Congenital EP Featured Articles of July 2017

Congenital Pediatric Cardiac EP Reviews of July/August 2017 Manuscripts

 

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

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

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

 

Take Home Points:

 

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

 

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

 

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

 

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

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

 

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

 

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

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

J Cardiovasc Electrophysiol 2017; 28:677-683.

 

Take Home Points:

 

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

 

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

 

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

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

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

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

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

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

 

 

CHD EP July 2017

 

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

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

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

PMID: 28730319

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

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

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

PMID: 28532774

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

Ayubi E, Safiri S, Mansournia MA.

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

PMID: 28560967

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

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

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

PMID: 28716702

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

Barbanti C, Maltret A, Sidi D.

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

PMID: 28685702

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

Bronzetti G, Brighenti M, Bonvicini M.

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

PMID: 28685697

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

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

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

PMID: 28684436 Free Article

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

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

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

PMID: 28696220

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

Sherwin ED, Berul CI.

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

PMID: 28687672

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

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

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

PMID: 28687669

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

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

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

PMID: 28183791

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

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

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

PMID: 28323170

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

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

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

PMID: 27913750

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

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

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

PMID: 28436549

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

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

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

PMID: 28185283

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

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

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

PMID: 28755092

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

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

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

PMID: 28749010

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

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

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

PMID: 28741726

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  1. Contemporary Outcomes in Patients With Long QT Syndrome.

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

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

PMID: 28728690

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

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

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

PMID: 28744930

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

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

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

PMID: 28741754

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

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

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

PMID: 28740174 Free PMC Article

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

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

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

PMID: 28720014

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

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

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

PMID: 28711964

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

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

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

PMID: 28716699

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

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

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

PMID: 28699125

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

Bronzetti G, Brighenti M, Bonvicini M.

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

PMID: 28685697

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Select item 28687889

 

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

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

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

PMID: 28677509

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Select item 28681101

 

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

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

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

PMID: 28728260

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

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

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

PMID: 28217909

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

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

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

PMID: 27778147

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Select item 27509882

 

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

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

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

PMID: 28482693

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

Kean AC, Rodefeld MD.

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

PMID: 28389305

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

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

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

PMID: 28347833

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

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

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

PMID: 28492868

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

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

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

PMID: 28436586

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Congenital EP Featured Articles of 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.

EP5

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

EP6

EP7

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

 

Reviewer perspective and thoughts for pediatric/CHD EP:

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

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

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

CHD EP April 2017

 

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

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

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

PMID: 28436549

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

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

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

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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.

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

Wiles BM, Fitzsimmons SJ, Roberts PR.

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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.

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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.

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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.

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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.

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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.

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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]

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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]

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

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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.

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

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

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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.

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

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

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

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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.

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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.

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

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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.

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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.

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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.

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PMID: 28139301 Free Article

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  1. Precision Cardiovascular Medicine: State of Genetic Testing.

Giudicessi JR, Kullo IJ, Ackerman MJ.

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PMID: 28385198

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  1. Early Repolarization in Normal Adolescents is Common.

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

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

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

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

PMID: 28271152

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

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

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

PMID: 28184977

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

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

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

PMID: 28078383

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  1. ECGs in the ED.

Tanel RE.

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

PMID: 28353534

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

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

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

PMID: 28429690 Free Article

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CHD Electrophysiology Articles of February 2016

1. PACES/HRS Expert Consensus Statement on the use of Catheter Ablation in Children and Patients with Congenital Heart Disease: Developed in partnership with the Pediatric and Congenital Electrophysiology Society (PACES) and the Heart Rhythm Society (HRS). Endorsed by the governing bodies of PACES, HRS, the American Academy of Pediatrics (AAP), and the American Heart Association (AHA).

