were matched to a non-paced single ventricle control group in a 1:2 fashion for age, gender, single ventricle morphology, and similar follow-up duration.

The authors noted similar baseline characteristics between cases and controls, including similar degrees of ventricular systolic function and AV valve regurgitation before pacing. Ventricular morphology was also similar and was most commonly related to HLHS (40%), followed by DILV (24%), TA (13%), and DORV (12%). The pacing indication was divided nearly equally between postoperative AV block and nonsurgical advanced second or third-degree AV block.

The main study findings were that patients exposed to ventricular pacing fared far worse than their non-paced controls. Patients in the paced group were more likely to develop moderate-to-severe ventricular systolic dysfunction (68% vs 15%), moderate-to-severe AV valve regurgitation (55% vs 8%), were more often treated with heart failure medication (65% vs 21%), and experienced transplant/death (23% vs 6%) vs controls over a mean of 6.6 years.

May ep 1

 

Red, severe; yellow, moderate; green, mild; blue, no ventricular systolic dysfunction

The authors concluded that single ventricle patients requiring ventricular pacing are at a much higher risk for developing moderate-to-severe ventricular systolic dysfunction and AV valve regurgitation compared to those who are not paced. There was a 5-fold increased risk for death or heart transplantation. These risks appeared to be independent of baseline characteristics and attributable solely to chronic ventricular pacing.

 

Arrhythmia burden and related outcomes in Eisenmenger syndrome.

Baskar S, Horne P, Fitzsimmons S, Khoury PR, Vettukattill J, Miwa K, Awake T, Spence M, Sakazaki H, Veldtman G.

Congenit Heart Dis. 2017 May 19. doi: 10.1111/chd.12481. [Epub ahead of print]

PMID: 28523857

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Select item 28523852

 

Take Home Points:

 

  • AV valve regurgitation and prolonged QRS duration appear to be two important factors associated with arrhythmia in adult patients with Eisenmenger syndrome.
  • Arrhythmias occurred in 18% of Eisenmenger patients in this study, with the most common mechanisms being supraventricular tachycardia (57%), followed by NSVT (21%) and VT (21%).
  • The most important factor associated with sudden death by multivariate analysis in this study was prior therapy with antiarrhythmic drugs.

 

Commentary by Dr. Jeremy Moore (Los Angeles) Congenital and Pediatric Cardiac EP section editor:  This was a multicenter retrospective study of arrhythmias in adult Eisenmenger patients (≥ 18 years of age) at 2 centers in the U.K. and one in Japan. The authors were able to identify a total of 167 patients with Eisenmenger syndrome who had complete follow-up data. Clinical tachycardias were classified as NSVT, VT, or SVT (the latter further characterized as AF, AF/AFL, and paroxysmal SVT); whereas atrial and ventricular ectopy were excluded from the analysis. The authors looked at univariate and multivariate predictors of 3 major predefined outcomes that included 1) arrhythmia, 2) overall mortality, and 3) sudden death.

 

Arrhythmia occurrence was relatively common, being observed in 30 (18%) patients and were most frequently classified as SVT in 57%, followed by NSVT/VT in 42%. Predictors of arrhythmia included AV valve regurgitation, prolonged QRS duration, and absence of Downs syndrome.

May ep 2

 

Of the total cohort, 21 patients died and 8 patients died suddenly (3 with documented VF at the time of the arrest). Predictors of overall mortality included any history of arrhythmia, antiarrhythmic drug therapy, and higher NYHA class. Predictors of sudden death included documented arrhythmia and need for antiarrhythmic drug therapy. In the multivariate analysis, only antiarrhythmic drug therapy predicted sudden death.

May EP 3

 

 DTT = disease targeting therapy (i.e. advanced pulmonary hypertension therapy)

 

The authors concluded that arrhythmias in this group of ACHD patients are common, affecting approximately one-fifth of patients and that AV valve regurgitation and prolonged QRS duration were associated. Likewise, arrhythmias themselves were associated with all-cause mortality and sudden death.

 

These study findings are not novel, but do strengthen prior observations regarding risk for sudden death in the adult Eisenmenger population. The largest report evaluating this risk previously was the case-control study from the Netherlands in 2012 which included 33 cases of sudden death.* This prior study by Koyak and colleagues only reported bivariate associations, but found that heart failure symptoms (OR 2.7), heart failure medication (OR 3.), antiarrhythmic drug therapy (OR 3.4), QRS duration (1.34 per 10-ms increase), impaired systemic ventricular function (OR 8.2), impaired subpulmonary ventricular function (OR 5.0) and increased creatinine (OR 1.12 per 10 µMol increase) were all associated with sudden death. Clearly, more data on the risk for sudden death in this population is needed, but a relationship with prior arrhythmic event and especially antiarrhythmic drug therapy seems particularly important based on data from this smaller, but updated report.

