Congenital EP Featured Articles of April 2017

 

Pediatric Cardiac and EP Reviews of April 2017 Manuscripts

 

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

Grubb AF, Shah G, Aziz PF, Krasuski RA

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

 

Take Home Points:

 

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

 

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

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

 

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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.

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

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

 

Reviewer perspective and thoughts for pediatric/CHD EP:

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

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

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

CHD EP April 2017

 

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

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

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

PMID: 28436549

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

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

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

PMID: 28431884

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

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

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

PMID: 28410419 Free PMC Article

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

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

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

PMID: 28402450

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

Wiles BM, Fitzsimmons SJ, Roberts PR.

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

PMID: 28374449

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

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

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

PMID: 28374048

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

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

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

PMID: 28441822

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

Wu JJ, Li F.

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

PMID: 28441820

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

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

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

PMID: 27766996

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

Miyazaki A, Doi H.

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

PMID: 28356308

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

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

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

PMID: 28431063

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  1. Outcomes of lead extraction in young adults.

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

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

PMID: 28189822

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

Lang CN, Steinfurt J, Odening KE.

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

PMID: 28233036

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

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

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

PMID: 28139301 Free Article

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

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

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

PMID: 28091832

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

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

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

PMID: 28155223

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

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

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

PMID: 27987225

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

Tran TV, Cortez D.

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

PMID: 28456831

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

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

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

PMID: 28453441

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

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

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

PMID: 28453898

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

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

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

PMID: 28450351

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

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

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

PMID: 28216547 Free Article

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

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

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

PMID: 28436586

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

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

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

PMID: 28440361 Free Article

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

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

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

PMID: 28429460

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

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

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

PMID: 28416468

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

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

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

PMID: 28412158

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

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

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

PMID: 28433559

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

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

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

PMID: 28394409

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

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

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

PMID: 28394443 Free Article

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

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

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

PMID: 28383202

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

Kean AC, Rodefeld M.

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

PMID: 28389305

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

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

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

PMID: 28404607

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

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

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

PMID: 28356306

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

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

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

PMID: 28431071

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

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

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

PMID: 28139301 Free Article

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  1. Precision Cardiovascular Medicine: State of Genetic Testing.

Giudicessi JR, Kullo IJ, Ackerman MJ.

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

PMID: 28385198

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  1. Early Repolarization in Normal Adolescents is Common.

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

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

PMID: 28367598

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

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

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

PMID: 28271152

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

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

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

PMID: 28184977

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

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

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

PMID: 28078383

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  1. ECGs in the ED.

Tanel RE.

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

PMID: 28353534

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

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

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

PMID: 28429690 Free Article

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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.

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28335848

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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.

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Select item 27981916 39.

 

  1. Traumatic Gerbode ventricular septal defect and third-degree heart block.

Campbell MJ, Lodge AJ, Miller SG.

Cardiol Young. 2017 Mar;27(2):404-406. doi: 10.1017/S1047951116001359. Epub 2016 Sep 19.

PMID:

 

27641901

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Select item 27640301 47.

 

  1. Lower spatial QRS-T angle rules out sustained ventricular arrhythmias in children with hypertrophic cardiomyopathy.

Cortez D, Sharma N, Cavanaugh J, Tuozo F, Derk G, Lundberg E, Weiner K, Kiciman N, Alejos J, Landeck B, Aboulhosn J, Miyamoto S, McCanta AC, Batra AS.

Cardiol Young. 2017 Mar;27(2):354-358. doi: 10.1017/S1047951116000640. Epub 2016 Jun 1.

PMID:

 

27246458

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Select item 27225605 51.

 

  1. Radiofrequency ablation of atrial tachyarrhythmias in adults with tetralogy of Fallot – predictors of success and outcome.

Ezzat VA, Ryan MJ, O’Leary J, Ariti C, Deanfield J, Pandya B, Cullen S, Walker F, Khan F, Abrams DJ, Lambiase PD, Lowe MD.

Cardiol Young. 2017 Mar;27(2):284-293. doi: 10.1017/S1047951116000482. Epub 2016 May 26.

PMID:

 

27225323

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Select item 27194079 54.

 

  1. Radiofrequency ablation versus cryoablation for atrioventricular nodal re-entrant tachycardia in children: a value comparison.

Oster ME, Yang Z, Stewart-Huey K, Glanville M, Porter A, Campbell R, Webb B, Strieper M.

Cardiol Young. 2017 Mar;27(2):224-228. doi: 10.1017/S1047951116000299. Epub 2016 Apr 18.

PMID:

 

27087499

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Select item 27086665 60

 

  1. Transseptal puncture for radiofrequency catheter ablations of left-sided arrhythmias in a paediatric population.

Ehrlinspiel DM, Gass M, Balmer C.

Cardiol Young. 2017 Mar;27(2):267-272. doi: 10.1017/S1047951116000457. Epub 2016 Apr 18.

PMID:

 

27086493

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Select item 27064196 62.

 

 

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Congenital EP Featured Articles of February 2017

Utility and Safety of Combined Interventional Catheterization and Electrophysiology Procedures in a Children’s Hospital.