Philip Saul J, Kanter RJ, Abrams D, Asirvatham S, Bar-Cohen Y, Blaufox AD, Cannon B, Clark J, Dick M, Freter A, Kertesz NJ, Kirsh JA, Kugler J, LaPage M, McGowan FX, Miyake CY, Nathan A, Papagiannis J, Paul T, Pflaumer A, Skanes AC, Stevenson WG, Von Bergen N, Zimmerman F; Task Force Co-Chairs. Heart Rhythm. 2016 Feb 17. PMID: 26899545

 

A PatelComment from Dr. Akash Patel (San Francisco, CA), section editor of Congenital Heart Electrophysiology Journal Watch:  This 2016 consensus statement provides the most up-to-date guidelines for the use of catheter ablation in pediatric patients (0-18 years of age) and all congenital heart disease patients.  The last consensus statement was made in 2002 and since then there have been many advances in technical innovations to improve the safety and efficacy of catheter ablation.  In addition, ablation therapy has become an important option in arrhythmia management based on our improved understanding of arrhythmia mechanisms in these populations.

Overall, this consensus statement provides a nice review of the evidence used to make recommendations for catheter-based ablation in this population.

Take Home Points:

  1. Catheter ablation for children and patients with congenital heart disease can be very effective based on arrhythmia type and location.
  2. Catheter ablation for children and patients with congenital heart disease has a low risk profile that has improved over time.

 

 

2. Implantable cardioverter-defibrillators in adults with congenital heart disease: a systematic review and meta-analysis.

Vehmeijer JT, Brouwer TF, Limpens J, Knops RE, Bouma BJ, Mulder BJ, de Groot JR. Eur Heart J. 2016 Feb 11. PMID:26873095

Comment from Dr. Akash Patel (San Francisco, CA), section editor of Congenital Heart Electrophysiology Journal Watch:  This systematic review and meta-analysis of published studies prior to November 2014 was used to investigate the indications, efficacy, and ICD-related harm for ICD implantation in adults with congenital heart disease.

The study utilized standard medical publication databases and included all patients ≥ 16 years who had congenital heart disease and underwent ICD implantation and met inclusion/exclusion criteria for the study.

Twenty-four studies were included which identified 2162 ACHD patients who underwent ICD implantation.  Transvenous ICD was employed in at least 96% of patients. The mean age was 36 years with the majority of patients having repaired tetralogy of Fallot or transposition of the great arteries (see figure below adapted from paper).

ep 2.1

Approximately half (53%) underwent implantation for primary prevention. Indications included predominately non-sustained ventricular tachycardia, impaired systemic ventricular function, inducible ventricular tachycardia, and syncope (see figure below adapted from paper).

ep 2.2

The remainder underwent implantation for secondary prevention: sustained VT (61%) and cardiac arrest (39%).

Secondary aim analysis was based on complete data that was only available for 20-25% on to the total cohort.

Regarding appropriate implantable cardioverter-defibrillator interventions, 24% of patients received one or more appropriate interventions with a mean follow-up of 3.7 years.  Twenty-two percent of patients with primary prevention and 35% of patients with secondary prevention received appropriate interventions.  There was no difference seen in the pooled data between those with or without tetralogy of Fallot.

Regarding inappropriate implantable cardioverter-defibrillator interventions, 25% of patients received one or more inappropriate interventions with a mean follow-up of 3.7 years.  The majority of inappropriate shocks occurred due to supraventricular tachycardia (68%).

Regarding implantable cardioverter-defibrillator complications,  26% of patients had an ICD-related complication with a mean follow-up of 3.8 years.  The majority of complications (76%) involved lead- or generator-related issues (see figure below adapted from paper).

ep 2.3

Regarding mortality, there was no ICD-related death reported.  Overall, 10% of patients died (based on 15 studies with n=440) with a mean follow-up of 3.7 years.  The majority of deaths were due to heart failure (41%) and other cardiac causes (11%).  Interestingly, 18% of patients had sudden cardiac death despite ICD implantation. Data regarding the specifics were not provided.

This study nicely summarizes and adds to our understanding of who is receiving ICD implantation in patients with ACHD. Almost half of all ACHD patients who received ICDs are for primary prevention with the main indications including non-sustained or inducible ventricular arrhythmias, impaired systemic ventricular function, and syncope.  The largest cohorts include tetralogy of Fallot or Transposition of the great arteries.

However, this study does not aid our understanding of who should receive ICD therapy based on specific congenital defects and what pre-implantation risk factors predicted appropriate therapy.   Though ICD therapy was effective in 24% of patients, there continues to be an increased rate of inappropriate therapy and ICD-related complications as compared to the non-ACHD population.   Unfortunately, this study design does not provide the specifics of each individual case to allow for a more robust analysis that would impact further recommendations regarding ICD therapy use in the ACHD population.