 

*Koyak et al. Circulation 2012; 126(16):1944-1954

 

CHD EP May 2017

 

  1. Congenital heart block and immune mediated sensorineural hearing loss: possible cross reactivity of immune response.

Bason C, Pagnini I, Brucato A, Maestroni S, Puccetti A, Lunardi C, Cimaz R.

Lupus. 2017 Jul;26(8):835-840. doi: 10.1177/0961203316682099. Epub 2016 Dec 5.

PMID: 27913750

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  1. Sudden death in athletes.

Corrado D, Zorzi A.

Int J Cardiol. 2017 Jun 15;237:67-70. doi: 10.1016/j.ijcard.2017.03.034. Epub 2017 Mar 10.

PMID: 28318658

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  1. [Pulmonary revalvulation and rhythmologenic risks in patients with repaired tetralogy of Fallot].

Pilato R, Lacroix D, Domanski O, Godart F.

Presse Med. 2017 Jun 2. pii: S0755-4982(17)30112-4. doi: 10.1016/j.lpm.2017.02.006. [Epub ahead of print] French.

PMID: 28583744

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  1. Cardiac Arrhythmias in Adults with Congenital Heart Disease.

Balaji S, Mandapati R, Shivkumar K.

Card Electrophysiol Clin. 2017 Jun;9(2):xv-xvi. doi: 10.1016/j.ccep.2017.04.001. No abstract available.

PMID: 28457248

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  1. Arrhythmia Surgery for Adults with Congenital Heart Disease.

Deal BJ, Mavroudis C.

Card Electrophysiol Clin. 2017 Jun;9(2):329-340. doi: 10.1016/j.ccep.2017.02.014. Review.

PMID: 28457246

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  1. Cardiac Arrhythmias in Adults with Congenital Heart Disease: Pacemakers, Implantable Cardiac Defibrillators, and Cardiac Resynchronization Therapy Devices.

Cecchin F, Halpern DG.

Card Electrophysiol Clin. 2017 Jun;9(2):319-328. doi: 10.1016/j.ccep.2017.02.013. Epub 2017 Mar 31. Review.

PMID: 28457245

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  1. Drug Therapy in Adult Congenital Heart Disease.

Contractor T, Levin V, Mandapati R.

Card Electrophysiol Clin. 2017 Jun;9(2):295-309. doi: 10.1016/j.ccep.2017.02.011. Review.

PMID: 28457243

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  1. Sudden Cardiac Death in Adult Congenital Heart Disease.

Ávila P, Chaix MA, Mondésert B, Khairy P.

Card Electrophysiol Clin. 2017 Jun;9(2):225-234. doi: 10.1016/j.ccep.2017.02.003. Epub 2017 Mar 18. Review.

PMID: 28457237

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  1. Ventricular Arrhythmias in Adult Congenital Heart Disease: Mechanisms, Diagnosis, and Clinical Aspects.

Sathananthan G, Harris L, Nair K.

Card Electrophysiol Clin. 2017 Jun;9(2):213-223. doi: 10.1016/j.ccep.2017.02.004. Epub 2017 Mar 18. Review.

PMID: 28457236

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  1. Supraventricular Tachycardia in Adult Congenital Heart Disease: Mechanisms, Diagnosis, and Clinical Aspects.

Janson CM, Shah MJ.

Card Electrophysiol Clin. 2017 Jun;9(2):189-211. doi: 10.1016/j.ccep.2017.02.005. Review.

PMID: 28457235

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  1. Bradyarrhythmias in Congenital Heart Disease.

Carlson SK, Patel AR, Chang PM.

Card Electrophysiol Clin. 2017 Jun;9(2):177-187. doi: 10.1016/j.ccep.2017.02.002. Epub 2017 Mar 22. Review.

PMID: 28457234

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  1. Introduction to the Congenital Heart Defects: Anatomy of the Conduction System.

Moore JP, Aboulhosn JA.

Card Electrophysiol Clin. 2017 Jun;9(2):167-175. doi: 10.1016/j.ccep.2017.02.001. Epub 2017 Mar 14. Review.

PMID: 28457233

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  1. Ventricular pacing in single ventricles-A bad combination.

Bulic A, Zimmerman FJ, Ceresnak SR, Shetty I, Motonaga KS, Freter A, Trela AV, Hanisch D, Russo L, Avasarala K, Dubin AM.

Heart Rhythm. 2017 Jun;14(6):853-857. doi: 10.1016/j.hrthm.2017.03.035.

PMID: 28528723

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  1. Predictors of acute inefficacy and the radiofrequency energy time required for cavotricuspid isthmus-dependent atrial flutter ablation.

Pérez-Rodon J, Rodriguez-García J, Sarrias-Merce A, Rivas-Gandara N, Roca-Luque I, Francisco-Pascual J, Santos-Ortega A, Martín-Sánchez G, Ferreira-González I, Rodríguez-Palomares J, Evangelista-Masip A, García-Dorado D, Moya-Mitjans À.

