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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  1. Familial Catecholamine-Induced QT Prolongation in Unexplained Sudden Cardiac Death.

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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  1. Ablation of supraventricular arrhythmias in adultcongenital heart disease: A contemporary review.

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.

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28359691

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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.

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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.

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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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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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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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  1. Ablation of Ventricular Tachycardia inCongenitaland Infiltrative Heart Disease.

Wijnmaalen AP, Zeppenfeld K.

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