Take Home Points:

  1. Knowledge of the outcomes of ICD implantation in ACHD patients is limited as compared to adult patients with non-structural adult heart disease and follow-up times are shorter.
  2. Almost half of patients (53%) underwent primary prevention implantation with the majority of patients having tetralogy of Fallot or transposition of the great arteries.
  3. Appropriate ICD therapy was demonstrated in 24% of patients.
  4. Inappropriate shocks occurred in 25% of patients.
  5. ICD-related complications were seen in 26% of patients.
  6. The overall mortality occurred in 10% of ACHD patients with ICD implantation though the follow-up duration and data is limited and not generalizable.

 

3. The Impact of Clinical and Genetic Findings on The Management of Young Brugada Syndrome Patients. Andorin A, Behr ER, Denjoy I, Crotti L, Dagradi F, Jesel L, Sacher F, Petit B, Mabo P, Maltret A, Wong LC, Degand B, Bertaux G, Maury P, Dulac Y, Delasalle B, Gourraud JB, Babuty D, Blom NA, Schwartz PJ, Wilde AA, Probst V. Heart Rhythm. 2016 Feb 24. PMID:26921764

Comment from Dr. Akash Patel (San Francisco, CA), section editor of Congenital Heart Electrophysiology Journal Watch:  This multi-center study from 16 European tertiary care centers aimed to describe the clinical presentation of Brugada Syndrome below the age of 19, to identify prognostic factors useful to improve risk stratification, and to propose an approach to management of this population.  This is the largest study to date regarding Brugada syndrome in young patients.

The study included 106 patients with a type 1 Brugada pattern either spontaneously or drug-induced.   The median age at diagnosis was 11 years with a median follow-up of 4.5 years with no sex differences seen under the age of 15.  Family history of sudden cardiac death was present in 43%.

Only 20% of patients were symptomatic at diagnosis which included syncope (71%), aborted sudden cardiac death (20%) and ventricular tachycardia (9%).  Males were 2.5 times more likely to have symptoms. The majority of patients were asymptomatic at diagnosis likely due to familial screening (63%).  Typically, sudden death with this disease is mainly seen in the adult population but interestingly, 5 of the 6 patients with aborted sudden cardiac death and ventricular tachycardia were under the age of 5 years.

The advent of genetic testing and familial screening has improved detection of this disease as mentioned.  Only 75 patients in the cohort underwent genetic testing but 77% were genotype positive.  In addition, 43% of index probands demonstrated a positive test for SCN5A mutations.  Interestingly, 16% of genotype positive patients had a life threatening event during follow-up while no patients with negative testing did.  However, this study lacked the ability to determine whether the presence or absence of an SCNA mutation would be useful for risk stratification.

ECG findings at diagnosis revealed that 34% presented with spontaneous type 1 Brugada pattern while the remaining were elicited with provocative testing.  Males were more likely to have a spontaneous type 1 Brugada pattern while females were more likely to have a drug-induced type 1 Brugada pattern.  Interestingly, spontaneous Brugada pattern patients were more symptomatic at diagnosis (39%) compared to drug-induced Brugada pattern (10%).   In addition, 19% of patients with spontaneous type I pattern only manifested the findings during a febrile episode.

The role of electrophysiology study (EPS) was assessed but limited to only 22 patients (21% of the total cohort) who underwent testing.  Nine patients were symptomatic and had a positive test or inducible VF.  Only 3 total patients (2 with a  positive EPS and 1 with a negative EPS) had a life threatening event during follow-up.  Based on the limited data, this study was unable to address the role of EPS in the risk stratification of young patients with Brugada syndrome.

Treatment for Brugada syndrome included 21% of patients with ICDs with the majority for primary prevention (86%), pacemakers (4%) for symptomatic sinus node dysfunction,  and quinidine (10%) for patients with the  spontaneous type I pattern and arrhythmic event(s).