Asaki SY, Orcutt JW, Miyake CY, Justino H, DE LA Uz CM, Kim JJ, Valdes SO, Qureshi AM.

Pacing Clin Electrophysiol. 2017 Feb 13. doi: 10.1111/pace.13047. [Epub ahead of print]

PMID: 28191656

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Select item 28190295

 

Take Home Points:

  • Patients with congenital heart disease often require both structural and electrophysiological interventions as part of their clinical management
  • Combining the two types of interventions into one procedure may be beneficial, but potentially challenging due to limitations in physician availability and the perception of increased procedural risk
  • Such combined procedures, as demonstrated in the present study, may be performed at high volume centers effectively and without elevated risk

 

J MooreCommentary from Dr. Jeremy Moore (Los Angeles), section editor of Pediatric Cardiac EP Journal Watch:  Patients with congenital heart disease are frequently exposed to both structural and electrophysiological procedures as a result of residual surgical lesions, collateral vessel formation, and either congenital or acquired rhythm abnormalities. These procedures are usually performed separately, which at least in theory, could have negative consequences in terms of repeated anesthetic exposures, exhaustion of patient and physician resources, and increases in health care costs (as compared to a single combined procedure). Investigators from Texas Children’s Hospital reported their experience with combining these two types of interventions into a single procedure over a study period from 2001 through 2014.

 

In total, the investigators identified 61 pediatric and adult patients that underwent a planned combination of structural and electrophysiologic intervention during this period. The most important subgroups included 1) ACHD (26%), 2) single ventricle (34%), and 3) heterotaxy patients (19%). Overall, structural interventions were successful in all but 2 cases and electrophysiological interventions were successful in all but 1 case. There were 3 adverse events (~5%), but none of these were felt to be secondary to the combined nature of the procedure and there were no prolonged hospital admissions as a result of this approach. Details of the procedures are shown in the table below.

EP media 1

 

Overall, the authors concluded that a combined approach could be attained in large volume centers without increased risk. There was a presumed benefit in terms of the single hospital encounter, lower overall anesthetic exposure, and potential reduction in health care costs. The major limitations of the study were the significant heterogeneity of the study population and the lack of a control arm. Future study to address these limitations was recommended.

 

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 Feb 9. pii: heartjnl-2016-310512. doi: 10.1136/heartjnl-2016-310512. [Epub ahead of print]

PMID: 28183791

Similar articles

Select item 28181926

 

Take Home Points:

  • Patients with D-TGA after the atrial switch operation are at elevated risk for heart failure and cardiovascular mortality
  • Robust risk stratification models in this population are limited
  • The appearance of a fractionated QRS complex is independently associated with the composite end-point of cardiovascular mortality, heart transplantation, or the need for ventricular assist device support for D-TGA patients after the atrial switch operation

 

Commentary from Dr. Jeremy Moore (Los Angeles), section editor of Pediatric Cardiac EP Journal Watch: The Mustard/Senning operations for physiologic repair of D-TGA was the primary surgical approach spanning a 20-year period from the early 1960’s through the mid 1980’s. Such patients are known to be at increased risk for heart failure and sudden cardiac death. Since existing risk stratification models are limited, these investigators from Belgium sought to evaluate the contribution of a novel non-invasive marker for the composite end-point of cardiovascular mortality, heart transplantation, or the need for ventricular assist device support.

 

From a cohort of 133 patients, all 12 lead ECGs were assessed for new-onset QRS fragmentation, which was defined as a previously normal ECG followed by an ECG with a fragmented QRS complex in the same patient, occurring in at least 2 contiguous right precordial, inferior, or left lateral leads (see examples below).

 

EP media 2

During the follow up period of nearly 17 years, approximately one-third of patients developed a fragmented QRS complex, most commonly in the inferior leads (suggesting myocardial involvement in the territory of the right coronary). Overall, 10% of patients experienced the composite outcome of cardiovascular mortality, heart transplantation, or the need for ventricular assist device support. In the multivariate analysis, both fractionated QRS and severe RV dysfunction independently predicted this composite outcome (HR 18.4, p=0.022 and HR 16.7, p=0.007; respectively).

 

The authors concluded that this electrocardiographic finding can be easily implemented and could be useful for clinical decision-making for the D-TGA population after the atrial switch operation.

 

CHD EP Feb 2017 

  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 Feb 28. doi: 10.1111/jce.13197. [Epub ahead of print]

PMID: 28245348

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Select item 28240408

 

  1. Tilt table testing to diagnose pseudosyncope in the pediatric population.

Robinson JA, Shivapour JK, Snyder CS.

Congenit Heart Dis. 2017 Feb 27. doi: 10.1111/chd.12458. [Epub ahead of print]

PMID: 28240408

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Select item 28239760

 

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

Lang CN, Steinfurt J, Odening KE.

Herz. 2017 Feb 23. doi: 10.1007/s00059-017-4549-2. [Epub ahead of print]

PMID: 28233036

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Select item 28231853

 

  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 KS, Rinne S, Matschke LA, Friedrich C, Schulze-Bahr E, Rust S, Seebohm G, Decher N, Schulze-Bahr E.