J Interv Card Electrophysiol. 2017 Jun;49(1):83-91. doi: 10.1007/s10840-017-0232-z. Epub 2017 Mar 6.

PMID: 28265782

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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 Jun;38(5):1004-1009. doi: 10.1007/s00246-017-1608-7. Epub 2017 Apr 3.

PMID: 28374048

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  1. Permanent nonselective His bundle pacing in an adult with L-transposition of the great arteries and complete AV block.

Kean AC, Kay WA, Patel JK, Miller JM, Dandamudi G.

Pacing Clin Electrophysiol. 2017 May 24. doi: 10.1111/pace.13107. [Epub ahead of print]

PMID: 28543795

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  1. Analysis of adults with congenital heart disease presenting to pediatric emergency departments with arrhythmias.

Mohan S, Moffett BS, Lam W, de la Uz C, Miyake C, Valdes SO, Kim JJ.

Congenit Heart Dis. 2017 May 22. doi: 10.1111/chd.12478. [Epub ahead of print]

PMID: 28544787

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  1. Analysis of Screening Electrocardiogram for the Subcutaneous Defibrillator in Adults with Congenital Heart Disease.

Thomas VC, Peterson M, McDaniel M, Restrepo H, Rothman A, Jain A.

Pediatr Cardiol. 2017 May 22. doi: 10.1007/s00246-017-1635-4. [Epub ahead of print]

PMID: 28534239

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  1. Arrhythmia burden and related outcomes in Eisenmenger syndrome.

Baskar S, Horne P, Fitzsimmons S, Khoury PR, Vettukattill J, Niwa K, Agaki T, Spence M, Sakazaki H, Veldtman G.

Congenit Heart Dis. 2017 May 19. doi: 10.1111/chd.12481. [Epub ahead of print]

PMID: 28523857

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  1. Force-Sensing Catheters During Pediatric Radiofrequency Ablation: The FEDERATION Study.

Dalal AS, Nguyen HH, Bowman T, Van Hare GF, Avari Silva JN.

J Am Heart Assoc. 2017 May 17;6(5). pii: e005772. doi: 10.1161/JAHA.117.005772.

PMID: 28515113 Free Article

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  1. Hypoplastic Left Heart Syndrome with Congenital Complete Heart Block.

Al-Kubaisi M, Aly SA, Mohammad Nijres B, Awad S.

Pediatr Cardiol. 2017 May 17. doi: 10.1007/s00246-017-1631-8. [Epub ahead of print]

PMID: 28512719

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  1. Epicardial cardioverter-defibrillator implantation in a 4-month-old infant bridged to heart transplantation.

Carro C, Cereda AF, Annoni G, Marianeschi SM.

Interact Cardiovasc Thorac Surg. 2017 May 16. doi: 10.1093/icvts/ivx129. [Epub ahead of print]

PMID: 28510647

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  1. Conventional and right-sided screening for subcutaneous ICD in a population with congenital heart disease at high risk of sudden cardiac death.

Alonso P, Osca J, Rueda J, Cano O, Pimenta P, Andres A, Sancho MJ, Martinez L.

Ann Noninvasive Electrocardiol. 2017 May 15. doi: 10.1111/anec.12461. [Epub ahead of print]

PMID: 28508439

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  1. Complete heart block complicating the acute phase of respiratory syncytial virus bronchiolitis.

Karatza AA, Kiaffas M, Rammos S.

Pediatr Pulmonol. 2017 May 9. doi: 10.1002/ppul.23714. [Epub ahead of print]

PMID: 28486756

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  1. Utility of Post-Mortem Genetic Testing in Cases of Sudden Arrhythmic Death Syndrome.

Lahrouchi N, Raju H, Lodder EM, Papatheodorou E, Ware JS, Papadakis M, Tadros R, Cole D, Skinner JR, Crawford J, Love DR, Pua CJ, Soh BY, Bhalshankar JD, Govind R, Tfelt-Hansen J, Winkel BG, van der Werf C, Wijeyeratne YD, Mellor G, Till J, Cohen MC, Tome-Esteban M, Sharma S, Wilde AAM, Cook SA, Bezzina CR, Sheppard MN, Behr ER.

J Am Coll Cardiol. 2017 May 2;69(17):2134-2145. doi: 10.1016/j.jacc.2017.02.046.

PMID: 28449774 Free PMC Article

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  1. Outcomes of Direct Current Cardioversion in Adults With Congenital Heart Disease.

Egbe AC, Asirvatham SJ, Connolly HM, Kapa S, Desimone CV, Vaidya VR, Deshmukh AJ, Khan AR, McLeod CJ, Melduni RM, Ammash NM.

Am J Cardiol. 2017 May 1;119(9):1468-1472. doi: 10.1016/j.amjcard.2017.01.018. Epub 2017 Feb 9.

PMID: 28262200

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  1. Increased risk of thromboembolic events in adult congenital heart disease patients with atrial tachyarrhythmias.