Follow-up as mentioned was for a median of 4.5 years with 10 patients (9%) having 15 life threatening arrhythmias defined as sudden death, documented VT or VF, or appropriate ICD shock.  Fever was associated with 4 events (27%).  There were 3 deaths (3%), all in patients with a spontaneous Type 1 Brugada pattern. Two died during a febrile illness and 1 died following admission for an aborted sudden cardiac death.  There were 3 episodes of VT during hospital admission for fever related episodes.  The majority was symptomatic at diagnosis (70%) and was genotype positive for a SCN5A mutation (90%).

Predictors of life threatening arrhythmias that were significant included symptoms at diagnosis (Hazard Ratio: 5.9) and spontaneous Type 1 pattern on electrocardiogram  (Hazard Ratio: 2.7).  In addition patients with spontaneous Type 1 pattern and symptoms at presentation had a shorter time to first event (Hazard ratio: 28.9) as compared to those with drug-induced Type 1 pattern and no symptoms at diagnosis.

Overall, this study adds to our understanding of Brugada syndrome in young patients.  Patients with symptoms at diagnosis and a spontaneous type I pattern have a higher incidence of life threatening arrhythmias and a shorter time to first event.  Patients with drug-induced type I Brugada pattern and no symptoms at diagnosis have a low arrhythmic risk and good prognosis during study follow-up. There continues to be lack of clarity regarding EP study findings and genetic testing results in terms of risk stratification. Therapy in symptomatic patients with a spontaneous type I pattern should include consideration for ICD implantation and/or quinidine therapy.  As Brugada syndrome is rare diagnosis in the pediatric population, there is an increasing rate of diagnosis due to genetic testing.  Therapy should thus be considered on a case-by-case basis.  The study authors proposed the following paradigm which is a reasonable approach (see figure below adapted from paper).

Take Home Points:

  1. One in 5 patients with Brugada syndrome presented with symptoms at diagnosis with a male predominance.
  2. Syncope was the most common symptom (71%).
  3. Genetic testing is useful if genotype positive relatives are available.
  4. One-third of patients had a spontaneous Type I Brugada Pattern with 19% only manifesting this finding during fever.
  5. Spontaneous Type I Brugada Pattern and symptoms at diagnosis are predictive of a life threatening arrhythmia (43%) and a shorter time to the first life threatening arrhythmia.
  6. Drug-induced spontaneous Type I Brugada Pattern and no symptoms at diagnosis are predictive of a low arrhythmic risk (2%) and good prognosis.
  7. ICD and/or quinidine therapy should be considered in symptomatic patients with spontaneous type I Brugada Pattern.
  8. Fever triggered events occurred and the authors advise for aggressive treatment even though events during fevers may be unpredictable.
  9. A case-by-case approach should be used for management decisions in young patients with Brugada syndrome.

 

CHD Electrophysiology Featured Articles of January 2016

1. Non-sustained ventricular tachycardia in patients with congenital heart disease: An important sign?

Teuwen CP, Ramdjan TT, Götte M, Brundel BJ, Evertz R, Vriend JW, Molhoek SG, Reinhart Dorman HG, van Opstal JM, Konings TC, van der Voort P, Delacretaz E, Wolfhagen NJ, van Gastel V, de Klerk P, Theuns DA, Witsenburg M, Roos-Hesselink JW, Triedman JK, Bogers AJ, de Groot NM.

Int J Cardiol. 2016 Jan 6. pii: S0167-5273(16)30006-7. doi: 10.1016/j.ijcard.2016.01.042. [Epub ahead of print]

PMID: 26805391

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Select item 26803251

J MooreComment from Dr. Jeremy Moore (Los Angeles, CA), section editor of Congenital Heart Electrophysiology Journal Watch: This study represents the latest from the Netherlands-based DANARA project, a registry established in 2012 to examine the time course of development of post-operative arrhythmias in CHD patients. The group has previously published on the evolution of atrial fibrillation in congenital heart disease patients.1 The current study aimed to determine the prognostic significance of documented non-sustained ventricular tachycardia (NSVT) (defined as ≥ 3 beats of VT at a rate >100 beats/minute lasting ≤30 seconds by ECG, Holter, or CIED electrograms) in adult patients with congenital heart disease.