Circ Res. 2017 Feb 20. pii: CIRCRESAHA.116.310112. doi: 10.1161/CIRCRESAHA.116.310112. [Epub ahead of print]

PMID: 28219978

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Select item 28217850

 

  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 Feb 14. pii: S1547-5271(17)30080-2. doi: 10.1016/j.hrthm.2017.01.030. [Epub ahead of print]

PMID: 28189822

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Select item 28195132

 

  1. Utility and Safety of Combined Interventional Catheterization and Electrophysiology Procedures in a Children’s Hospital.

Asaki SY, Orcutt JW, Miyake CY, Justino H, DE LA Uz CM, Kim JJ, Valdes SO, Qureshi AM.

Pacing Clin Electrophysiol. 2017 Feb 13. doi: 10.1111/pace.13047. [Epub ahead of print]

PMID: 28191656

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Select item 28190295

 

  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 Feb 9. pii: heartjnl-2016-310512. doi: 10.1136/heartjnl-2016-310512. [Epub ahead of print]

PMID: 28183791

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Select item 28181926

 

  1. Autonomic Nervous System Function After a Skin-to-Skin Contact Intervention in Infants With Congenital Heart Disease.

Harrison TM, Brown R.

J Cardiovasc Nurs. 2017 Feb 9. doi: 10.1097/JCN.0000000000000397. [Epub ahead of print]

PMID: 28181926

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Select item 28182287

 

  1. Application of pediatric appropriate use criteria for initial outpatient evaluation of syncope.

Phelps HM, Kelleman MS, McCracken CE, Benavidez OJ, Campbell RM, Douglas PS, Eidem BW, Lai WW, Lopez L, Stern KW, Welch E, Sachdeva R.

Echocardiography. 2017 Feb 8. doi: 10.1111/echo.13475. [Epub ahead of print]

PMID: 28177138

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Select item 28228731

 

  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 Feb 8. doi: 10.1111/petr.12894. [Epub ahead of print]

PMID: 28181355

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Select item 28189544

 

  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 Feb 5. pii: S0167-5273(17)30680-0. doi: 10.1016/j.ijcard.2017.02.004. [Epub ahead of print]

PMID: 28209388

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Select item 28219755

 

  1. A congenital diverticulum of the left ventricular apex manifested by stroke and recurrent ventricular tachycardia.

Dostálová G, Paleček T, Kuchynka P, Havránek Š, Mašek M, Hlubocká Z, Karetová D, Wichterle D, Dušková J, Lindner J, Linhart A.

Cardiovasc Pathol. 2017 Feb 4;28:3-6. doi: 10.1016/j.carpath.2017.01.009. [Epub ahead of print]

PMID: 28219755

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Select item 28238509

 

  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 Feb 3. doi: 10.1111/pace.13040. [Epub ahead of print]

PMID: 28155223

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Select item 28153876

 

  1. Hemodynamic effects of sustained postoperative cardiac resynchronization therapy in infants after repair of congenital heart disease: Results of a randomized clinical trial.

Friedberg MK, Schwartz SM, Zhang H, Chiu-Man C, Manlhiot C, Ilina MV, Arsdell GV, Kirsh JA, McCrindle BW, Stephenson EA.

Heart Rhythm. 2017 Feb;14(2):240-247. doi: 10.1016/j.hrthm.2016.09.025.

PMID: 27687644

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Select item 27756709

 

  1. Interventricular dyssynchrony in a patient with a biventricular physiology and a systemic right ventricle.

Miyazaki A, Sakaguchi H, Noritake K, Hayama Y, Negishi J, Kagisaki K, Yasuda K, Ichikawa H, Ohuchi H.

Heart Vessels. 2017 Feb;32(2):234-239. doi: 10.1007/s00380-016-0876-0.

PMID: 27385023

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Select item 27912205

 

  1. Biventricular Paced QRS Area Predicts Acute Hemodynamic CRT Response Better Than QRS Duration or QRS Amplitudes.

DE Pooter J, El Haddad M, DE Buyzere M, Aranda HA, Cornelussen R, Stegemann B, Rinaldi CA, Sterlinski M, Sokal A, Francis DP, Jordaens L, Stroobandt RX, VAN Heuverswyn F, Timmermans F.

J Cardiovasc Electrophysiol. 2017 Feb;28(2):192-200. doi: 10.1111/jce.13132.

PMID: 27885752

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Select item 27592026

 

  1. Acute Management of Refractory and Unstable Pediatric Supraventricular Tachycardia.

Lewis J, Arora G, Tudorascu DL, Hickey RW, Saladino RA, Manole MD.

J Pediatr. 2017 Feb;181:177-182.e2. doi: 10.1016/j.jpeds.2016.10.051.

PMID: 27912926

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Select item 27837950

 

  1. Implantation of VVI pacemaker in a patient with dextrocardia, persistent left superior vena cava, and sick sinus syndrome: A case report.

Guo G, Yang L, Wu J, Sun L.

Medicine (Baltimore). 2017 Feb;96(5):e6028. doi: 10.1097/MD.0000000000006028.