Masuda K, Ishizu T, Niwa K, Takechi F, Tateno S, Horigome H, Aonuma K.

Int J Cardiol. 2017 May 1;234:69-75. doi: 10.1016/j.ijcard.2017.02.004. Epub 2017 Feb 5.

PMID: 28209388

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  1. Acute Cardiac MRI Assessment of Radiofrequency Ablation Lesions for Pediatric Ventricular Arrhythmia: Feasibility and Clinical Correlation.

Grant EK, Berul CI, Cross RR, Moak JP, Hamann KS, Sumihara K, Cronin I, O’Brien KJ, Ratnayaka K, Hansen MS, Kellman P, Olivieri LJ.

J Cardiovasc Electrophysiol. 2017 May;28(5):517-522. doi: 10.1111/jce.13197. Epub 2017 Mar 28.

PMID: 28245348

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  1. Repetitive ventricular tachycardia in a syncopal child: Cause or incidental finding ?

Massin M, Jacquemart C, Khaldi K.

Ann Noninvasive Electrocardiol. 2017 May 30. doi: 10.1111/anec.12472. [Epub ahead of print]

PMID: 28557290

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  1. Family Screening for Brugada Syndrome in Asymptomatic Young Patients. Is it Better not to Know?

Gonzalez Corcia MC, Brugada P.

Pediatr Cardiol. 2017 May 26. doi: 10.1007/s00246-017-1637-2. [Epub ahead of print] No abstract available.

PMID: 28550419

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  1. Permanent nonselective His bundle pacing in an adult with L-transposition of the great arteries and complete AV block.

Kean AC, Kay WA, Patel JK, Miller JM, Dandamudi G.

Pacing Clin Electrophysiol. 2017 May 24. doi: 10.1111/pace.13107. [Epub ahead of print]

PMID: 28543795

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  1. Analysis of adults with congenital heart disease presenting to pediatric emergency departments with arrhythmias.

Mohan S, Moffett BS, Lam W, de la Uz C, Miyake C, Valdes SO, Kim JJ.

Congenit Heart Dis. 2017 May 22. doi: 10.1111/chd.12478. [Epub ahead of print]

PMID: 28544787

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  1. The effects of gender on electrical therapies for the heart: physiology, epidemiology, and access to therapies: A report from the XII Congress of the Italian Association on Arrhythmology and Cardiostimulation (AIAC).

Boriani G, Lorenzetti S, Cerbai E, Oreto G, Bronzetti G, Malavasi VL, Biffi A, Padeletti L, Botto G, Diemberger I.

Europace. 2017 May 19. doi: 10.1093/europace/eux068. [Epub ahead of print]

PMID: 28525585

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  1. Cost of Unnecessary Testing in the Evaluation of Pediatric Syncope.

Redd C, Thomas C, Willis M, Amos M, Anderson J.

Pediatr Cardiol. 2017 May 18. doi: 10.1007/s00246-017-1625-6. [Epub ahead of print]

PMID: 28523341

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  1. Pacing Induced Ventricular Dysfunction in a Child: Improvement with Reduction in Paced Rate.

Garg S, Balaji S.

Pediatr Cardiol. 2017 May 17. doi: 10.1007/s00246-017-1629-2. [Epub ahead of print]

PMID: 28512720

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  1. Propranolol Versus Digoxin in the Neonate for Supraventricular Tachycardia (from the Pediatric Health Information System).

Bolin EH, Lang SM, Tang X, Collins RT.

Am J Cardiol. 2017 May 15;119(10):1605-1610. doi: 10.1016/j.amjcard.2017.02.017. Epub 2017 Mar 1.

PMID: 28363353

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  1. A Mutation in the G-Protein Gene GNB2 Causes Familial Sinus Node and Atrioventricular Conduction Dysfunction.

Stallmeyer B, Kuß J, Kotthoff S, Zumhagen S, Vowinkel K, Rinné S, Matschke LA, Friedrich C, Schulze-Bahr E, Rust S, Seebohm G, Decher N, Schulze-Bahr E.

Circ Res. 2017 May 12;120(10):e33-e44. doi: 10.1161/CIRCRESAHA.116.310112. Epub 2017 Feb 20.

PMID: 28219978

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  1. Efficacy of Flecainide in the Treatment of Catecholaminergic Polymorphic Ventricular Tachycardia: A Randomized Clinical Trial.

Kannankeril PJ, Moore JP, Cerrone M, Priori SG, Kertesz NJ, Ro PS, Batra AS, Kaufman ES, Fairbrother DL, Saarel EV, Etheridge SP, Kanter RJ, Carboni MP, Dzurik MV, Fountain D, Chen H, Ely EW, Roden DM, Knollmann BC.

JAMA Cardiol. 2017 May 10. doi: 10.1001/jamacardio.2017.1320. [Epub ahead of print]

PMID: 28492868

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  1. Successful catheter ablation of ventricular ectopy in a young patient with implanted Melody valve.