A total of 145 patients were included in the study and were followed for a median duration of 5 years. The majority of patients (67%) carried one of the following diagnoses: TOF (n=42), TGA (n=19, presumably Mustard/Senning patients), single ventricle heart disease (n=18), or aortic valve disease (n=18). Ventricular arrhythmias were predominantly NSVT (n=103, 71%) and occurred at the youngest age in those patients with single ventricle heart disease (30 ± 12 years) and those with complex CHD (45 ± 13 years).

Sixteen patients (16%) with NSVT underwent ICD implantation. Of these, 3 experienced inappropriate shocks and one died during defibrillation testing.

Five patients with NSVT experienced significant ventricular arrhythmias during the follow-up period. These included an appropriate shock (unspecified ventricular arrhythmia) in a patient with congenital mitral insufficiency, sustained episodes of VT in one patient with TGA and one patient with TOF, and VF in one patient with an ASD and one patient with aortic valve disease. The patient with the ASD died as a result of the ventricular arrhythmia.

Overall, this represents an incidence of approximately 1% per year for significant ventricular arrhythmia developing after initial presentation with NSVT in this study.

The authors suggested that in CHD patients with moderate to good ventricular function, normal or limited conduction delay and NSVT, a “wait-and-see approach” strategy could be justified. They recommended against the use of NSVT as a sole indication for ICD implantation.

A limitation of this study is the attempt to predict risk for a wide spectrum of CHD types, in whom the a priori risk likely differs widely and individual risk factors are of varying importance. Nevertheless, the study provides useful and novel information on this challenging group of patients.

Take home points:

  1. NSVT is relatively common in adult patients with congenital heart disease.
  2. Sustained VT/VF occurred in ~1% of patients presenting with NSVT in this study over a median of 5 years and one patient died.
  3. ICD implantation is not without associated morbidity .

ep 1.1

Teuwen CP, et al. Time Course of Atrial Fibrillation in Patients With Congenital Heart Defects. Circ Arrhythm Electrophysiol. 2015 Oct;8(5):1065-72

 

2. Catheter Ablation for Atrioventricular Nodal Reentry Tachycardia in Patients with Congenital Heart Disease.

Upadhyay S, Marie Valente A, Triedman JK, Walsh EP.

Heart Rhythm. 2016 Jan 21. pii: S1547-5271(16)00066-7. doi: 10.1016/j.hrthm.2016.01.020. [Epub ahead of print]

PMID: 26804568

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Select item 26795904

Comment from Dr. Jeremy Moore (Los Angeles, CA), section editor of Congenital Heart Electrophysiology Journal Watch:  This is a very nice single center study evaluating the characteristics of catheter ablation for AV node reentry tachycardia in patients with congenital heart disease from the Boston Children’s Heart Program. The study was the culmination of a 12-year experience of 49 cases of AVNRT. Congenital diagnoses included DTGA after atrial switch (n=6), CCTGA (n=4), Ebstein anomaly (n=4), TOF (n=5), venous anomalies (n=8), single ventricle (n=16), and miscellaneous lesions (n=6). Interestingly, AVNRT was commonly induced while targeting another tachyarrhythmia in 32% of patients.

Two CHD populations are noteworthy in this study as descriptions are limited in the existing literature:

  1. CCTGA: For patients with CCTGA undergoing attempted catheter ablation (n=3) the successful site of slow pathway modification was encountered in 2 patients: 1) at the conventional location in a patient with l-looped ventricles and 2) in a “mirror image” location on the left side of the septum in a patient with d-looped {I,D,D} ventricles.
  1. Single ventricle anatomy: For patients with single ventricle anatomy, catheter ablation was attempted in 10 patients and was successful in 8/10. The authors comment that cryoablation was commonly used due to uncertainty as to the exact location of the compact AV node for many of these patients. General principles included mapping of the His bundle and coronary sinus os location (whenever possible), identification of highly complex atrial signal, and initiation of catheter ablation at sites well inferior to the location of the His bundle.

Overall, slow pathway modification was attempted in 39/49 cases (80%) and was deemed successful in 36/39 (92%) with only one recurrence over a median follow up of 32 months. There was one case of first degree AV block that persisted after catheter ablation.