PMID: 28151908 Free PMC Article

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Select item 28151866

 

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

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

Pacing Clin Electrophysiol. 2017 Feb;40(2):145-153. doi: 10.1111/pace.13017.

PMID: 28070890

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Select item 27935164

 

  1. Selective use of the electrocardiogram in pediatric preparticipation athletic examinations among pediatric primary care providers.

Clark BC, Hayman JM, Berul CI, Burns KM, Kaltman JR.

Ann Noninvasive Electrocardiol. 2017 Feb 21. doi: 10.1111/anec.12446. [Epub ahead of print]

PMID: 28220574

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Select item 28235645

 

  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 KS, Rinne S, Matschke LA, Friedrich C, Schulze-Bahr E, Rust S, Seebohm G, Decher N, Schulze-Bahr E.

Circ Res. 2017 Feb 20. pii: CIRCRESAHA.116.310112. doi: 10.1161/CIRCRESAHA.116.310112. [Epub ahead of print]

PMID: 28219978

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Select item 28239450

 

  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 Feb 20:1-4. doi: 10.1017/S1049023X17000048. [Epub ahead of print]

PMID: 28215188

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Select item 28217909

 

  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 Feb 19. doi: 10.1111/anec.12435. [Epub ahead of print]

PMID: 28217909

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Select item 28223051

 

  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 Feb 18. doi: 10.1253/circj.CJ-16-0991. [Epub ahead of print]

PMID: 28216547 Free Article

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Select item 28213945

 

  1. Radiofrequency catheter ablation of left-sided accessory pathways in children using a new fluoroscopy integrated 3D-mapping system.

Drago F, Grifoni G, Remoli R, Russo MS, Righi D, Pazzano V, Palmieri R, Placidi S, Saputo FA, Silvetti MS.

Europace. 2017 Feb 15. doi: 10.1093/europace/euw220. [Epub ahead of print] No abstract available.

PMID: 28201731

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Select item 28198527

 

  1. Commentary: Clinical Phenotype of HCN4-related Sick Sinus Syndrome.

Raucci FJ Jr, Shoemaker MB, Knollmann BC.

Heart Rhythm. 2017 Feb 14. pii: S1547-5271(17)30164-9. doi: 10.1016/j.hrthm.2017.02.006. [Epub ahead of print] No abstract available.

PMID: 28213054

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Select item 28197645

 

  1. Pilot Study Analyzing Automated ECG Screening of Hypertrophic Cardiomyopathy.

Campbell M, Zhou X, Han C, Abrishami H, Webster G, Miyake CY, Sower CT, Anderson JB, Knilans TK, Czosek RJ.

Heart Rhythm. 2017 Feb 10. pii: S1547-5271(17)30169-8. doi: 10.1016/j.hrthm.2017.02.011. [Epub ahead of print]

PMID: 28193509

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Select item 28186551

 

  1. Prenatal exposure to snus alters heart rate variability in the infant.

Nordenstam F, Lundell B, Cohen G, Tessma MK, Raaschou P, Wickström R.

Nicotine Tob Res. 2017 Feb 10. doi: 10.1093/ntr/ntx035. [Epub ahead of print] No abstract available.

PMID: 28186551

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Select item 28183324

 

  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 Feb 10. doi: 10.1007/s00246-017-1571-3. [Epub ahead of print]

PMID: 28184977

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Select item 28184976

 

  1. Incidence of Idiopathic Ventricular Arrhythmias: A Population-Based Study.

Sirichand S, Killu AM, Padmanabhan D, Hodge DO, Chamberlain AM, Brady PA, Kapa S, Noseworthy PA, Packer DL, Munger TM, Gersh BJ, McLeod CJ, Shen WK, Cha YM, Asirvatham SJ, Friedman PA, Mulpuru SK.

Circ Arrhythm Electrophysiol. 2017 Feb;10(2). pii: e004662. doi: 10.1161/CIRCEP.116.004662.

PMID: 28183845

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Select item 28193613

 

  1. Prominent QTc prolongation in a patient with a rare variant in the cardiac ryanodine receptor gene.

Taniguchi Y, Miyazaki A, Sakaguchi H, Hayama Y, Ebishima N, Negishi J, Noritake K, Miyamoto Y, Shimizu W, Aiba T, Ohuchi H.

Heart Vessels. 2017 Feb;32(2):229-233. doi: 10.1007/s00380-016-0869-z.

PMID: 27401738

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Select item 27385023

 

  1. Prevalence and Risk Factors of Prolonged Corrected QT Interval Among Children and Adolescents Treated With Antipsychotic Medications: A Long-Term Follow-Up in a Real-World Population.

Palanca-Maresca I, Ruiz-Antorán B, Centeno-Soto GA, Forti-Buratti MA, Siles A, Usano A, Avendaño-Solá C.

J Clin Psychopharmacol. 2017 Feb;37(1):78-83. doi: 10.1097/JCP.0000000000000639.

PMID: 27930499

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Select item 27989540

 

  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 Feb 1. pii: S0022-0736(17)30018-3. doi: 10.1016/j.jelectrocard.2017.01.015. [Epub ahead of print]

PMID: 28189280

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Select item 27592026

 

  1. Characterizing cardiac arrest in children undergoing cardiac surgery: A single-center study.

Gupta P, Wilcox A, Noel TR, Gossett JM, Rockett SR, Eble BK, Rettiganti M.