Jan M, Žižek D, Mazic U, Klemen L, Mijovski G, Peichl P.

Europace. 2017 May 4. doi: 10.1093/europace/eux033. [Epub ahead of print] No abstract available.

PMID: 28472281

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  1. Phase-contrast magnet resonance imaging reveals regional, transmural, and base-to-apex dispersion of mechanical dysfunction in patients with long QT syndrome.

Brado J, Dechant MJ, Menza M, Komancsek A, Lang CN, Bugger H, Foell D, Jung BA, Stiller B, Bode C, Odening KE.

Heart Rhythm. 2017 May 4. pii: S1547-5271(17)30578-7. doi: 10.1016/j.hrthm.2017.04.045. [Epub ahead of print]

PMID: 28479515

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  1. Efficacy of Intravenous Sotalol for Treatment of Incessant Tachyarrhythmias in Children.

Li X, Zhang Y, Liu H, Jiang H, Ge H, Zhang Y.

Am J Cardiol. 2017 May 1;119(9):1366-1370. doi: 10.1016/j.amjcard.2017.01.034. Epub 2017 Feb 10.

PMID: 28283175 Free Article

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  1. Intraoperative Three-Dimensional Mapping of Supraventricular Tachycardia in a Young Fontan Patient.

Perry JC, Williams MR, Shepard S, Bratincsak A, Murphy C, Murthy R, Ebrahim M, Lamberti JJ.

Ann Thorac Surg. 2017 May;103(5):e453-e456. doi: 10.1016/j.athoracsur.2016.10.069.

PMID: 28431725

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  1. Retrosternal Implantation of the Cardioverter-Defibrillator Lead in an Infant.

Hata H, Sumitomo N, Nakai T, Amano A.

Ann Thorac Surg. 2017 May;103(5):e449-e451. doi: 10.1016/j.athoracsur.2016.10.063.

PMID: 28431724

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  1. Wolff-Parkinson-White syndrome: a single exercise stress test might be misleading.

Salavitabar A, Silver ES, Liberman L.

Cardiol Young. 2017 May;27(4):804-807. doi: 10.1017/S1047951116002055. Epub 2016 Nov 21.

PMID: 27869041

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  1. A Toddler With Central Venous Catheter Develops Tachycardia.

Shapiro DR, Dudek CJ, Ho JG.

Clin Pediatr (Phila). 2017 May;56(5):496-499. doi: 10.1177/0009922816661331. Epub 2016 Jul 26. No abstract available.

PMID: 27466077

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  1. Junctional ectopic tachycardia in late period after early postoperative complete atrioventricular block: Messenger of return to normal sinus rhythm? : Explanation with four case series.

Ozyilmaz I, Ergul Y, Ozyilmaz S, Guzeltas A.

J Electrocardiol. 2017 May – Jun;50(3):378-382. doi: 10.1016/j.jelectrocard.2017.01.015. Epub 2017 Feb 1.

PMID: 28189280

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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.

 

EP1

 

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.

EP6

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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Select item 28366171

 

  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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Select item 28371362

 

  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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Select item 28441821

 

  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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Select item 27167242

 

  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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Select item 28293976

 

  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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Select item 28233036

 

  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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Select item 28032672

 

  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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Select item 28093463

 

  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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Select item 27987225

 

  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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Select item 28394251

 

  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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Select item 28108573

 

  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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Select item 27827522

 

  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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Select item 28361263

 

  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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Select item 28239753

 

  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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Select item 28184976

 

  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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Select item 28212163

 

  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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Congenital EP Featured Articles of March 2017

Congenital and Pediatric Cardiac EP Reviews of March 2017 Publications

 

Mechanisms and predictors of recurrent tachycardia after catheter ablation for d-transposition of the great arteries after the Mustard or Senning operation.

Gallotti RG, Madnawat H, Shannon KM, Aboulhosn JA, Nik-Ahd F, Moore JP.

Heart Rhythm. 2017 Mar;14(3):350-356. doi: 10.1016/j.hrthm.2016.11.031. Epub 2016 Nov 28.

PMID: 27908766

 

Take Home Points:

 

  • Intra-atrial reentrant remains the most common form of supraventricular tachyarrhythmia seen in D-transposition of the great arteries after Senning or Mustard operation
  • Recurrence after ablation occurs in nearly 1/3 of patients within less than 2 years
  • Of importance, recurrence of arrhythmias is often is due to an alternative arrhythmia mechanism rather than the prior ablated substrate
  • Clearly, the risk of arrhythmia recurrence differs between surgical technique with higher recurrent in patients who have undergone Senning operation.
  • Overall success rates after repeated ablation with short-term follow-up are very high (96%).

 

A PatelCommentary from Dr. Akash Patel (San Francisco), section editor of Congenital Electrophysiology Journal Watch:  This large single center study from UCLA aimed to improve our understanding of supraventricular tachyarrhythmia mechanisms after Senning and Mustard operations in patients with D-transposition of the great arteries and identify predictors for recurrence. Previous studies have looked at identifying arrhythmia mechanisms and ablation outcomes for arrhythmias after Mustard or Senning operations.  However, there is limited data regarding the difference between these 2 surgical techniques on mechanisms of arrhythmias and ablation outcomes.