The study adds insight into the techniques and challenges for patients with congenital heart disease. In particular, information on patients with CCTGA and those with single ventricle anatomy is particularly useful, given the often unexpected location of the compact AV node in these patients as well as the amplified importance of intact AV conduction for these groups of patients who historically do not do well with ventricular pacing.

Take home points:

  1. AVNRT is observed after almost all forms of palliated/repaired congenital heart disease.
  2. This arrhythmia may be induced during subsequent testing after targeting other forms of tachyarrhythmia by catheter ablation.
  3. Specific subsets of patients, particularly those with single ventricle heart disease may pose particular challenges due to uncertainty to the precise location of the compact AV node.
  4. Acute and long-term success can be achieved in the majority of patients with a limited number of adverse events.

 

3. Subcutaneous implantable cardioverter defibrillators in children, young adults and patients with congenital heart disease.

Bordachar P, Marquié C, Pospiech T, Pasquié JL, Jalal Z, Haissaguerre M, Thambo JB.

Int J Cardiol. 2016 Jan 15;203:251-8. doi: 10.1016/j.ijcard.2015.09.083. Epub 2015 Sep 26. Review.

PMID: 26519678

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Select item 26519677

A PatelComment from Dr. Akash Patel (San Francisco, CA), section editor of Congenital Heart Electrophysiology Journal Watch:  This review article on subcutaneous implantable cardioverter defibrillator provides a balanced review of the benefits and limitations of this new technology in children, young adults, and patients with congenital heart disease.

The authors highlight basic operational differences with subcutaneous ICDs and transvenous ICD including some theoretical limitations such as smaller intracardiac signals, a lower signal-to-noise ratio, and greater postural variations thus requiring the need for a pre-implant screening test to assess eligibility. In addition, the device has limited programming capabilities that allow for ease of programming but limit fine-tuning of the device.  Validation studies for this device are smaller as compared to the transvenous ICD studies but include a larger proportion of young patients, children or patients with congenital heart disease.

Advantages of a subcutaneous ICD include:

  • Absence of an endocardial device
  • Simplified Implantation
  • A theoretically more reliable lead and risk-free extraction procedures
  • Less deleterious effect of electrical shocks/reduction in certain inappropriate therapies

Disadvantages of a subcutaneous ICD include:

  • A certain number of patients are not able to benefit from this system due to negative screening test, young child, patient requiring anti-bradycardia or anti-tachycardia pacing
  • Reduced battery longevity
  • Not currently MRI compatible
  • Longer time for delivered therapy due to latency in arrhythmia detection and longer charge time

Overall, this review highlights the current limited evidence available to help guide appropriate device selection for this unique population. The authors propose preferential indications including that patients with complex congenital heart disease with no venous access to the heart or with a persistent shunt increasing the risk of systemic emboli who do not require long-term pacing and pass the screening test would be suitable candidates. Another preferred group would be young patients with channelopathy or hypertrophic cardiomyopathy who would not receive benefit from anti-tachycardia pacing.

Take home points:

  1. Subcutaneous ICDs offer a less invasive alternative to traditional transvenous and epicardial ICDs with potential applications to children, young adults, and patients with congenital heart disease.
  2. Subcutaneous ICDs use however is limited due to lack of pacing capabilities and need to pass a screening test to assess for eligibility.
  3. Data regarding its use in children, young adults, and patients with congenital heart disease is limited but growing.

 

4. Myocardial ECV Fraction Assessed by CMR Is Associated With Type of Hemodynamic Load and Arrhythmia in Repaired Tetralogy of Fallot.

Chen CA, Dusenbery SM, Valente AM, Powell AJ, Geva T.

JACC Cardiovasc Imaging. 2016 Jan;9(1):1-10. doi: 10.1016/j.jcmg.2015.09.011. Epub 2015 Dec 9.

PMID: 26684969

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Select item 26798219

Comment from Dr. Akash Patel (San Francisco, CA), section editor of Congenital Heart Electrophysiology Journal Watch:  This single center study from the Boston Children’s Hospital aimed to investigate the extent of diffuse myocardial fibrosis by measuring left (LV) and right ventricular (RV) extracellular volume fraction (ECV) in patients with repaired tetralogy of Fallot (rTOF) and to explore its association with ventricular remodeling, hemodynamic load, and clinical parameters.  MRI assessment of ECV is a relatively new technique for measuring diffuse myocardial fibrosis that is an early sign of adverse myocardial remodeling.