J Thorac Cardiovasc Surg. 2017 Feb;153(2):450-458.e1. doi: 10.1016/j.jtcvs.2016.10.013.

PMID: 27866783

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Select item 27992621

 

  1. Key Role of Pacing Site as Determinant Factor of Exercise Testing Performance in Pediatric Patients with Chronic Ventricular Pacing.

Cabrera Ortega M, Duamy HT, Benítez Ramos DB.

Pediatr Cardiol. 2017 Feb;38(2):368-374. doi: 10.1007/s00246-016-1523-3.

PMID: 27913834

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Select item 27900454

 

  1. Predictors of Pharmacological Therapy of Ectopic Atrial Tachycardia in Children.

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

Pediatr Cardiol. 2017 Feb;38(2):289-295. doi: 10.1007/s00246-016-1511-7.

PMID: 27882422

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Select item 27878633

 

  1. Rate Control by Transoesophageal Atrial Overdrive Pacing for Refractory Supraventricular Tachycardia with Severe Ventricular Dysfunction: A Bridge to Recovery.

Paech C, Janousek J, Wagner F, Gebauer RA.

Pediatr Cardiol. 2017 Feb;38(2):228-233. doi: 10.1007/s00246-016-1503-7.

PMID: 27837305

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Select item 27837302


 

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Congenital EP Featured Articles of January 2017

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

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

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

PMID: 27780555

Similar articles

Select item 27780553

Take Home Points

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

 

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

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

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

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

ep 1

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

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

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

ep 2

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

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

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

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

 

Reviewer perspective and thoughts for pediatric/CHD EP:

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

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

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

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

 

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

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

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

PMID:  27862589

Similar articles

 

Take Home Points

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

 

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

Article summary:

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

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

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

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

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

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

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

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

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

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

Reviewer perspective and thoughts for pediatric/CHD EP:

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

CHD EP Jan 2017

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

10. Molecular Pathophysiology of Congenital Long QT Syndrome.

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

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

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

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

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

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

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

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

 

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

Erickson CC, Salerno JC.

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

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

Fischbach P.

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

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

Benson DW, Cohen MI.

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

25. Sports participation in long QT syndrome.

Aziz PF, Saarel EV.

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

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

Tester DJ, Ackerman MJ.

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

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

Erickson CC.

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

28. Arrhythmogenic right ventricular dysplasia/cardiomyopathy.

Orgeron GM, Crosson JE.

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

Yamada T, Lau YR, Kay GN.

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

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

Gulgun M.

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

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

Gulgun M.

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

42. ECGs in the ED.

Tanel RE.

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

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

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

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

44. Para-Hisian Pacing: A Paradoxical Response?

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

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

 

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Congenital EP Featured Articles of December 2016

Follow-Up of a Prospective Surgical Strategy to Prevent Intra-Atrial Reentrant Tachycardia after the Fontan Operation.

Law IH, Alam O, Bove EL, Ohye RG, Bradley DJ, Yu S, Dick M 2nd.

Circ Arrhythm Electrophysiol. 2016 Dec;9(12). pii: e004478.

Take Home Points

  • The incidence of intra-atrial reentrant tachycardia (IART) after Fontan palliation has been reported to be between 10% and 40%.
  • The incidence of IART in this cohort who underwent lateral tunnel Fontan palliation was quite low (3.5%) at ~8 year follow-up.
  • There was no difference in the incidence of IART after Fontan palliation with the addition of minimal arrhythmia surgery to create a line of isthmus block between the lateral tunnel incision and tricuspid valve annulus.
  • There were no adverse effects from additional arrhythmia surgery including no changes to sinus node function, AV node function or inducibility of IART.

A PatelCommentary from Dr. Akash Patel (San Francisco), section editor of Pediatric Cardiac EP Journal Watch: The incidence of intra-atrial reentrant tachycardia after Fontan palliation has improved over the years but the rates of occurrence have been reported between 10 and 40% of patients.  The use of the lateral tunnel Fontan palliation demonstrated lower arrhythmia rates between the 10 to 20%, however this continues to be a significant problem as survival has improved and this population ages.  This study attempted to use a novel modified surgical approach to reduce the risk of IART in Fontan palliated patients.

The study was a prospective single center randomized double blind study to evaluate the impact of an incision in the right atrium joining the anterior lateral tunnel suture line and the tricuspid valve annulus with additional cryosurgical lesions placed at the tricuspid end of the incision to create a line of isthmus block.  See Figure 1 below.

ep 1

Between 1998 and 2003 at the University of Michigan all patients with univentricular anatomy who were scheduled for a fenestrated lateral tunnel Fontan were approached for the study with a total of 134 patients enrolled.  67 patients underwent a standard fenestrated lateral tunnel operation and 67 underwent the same operation plus an additional surgical incision and cryosurgical lesions as mentioned above.