 

This study included 28 patients who underwent 38 catheter ablations from 2004 – 2016. The majority underwent Mustard operation (64%).  89% were treated with antiarrhythmic drugs prior to ablation and 89% achieved initial ablation success.

 

At the time of initial ablation, the majority of arrhythmias were due to intra-atrial reentrant (70%). See Table and Figure Below.

EP media 1 ep media 2

Diagram depicting the sites of acutely successful catheter ablation for the initial catheter ablation procedures in a modified left anterior oblique view. For intra-atrial reentrant tachycardia (IART) that was successfully ablated at the cavotricuspid isthmus (CTI), the number of patients after either the Senning or the Mustard operation is totaled in parentheses. The systemic venous atrium is depicted in blue and the pulmonary venous atrium in pink. M = Mustard; S = Senning. IART, Focal atrial tachycardia, Atrioventricular nodal reentrant tachycardia.

 

Recurrence was seen in 9 patients (32%) with 7 (78%) achieving procedural success. Most recurrences were seen after Senning operation (60% vs. 17%, p =0.34).  In addition, no other risk factor including patients characteristics, arrhythmia mechanism, procedural characteristics were found to be predictive of recurrence.

 

At the time of repeat ablation, most arrhythmias mechanisms where due to intra-atrial reentrant (54%) but compromised a smaller portion compared to the initial procedure. See Table and Figure Below.

ep media 3

ep media 4
Of significant importance was that 10 of 13 (77%) arrhythmias seen during repeat procedure for recurrence were not seen in initial procedure. In particular, Senning operation conferred a higher risk of procedural recurrence with the location of the arrhythmia occurring in the pulmonary venous atrium in 4 out of 6 patients. Of the 3 cases identified at the prior procedure, 2 required ablation in the pulmonary venous atrium for the same mechanism approached only via the systemic venous atrium to achieve procedural success. Overall, 96% of patients remained arrhythmia-free after their final procedure with a median follow-up of 1.3 years.
Prior studies and this continue to demonstrate the significance of supraventricular tachyarrhythmia associated with adult with congenital heart disease.  This study highlights the importance of variations in surgical technique (Senning vs Mustard Operation) on arrhythmia mechanism.  In addition, previously perceived “failed ablations” more often are due to additional arrhythmia mechanisms.  These findings may warrant a more comprehensive approach at the initial procedure.

 

Genotypic and phenotypic predictors of complete heart block and recovery of conduction after surgical repair of congenital heart disease.

Murray LE, Smith AH, Flack EC, Crum K, Owen J, Kannankeril PJ.

Heart Rhythm. 2017 Mar;14(3):402-409. doi: 10.1016/j.hrthm.2016.11.010. Epub 2016 Nov 5.

PMID:27826129

 

Take Home Points:

 

  • Incidence of permanent post-operative complete heart block after congenital heart surgery has remained constant over the last decades around 1-3%.
  • In addition to direct trauma, inflammation, or edema to the AV node there may potential intrinsic factors that may place patients at risk for complete heart block.
  • A gap junction protein, connexin-40, has been implicated in a familial form of complete heart block when malformed due to genetic mutations.
  • A common missense mutation polymorphism for GJA5 (genotype TT) that encodes connexin-40 was associated with a 2-fold increase in the risk of post-operative complete heart block that is similar to the risk that VSD closure confers.
  • Junctional arrhythmia and intermittent conduction were predictive of AV node recovery.
  • Early recovery of AV node function can still result in episodes of higher degree AV block during the post-operative period that necessitates pacemaker therapy.
  • Permanent AV block can rarely have late return of AV conduction.

 

Commentary from Dr. Akash Patel (San Francisco), section editor of Congenital Electrophysiology Journal Watch:  This large single center study from Vanderbilt aimed to improve our understanding of post-operative complete heart block with the assessment of a genetic polymorphism in the gap junction protein, connexin-40, in addition to typical risk factors including diagnosis, preoperative and operative characteristics.

 

This study included 1199 patients who underwent one or more congenital heart surgeries with cardiopulmonary bypass at Vanderbilt from 2007 to 2015.  The case mix was standard for congenital heart surgical program with the 5 most common diagnoses were tetralogy of Fallot (11.3%), ventricular septal defect (10.4%), hypoplastic left heart (9.2%), atrial septal defect (8.3%) and complete AV canal defect (7.3%). The primary surgical procedure involved a ventricular septal defect closure in 41% of the cases.  There were 56 patients (4.7%) who had complete heart block in the operating room or within the first 48 hours.