The study included 83 patients with a median age of 23 years  with repaired tetralogy of Fallot who underwent an MRI using a special technique to access ECV.   The cohort was compared between volume load lesions  (pulmonary regurgitation fraction ≥25% and peak RVOT gradient ≤25 mm Hg, n = 45 ) and mixed volume and pressure load lesions (all remaining patients, n =39).

ECV for both the right and left ventricles demonstrated a positive and linear correlation suggesting that the diffuse myocardial fibrosis affects both ventricles.  Abnormally elevated ECV was seen in the left ventricle in 13.1% and right ventricle in 10.7% of the patients and was not dependent on anatomic diagnosis and age of repair.  =

Patients with volume overload had a significantly greater RV and LV ECV than those with mixed pressure/volume loads.   Moreover, analysis of the entire cohort found that RV ECV was inversely related to degree of right-sided obstruction and hypertrophy. There was no difference in RV ejection fraction (47% for the total cohort) which would suggest that the ECV changes occur before a change in function.  However, the mixed volume/pressure load group had a significantly lower LV EF at 52% vs 56% which cannot be clearly explained by the degree of myocardial fibrosis (LV ECV).

There were 34 arrhythmias detected in 25 patients (30%) of the total cohort as shown below with no difference between the volume and mixed pressure/volume group. See table below.

All Patients(n = 84) Volume Overload(n = 45) Mixed Volume-Pressure Load(n = 39) p-value
 Arrhythmias 25 (29.8) 15 (33.3) 10 (25.6) 0.441
 Frequent ventricular ectopy 11 6 5
  Nonsustained VT 2 1 1
 Sustained VT 1 1 0
 Supraventricular tachycardia 12 7 5
 Atrial flutter 7 4 3
 Atrial fibrillation 1 1 0

Multivariate analysis showed increased LV ECV (>28%) and not RV ECV predicted arrhythmia risk with an OR of 5.69.  However, the majority of the arrhythmias in this cohort were supraventricular arrhythmias (59%) that may not reflect any true pathophysiologic relationship with ventricular fibrosis identified in this study. Though not stated, of interest would be the relationship of ventricular ectopy and ventricular tachycardia based on degree of LV and/or RV ECV, which would provide an clue as to identifying at risk patients.

This study adds in our understanding of adverse myocardial remodeling in patients with repaired tetralogy of Fallot and provides a potential role for cardiac MRI to aid in identifying at-risk patients prior to overt changes in cardiac function.   From an arrhythmia standpoint, there is clear evidence that significant ventricular dysfunction and ventricular arrhythmias are clear indicators of sudden death risk.  The changes seen in ventricular maladaption defined by ECV are only seen a portion of the total cohort (10-13%) and may potentially provide another useful marker for identification of at-risk patients for heart failure, ventricular arrhythmias, and sudden death.  Unfortunately, this study design excluded patients with ICDs and pacemakers likely due to MRI compatibility issues that limited the number of patients with ventricular arrhythmias.  Therefore, one of the study’s conclusions that increased fibrosis defined as LV ECV (>28%) increases arrhythmias risk, though statistically significant and interesting, may not reflect a true cause-and-effect relationship and further investigation is warranted.

Take home points:

  1. A novel method measuring extracellular volume fraction (ECV) was developed utilizing cardiac MRI to assess for early signs of adverse ventricular myocardial remodeling in young adult patients with repair tetralogy of Fallot.
  2. Elevated Extracellular Volume Fraction (i.e. early signs of diffuse myocardial fibrosis) was seen 13% of left ventricles and 11% of right ventricles.
  3. Patients with a residual volume overload lesions (pulmonary regurgitation fraction ≥25% and peak RVOT gradient ≤25 mm Hg) had significantly more adverse remodeling than those with residual mixed or pressure load lesions.
  4. Elevated ECV was associated with increased arrhythmias but the majority were supraventricular tachyarrhythmias (59%) and thus may not reflect a true pathophysiologic relationship .