The baseline groups showed no differences based on baseline characteristics, pre-Fontan catheterization data, pre-Fontan electrophysiology data, intraoperative characteristics hospital course, and follow-up (see Table 1 and 2 below).  The intra-operative cross-clamp time was 6 minutes longer in the incision group.

ep 2

ep 3

Intra-atrial reentrant tachycardia was induced in 22% of the total cohort prior to Fontan surgery with no difference between groups.  After surgery, transthoracic epicardial wires were used to perform an EP study with inducible IART seen in 16.4% with no difference between groups.  In addition, 114 had long-term follow-up with a median follow-up time of 8.2 years with only 3.5% demonstrating IART with again no differences between the groups. (See Table 3 below).

ep 4

There was no difference in sinus node and AV nodal conduction properties as a result of the surgical approaches. There was no difference in inducibility of IART pre –and post- Fontan alleviating concerns of pro-arrhythmia due to the additional surgical procedure.  Finally, there were only 2 patients (2%) of patients with long-term follow-up who received pacemaker therapy for sick sinus syndrome.

This novel approach to reduce the incidence of IART in this cohort did not show any short-term and intermediate-term impact in the first decade after surgery but the median age at follow-up remains young teenagers. Thus, the long-term implications of this approach are unknown and may provide future benefit as this population ages into the 2nd or 3rd decades of life.  It is clear there appears to be no short-term adverse effects of this surgical approach.  Finally, most notable is the low incidence (3.5%) of IART compared to other studies despite the intervention used in this study. This may reflect on improvements in surgical approach and management.

 

 

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. 2016 Dec 16. doi: 10.1111/pace.12983. [Epub ahead of print]

Take Home Points:

  • 25% of adult admissions with congenital heart disease are associated with arrhythmia diagnoses
  • As expected, arrhythmia prevalence and types are associated with specific congenital heart defects
  • Atrial fibrillation comprised the majority of arrhythmia diagnosed at 86%.
  • Comorbid conditions – Smoking, Diabetes, Hypertension, Obstructive Sleep Apnea, Heart Failure, Myocardial Infarction, and Kidney Disease – are associated with increased likelihood of arrhythmias in the ACHD population.
  • Arrhythmias diagnoses during inpatient hospitalization for ACHD are associated with increased electrophysiology procedures, length of stay, hospitalization cost, and inpatient mortality

Commentary from Dr. Akash Patel (San Francisco), section editor of Pediatric Cardiac EP Journal Watch:  The prevalence of arrhythmias in adult congenital heart disease patients is a known and growing issue as the ACHD population ages. The risk of arrhythmia development has been well studied in a variety of specific congenital heart defects such as tetralogy of Fallot. However, there is limited population-based data on the prevalence and associated risk factors for arrhythmias in the inpatient ACHD population.

This study used a population-based approach by utilizing administrative data via the Nationwide Inpatient Sample. While limited in important ways, this is the largest publicly available all-payer hospital inpatient care database in the United States that captures data from ~20% of all community hospital admissions in the United States.   The study population included inpatient hospitalizations from 2005 to 2012 of patients over the age of 18 years who had admissions with ICD codes for arrhythmias and specific congenital heart defects. The aim of this study was to determine the prevalence of arrhythmias in the ACHD inpatient population with specific cardiac lesions, determine associated risk factors, and impact on outcomes including mortality and health care utilization.

This study included 109,168 patients with specific preselected congenital heart diseases of which 27,088 (24.8%) had an arrhythmia.  The median age of the patients without arrhythmias was 55 years and with arrhythmias 67 years.   Males were associated with increased risk of arrhythmias (50% vs. 45%).   The most common congenital defect was coronary anomaly in this cohort which compromised 78% followed by tetralogy of Fallot (9%), total anomalous pulmonary venous connection (8%), other included specific congenital lesions (5%). The table below shows the prevalence of arrhythmia based on ACHD lesion and significantly associated arrhythmias.

ep 5

The congenital defects with the most prevalent arrhythmias diagnosed included tricuspid atresia (51%), Ebstein’s anomaly (39%), congenitally corrected transposition (38%) and transposition of the great arteries (36%).  In addition, as expected the types of arrhythmias variety based on congenital defect.

The total arrhythmia burden as mentioned was 25%. The table below shows the prevalence by arrhythmia type with atrial arrhythmia comprising the majority – atrial fibrillation (85.7%) and atrial flutter (19.5%).

Type of Arrhythmia Prevalence
Atrial Fibrillation 23205 (85.7%)
Atrial Flutter 5273 (19.5%)
Paroxysmal VT 1458 (5.4%)
Sinus Node Dysfunction 1273 (4.7%)
1st Degree AV Block 1186 (4.4%)
Ventricular Fibrillation 576 (2.1%)
3rd Degree AV Block 548 (2.0%)
Paroxysmal SVT 453 (1.7%)
2nd Degree AV Block Type I 399 (1.5%)
2nd Degree AV Block Type II 92 (0.3%)
Ventricular Flutter 22 (0.1%)

In addition to the type of congenital heart defects, common adult comorbidities were associated with increased arrhythmia risk. This included smoking, diabetes, hypertension, obstructive sleep apnea, heart failure, myocardial infarction, and kidney disease.   Heart failure provided the greatest risk with an odds ratio of 3.5.  Interestingly, hyperlipidemia was associated with a decreased risk with an odds ratio of 0.93.