 

Perioperative risk factors associated with complete heart block included younger age, small size (weight), pre-operative digoxin use, and earlier surgical era. See Table below.

ep media 5Intraoperative risk factors associated with complete heart block included longer cardiopulmonary bypass time, longer aortic cross clamp time, and procedures with ventricular septal defect closure based on univariate analysis. See Table below.

 

 

ep media 6
Post-operative risk factors associated with complete heart block included use of dopamine, use of milrinone, lower pO2, higher lactate on admission to CICU, higher ionized calcium on admission to CICU, need for post-operative ecmo, longer duration of mechanical ventilation, longer CU and hospital length of stay, and increased mortality based on univariate analysis. See Table below.

ep media 7
Genetic risk factors of GJA5 rs10465885 TT genotype polymorphism was associated with increased risk of complete heart block based on univariate analysis.  There were 3 genotype polymorphisms seen in the total cohort C/C (n = 245, 20%), T/C (n=607, 51%), and T/T (n=347, 29%). Overall risk for complete heart block by genotype was 2.8% for C/C, 3.9% for C/T, and 7.2% for T/T. See table below.

ep media 8
On multivariate analysis, use of pre-op digoxin, procedure with VSD closure, and prolonged aortic cross clamp time conferred increased risk. In addition, the presence of the GJA5 polymorphism with TT genotype carried a 2.1-fold risk comparable to the 2.2-fold risk with VSD closure.  See table below.

ep media 9

Recovery of AV node function was seen in 35 of the 56 patients (63%) and pacemaker free recovery was seen in 27 patients (48%) with a median recovery time of 3 days.  Based on assessment of the same pre-operative, intraoperative, post-operative, and genetic risk factors; only pre-operative ACE inhibitor use, intermittent AV conduction, and junctional acceleration (junctional ectopic tachycardia and accelerated junctional rhythm) were associated with recovery of AV node function. Multivariate analysis demonstrated only intermittent AV conduction (Adjusted OR of 9.1) and junctional acceleration (Adjusted OR of 4) were associate with recovery with positive predictive value of 89%.  Of interest were 8 patients with recovery had a pacemaker implanted but 6 (75%) had evidence of 2nd degree or higher AV block. Three had evidence of transient high grade AV block and 3 had evidence of residual conduction disease with first degree and periods of second degree AV block.  All patients received their devices between 7 and 21 days post-operatively.

 

Permanent heart block was seen in 1.4% of the total group. Of interest was late recurrent of AV conduction was seen in 1 patient noted at 101 days post-operative who had pacemaker implanted after nearly 2 weeks of complete heart block.

 

This study raises the intriguing possibility of genetic risks factors for post-operative AV block in addition the conventional wisdom of trauma from surgery.  Further investigation looking at other intrinsic factors may provide for insights into patient specific vulnerability for AV block.

 

 

 

Additional Articles.

 

  1. 2017 ACC/AHA/HRS Guideline for the Evaluation and Management of Patients with Syncope: A Report of the American College of Cardiology/American HeartAssociation Task Force on Clinical Practice Guidelines, and the HeartRhythm Society.

Shen WK, Sheldon RS, Benditt DG, Cohen MI, Forman DE, Goldberger ZD, Grubb BP, Hamdan MH, Krahn AD, Link MS, Olshansky B, Raj SR, Sandhu RK, Sorajja D, Sun BC, Yancy CW.

Heart Rhythm. 2017 Mar 9. pii: S1547-5271(17)30297-7. doi: 10.1016/j.hrthm.2017.03.004. [Epub ahead of print] No abstract available.

PMID: 28286247

 

  1. Genotype-Phenotype Correlation of SCN5AMutation for the Clinical and Electrocardiographic Characteristics of Probands with Brugada Syndrome: A Japanese Multicenter Registry.

Yamagata K, Horie M, Aiba T, Ogawa S, Aizawa Y, Ohe T, Yamagishi M, Makita N, Sakurada H, Tanaka T, Shimizu A, Hagiwara N, Kishi R, Nakano Y, Takagi M, Makiyama T, Ohno S, Fukuda K, Watanabe H, Morita H, Hayashi K, Kusano K, Kamakura S, Yasuda S, Ogawa H, Miyamoto Y, Kapplinger JD, Ackerman MJ, Shimizu W.

Circulation. 2017 Mar 24. pii: CIRCULATIONAHA.117.027983. doi: 10.1161/CIRCULATIONAHA.117.027983. [Epub ahead of print]

PMID:28341781

 

CHD EP March 2017

 

  1. Sudden Cardiac Death in Pre-Excitation and Wolff-Parkinson-White: Demographic and Clinical Features.

Finocchiaro G, Papadakis M, Behr ER, Sharma S, Sheppard M.

J Am Coll Cardiol. 2017 Mar 28;69(12):1644-1645. doi: 10.1016/j.jacc.2017.01.023. No abstract available.

PMID:

 

28335848

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Huchet F, Kyndt F, Barc J, Thollet A, Charpentier F, Redon R, Schott JJ, le Marec H, Probst V, Gourraud JB.