Finally, arrhythmias diagnoses during inpatient hospitalization for ACHD were significantly associated with increased health care utilization and mortality. There was a significant increase in electrophysiology procedures – electrophysiology study (5.8% vs. 0.8%), catheter ablation (6.9% vs. 0.5%), pacemaker insertion (5.3% vs. 1%), and AICD insertion (1.6% vs. 0.5%).  There was an increased in the length of stay (6.9 days vs. 5.8 days). There was increased cost ($110,205 vs. $86,974).  Finally, there was increased mortality (3% vs. 2.1%).

Clearly, the use of administrative data to understand arrhythmias in the ACHD population can only provide a broad overview and is limited due to the lack of adjudication of cardiac and arrhythmia diagnoses. In addition, the NIS database is a sample of the hospitals and may represent a skewed population that may or may not include ACHD centers. Finally, the specifics of congenital heart defects, residual hemodynamic burden, and surgical approaches are unknown which are identified risk factors in arrhythmia development. Nonetheless, this study provides some insights into the extent of arrhythmia burden in inpatient ACHD hospitalizations, associated risk factors including lesion type, and the negative impact on health outcomes including increased utilization of care and mortality.

 


CHD EP Articles – December 2016

 

  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. 2016 Dec 16. doi: 10.1111/pace.12983. [Epub ahead of print]

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

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

Nat Biotechnol. 2016 Dec 12. doi: 10.1038/nbt.3745. [Epub ahead of print]

  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. 2016 Dec 2. pii: 0961203316682099. [Epub ahead of print]

  1. 4. Follow-Up of a Prospective Surgical Strategy to Prevent Intra-Atrial Reentrant Tachycardia After the Fontan Operation.

Law IH, Alam O, Bove EL, Ohye RG, Bradley DJ, Yu S, Dick M 2nd.

Circ Arrhythm Electrophysiol. 2016 Dec;9(12). pii: e004478.

  1. Molecular and Functional Characterization of Rare CACNA1C Variants in Sudden Unexplained Death in the Young.

Sutphin BS, Boczek NJ, Barajas-Martínez H, Hu D, Ye D, Tester DJ, Antzelevitch C, Ackerman MJ.

Congenit Heart Dis. 2016 Dec;11(6):683-692. doi: 10.1111/chd.12371.

  1. Improved Ventricular Function after TEE-guided Cardioversion of Atrial Arrhythmias in Patients after the Fontan Operation.

Poterucha JT, Egbe AC, Johnson JN, Niaz T, Wackel PL, Cannon BC, Eidem BW, Cetta F.

Congenit Heart Dis. 2016 Dec;11(6):578-583. doi: 10.1111/chd.12339.

  1. Patient-specific modeling of left ventricular electromechanics as a driver for haemodynamic analysis.

Augustin CM, Crozier A, Neic A, Prassl AJ, Karabelas E, Ferreira da Silva T, Fernandes JF, Campos F, Kuehne T, Plank G.

Europace. 2016 Dec;18(suppl 4):iv121-iv129. doi: 10.1093/europace/euw369.

  1. Ischemia-reperfusion destabilizes rhythmicity in immature atrioventricular pacemakers: A predisposing factor for postoperative arrhythmias in neonate rabbits.

Chenliu C, Sheng X, Dan P, Qu Y, Claydon VE, Lin E, Hove-Madsen L, Sanatani S, Tibbits GF.

Heart Rhythm. 2016 Dec;13(12):2348-2355. doi: 10.1016/j.hrthm.2016.07.022.

  1. Percutaneous Pulmonary Valve Implantation-Associated Ventricular Tachycardia in Congenital Heart Disease.

Loar RW, Qureshi AM, Miyake CY, Valdes SO, Kim JJ, De la Uz CM.

J Interv Cardiol. 2016 Dec;29(6):639-645. doi: 10.1111/joic.12344.

  1. Variability of Myocardial Repolarization in Pediatric Patients with a Ventricular Septal Defect.

Uchida H, Nishio M, Omeki Y, Takeuchi Y, Nagata R, Oikawa S, Nagatani A, Eryu Y, Hata T, Yoshikawa T.

Pediatr Cardiol. 2016 Dec;37(8):1458-1464.

  1. Cytokine Elevation in Sudden Death With Respiratory Syncytial Virus: A Case Report of 2 Children.

Kakimoto Y, Seto Y, Ochiai E, Satoh F, Osawa M.

Pediatrics. 2016 Dec;138(6). pii: e20161293.

  1. SUPRAVENTRICULAR TACHYCARDIA DURING THE FIRST YEAR OF LIFE: IS SUBCLINICAL INFLAMMATION THE TRIGGER?

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

J Matern Fetal Neonatal Med. 2016 Dec 23:1-17. doi: 10.1080/14767058.2016.1275545. [Epub ahead of print]

  1. Epidemiology and outcomes of cardiac arrest among children with Down syndrome: a multicenter analysis.

Padiyath A, Rettiganti M, Gossett JM, Tadphale SD, Garcia X, Seib PM, Gupta P.