J Am Coll Cardiol. 2017 Mar 28;69(12):1642-1643. doi: 10.1016/j.jacc.2017.01.030. No abstract available.

PMID:

 

28335847

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Combes N, Derval N, Hascoët S, Zhao A, Amet D, Le Bloa M, Maltret A, Heitz F, Thambo JB, Marijon E.

Arch Cardiovasc Dis. 2017 Mar 27. pii: S1875-2136(17)30046-3. doi: 10.1016/j.acvd.2017.01.007. [Epub ahead of print] Review.

PMID:

 

28359691

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  1. Whole exome sequencing with genomic triangulation implicates CDH2-encoded N-cadherin as a novel pathogenic substrate for arrhythmogenic cardiomyopathy.

Turkowski KL, Tester DJ, Bos JM, Haugaa KH, Ackerman MJ.

Congenit Heart Dis. 2017 Mar 21. doi: 10.1111/chd.12462. [Epub ahead of print]

PMID:

 

28326674

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  1. Current Interventional and Surgical Management ofCongenital Heart Disease: Specific Focus on ValvularDisease and Cardiac Arrhythmias.

Holst KA, Said SM, Nelson TJ, Cannon BC, Dearani JA.

Circ Res. 2017 Mar 17;120(6):1027-1044. doi: 10.1161/CIRCRESAHA.117.309186.

PMID:

 

28302746

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  1. Localized Atrial Reentrant Tachycardia in Tetralogy of Fallot: Ultra-High Resolution Mapping and Termination by Non-Propagated Atrial Pacing Stimulus.

Lee A, Kite J, Davison O, Haqqani HM.

Heart Rhythm. 2017 Mar 16. pii: S1547-5271(17)30300-4. doi: 10.1016/j.hrthm.2017.03.007. [Epub ahead of print] No abstract available.

PMID:

 

28323170

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  1. 2017 ACC/AHA/HRS Guideline for the Evaluation and Management of Patients with Syncope: A Report of the American College of Cardiology/AmericanHeartAssociation Task Force on Clinical Practice Guidelines, and the Heart Rhythm Society.

Shen WK, Sheldon RS, Benditt DG, Cohen MI, Forman DE, Goldberger ZD, Grubb BP, Hamdan MH, Krahn AD, Link MS, Olshansky B, Raj SR, Sandhu RK, Sorajja D, Sun BC, Yancy CW.

Heart Rhythm. 2017 Mar 9. pii: S1547-5271(17)30297-7. doi: 10.1016/j.hrthm.2017.03.004. [Epub ahead of print] No abstract available.

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28286247

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  1. 2017 ACC/AHA/HRS Guideline for the Evaluation and Management of Patients With Syncope: Executive Summary: A Report of the American College of Cardiology/AmericanHeartAssociation Task Force on Clinical Practice Guidelines, and the Heart Rhythm Society.

Shen WK, Sheldon RS, Benditt DG, Cohen MI, Forman DE, Goldberger ZD, Grubb BP, Hamdan MH, Krahn AD, Link MS, Olshansky B, Raj SR, Sandhu RK, Sorajja D, Sun BC, Yancy CW.

Heart Rhythm. 2017 Mar 9. pii: S1547-5271(17)30298-9. doi: 10.1016/j.hrthm.2017.03.005. [Epub ahead of print] No abstract available.

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28286246

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  1. Pacing as a Treatment for Reflex-Mediated (Vasovagal, Situational, or Carotid Sinus Hypersensitivity) Syncope: A Systematic Review for the 2017 ACC/AHA/HRS Guideline for the Evaluation and Management of Patients with Syncope: A Report of the American College of Cardiology/AmericanHeartAssociation Task Force on Clinical Practice Guidelines and the Heart Rhythm Society.

Varosy PD, Chen LY, Miller AL, Noseworthy PA, Slotwiner DJ, Thiruganasambandamoorthy V.

Heart Rhythm. 2017 Mar 9. pii: S1547-5271(17)30299-0. doi: 10.1016/j.hrthm.2017.03.006. [Epub ahead of print]

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28286245

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Midha D, Chen Z, Jones DG, Williams HJ, Lascelles K, Jarman J, Clague J, Till J, Dimopoulos K, Babu-Narayan SV, Markides V, Gatzoulis MA, Wong T.

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Wijnmaalen AP, Zeppenfeld K.

Card Electrophysiol Clin. 2017 Mar;9(1):107-117. doi: 10.1016/j.ccep.2016.10.008. Epub 2016 Dec 24. Review.

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28167078

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Clin Cardiol. 2017 Mar;40(3):177-185. doi: 10.1002/clc.22666. Epub 2017 Mar 8. Review.

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Echocardiography. 2017 Mar;34(3):441-445. doi: 10.1111/echo.13475. Epub 2017 Feb 8.

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Zhao LJ, Han B, Zhang JJ, Yi YC, Jiang DD, Lyu JL.

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