Minerva Anestesiol. 2016 Dec 22. [Epub ahead of print]

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

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

Pacing Clin Electrophysiol. 2016 Dec 22. doi: 10.1111/pace.12995. [Epub ahead of print]

  1. 1 Duration of Prehospital Cardiopulmonary Resuscitation and Favorable Neurological Outcomes for Pediatric Out-of-Hospital Cardiac Arrests: A Nationwide, Population-Based Cohort Study.

Goto Y, Funada A, Goto Y.

Circulation. 2016 Dec 20;134(25):2046-2059.

  1. Nadolol for Treatment of Supraventricular Tachycardia in Infants and Young Children.

von Alvensleben JC, LaPage MJ, Caruthers R, Bradley DJ.

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

  1. Fluorinated steroids do not improve outcome of isolated atrioventricular block.

Van den Berg NW, Slieker MG, van Beynum IM, Bilardo CM, de Bruijn D, Clur SA, Cornette JM, Frohn-Mulder IM, Haak MC, van Loo-Maurus KE, Manten GT, Rackowitz AB, Rammeloo LA, Reimer A, Rijlaarsdam ME, Freund MW.

Int J Cardiol. 2016 Dec 15;225:167-171. doi: 10.1016/j.ijcard.2016.09.119.

  1. Prenatal diagnosis and prognosis of accelerate idioventricular rhythm.

Fouron JC, McNeal-Davidson A, Abadir S, Fournier A, Bigras JL, Boutin C, Brassard M, Raboisson MJ, van Doesburg N, Berger A, Brisebois S, Gendron R.

Ultrasound Obstet Gynecol. 2016 Dec 10. doi: 10.1002/uog.17382. [Epub ahead of print]

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

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

Forensic Sci Int. 2016 Dec 9;270:173-177. doi: 10.1016/j.forsciint.2016.12.001. [Epub ahead of print]

  1. Key Role of Pacing Site as Determinant Factor of Exercise Testing Performance in Pediatric Patients with Chronic Ventricular Pacing.

Cabrera Ortega M, Duamy HT, Benítez Ramos DB.

Pediatr Cardiol. 2016 Dec 2. [Epub ahead of print]

  1. The Canadian Arrhythmogenic Right Ventricular Cardiomyopathy Registry: Rationale, Design, and Preliminary Recruitment.

Krahn AD, Healey JS, Gerull B, Angaran P, Chakrabarti S, Sanatani S, Arbour L, Laksman ZW, Carroll SL, Seifer C, Green M, Roberts JD, Talajic M, Hamilton R, Gardner M.

Can J Cardiol. 2016 Dec;32(12):1396-1401. doi: 10.1016/j.cjca.2016.04.004.

  1. Letter by Amin et al Regarding Article, “Genetic Modifiers for the Long-QT Syndrome: How Important Is the Role of Variants in the 3′ Untranslated Region of KCNQ1?”

Amin AS, Pinto YM, Ackerman MJ, Wilde AA.

Circ Cardiovasc Genet. 2016 Dec;9(6):580. doi: 10.1161/CIRCGENETICS.116.001629. No abstract available.

  1. Environmental risk factors for sudden infant death syndrome in Japan.

Hirabayashi M, Yoshinaga M, Nomura Y, Ushinohama H, Sato S, Tauchi N, Horigome H, Takahashi H, Sumitomo N, Shiraishi H, Nagashima M.

Eur J Pediatr. 2016 Dec;175(12):1921-1926.

  1. Effectiveness of Radiofrequency Catheter Ablation of Outflow Tract Ventricular Arrhythmias in Children and Adolescents.

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

Pediatr Cardiol. 2016 Dec;37(8):1475-1481.

  1. Radiofrequency Ablation in the Sinus of Valsalva for Ventricular Arrhythmia in Pediatric Patients.

Wackel PL, McCrary AW, Idriss SF, Asirvatham SJ, Cannon BC.

Pediatr Cardiol. 2016 Dec;37(8):1534-1538.

  1. Arrhythmias After Stage I Hybrid Palliation in Single-Ventricle Patients.

Moore J, Paulus D, Cua CL, Kertesz NJ, Miao Y, Cheatham JP, Galantowicz M, Fernandez R.

Pediatr Cardiol. 2016 Dec;37(8):1416-1421.

  1. Installation of multiple automated external defibrillators to prevent sudden death in school-aged children.

Higaki T, Chisaka T, Moritani T, Ohta M, Takata H, Yamauchi T, Yamaguchi Y, Konishi K, Yamamoto E, Ochi F, Eguchi M, Eguchi-Ishimae M, Mitani Y, Ishii E.

Pediatr Int. 2016 Dec;58(12):1261-1265. doi: 10.1111/ped.13143.

  1. Paroxysmal atrioventricular block after heart transplantation in children: an early sign of rejection?

Grutter G, Alfieri S, Calcagni G, Castelluzzo MA, Silvetti MS, Parisi F, Drago F.

Pediatr Transplant. 2016 Dec;20(8):1164-1167. doi: 10.1111/petr.12832.

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