CHD EP Articles of January 2016

  1. Using a Cardiac Event Recorder in Children with Potentially Arrhythmia-Related Symptoms.

Saygi M, Ergul Y, Ozyilmaz I, Sengul FS, Guvenc O, Aslan E, Guzeltas A, Akdeniz C, Tuzcu V.

Ann Noninvasive Electrocardiol. 2016 Jan 21. doi: 10.1111/anec.12339. [Epub ahead of print]

PMID: 26791967

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Select item 26797468

 

  1. Catheter Ablation for Atrioventricular Nodal Reentry Tachycardia in Patients with Congenital Heart Disease.

Upadhyay S, Marie Valente A, Triedman JK, Walsh EP.

Heart Rhythm. 2016 Jan 21. pii: S1547-5271(16)00066-7. doi: 10.1016/j.hrthm.2016.01.020. [Epub ahead of print]

PMID: 26804568

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Select item 26795904

 

  1. Congenital heart block related to maternal autoantibodies: descriptive analysis of a series of 18 cases from a single center.

Doti PI, Escoda O, Cesar-Díaz S, Palasti S, Teixidó I, Sarquella-Brugada G, Gómez O, Martínez JM, Espinosa G.

Clin Rheumatol. 2016 Jan 20. [Epub ahead of print]

PMID: 26791874

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Select item 26790106

 

  1. Pseudo-reentry of atrial tachycardia after surgical repair of congenital heart disease.

Yokota J, Fujiu K, Kojima T, Komuro I.

J Cardiovasc Electrophysiol. 2016 Jan 17. doi: 10.1111/jce.12932. [Epub ahead of print]

PMID: 26776918

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Select item 26555371

 

  1. Left persistent superior vena cava as a source of focal atrial arrhythmias: A late arrhythmia recurrence due to a latent proximal focus.

Battaglia A, Anselmino M, Ferraris F, Gaita F.

Int J Cardiol. 2016 Jan 15;203:523-4. doi: 10.1016/j.ijcard.2015.10.241. Epub 2015 Nov 2. No abstract available.

PMID: 26562535

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Select item 26551885

 

  1. Subcutaneous implantable cardioverter defibrillators in children, young adults and patients with congenital heart disease.

Bordachar P, Marquié C, Pospiech T, Pasquié JL, Jalal Z, Haissaguerre M, Thambo JB.

Int J Cardiol. 2016 Jan 15;203:251-8. doi: 10.1016/j.ijcard.2015.09.083. Epub 2015 Sep 26. Review.

PMID: 26519678

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Select item 26519677

 

  1. Getting to zero: impact of electroanatomical mapping on fluoroscopy use in pediatric catheter ablation.

Clark BC, Sumihara K, McCarter R, Berul CI, Moak JP.

J Interv Card Electrophysiol. 2016 Jan 15. [Epub ahead of print]

PMID: 26768435

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Select item 26768968

 

  1. Arrhythmia diagnosis and management throughout life in congenital heart disease.

Clark BC, Berul CI.

Expert Rev Cardiovasc Ther. 2016 Jan 9:1-20. [Epub ahead of print]

PMID: 26642231

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Select item 26808989

 

  1. Is non-sustained ventricular tachycardia a predictor of sudden death in adults with congenital heart disease?

Lim E, Wong T.

Int J Cardiol. 2016 Jan 9;207:264-265. doi: 10.1016/j.ijcard.2016.01.161. [Epub ahead of print] No abstract available.

PMID: 26808989

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Select item 26788818

 

  1. Contact force monitoring during catheter ablation of intraatrial reentrant tachycardia in patients with congenital heart disease.

Krause U, Backhoff D, Klehs S, Schneider HE, Paul T.

J Interv Card Electrophysiol. 2016 Jan 7. [Epub ahead of print]

PMID: 26743070

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Select item 26739006

 

  1. Non-sustained ventricular tachycardia in patients with congenital heart disease: An important sign?

Teuwen CP, Ramdjan TT, Götte M, Brundel BJ, Evertz R, Vriend JW, Molhoek SG, Reinhart Dorman HG, van Opstal JM, Konings TC, van der Voort P, Delacretaz E, Wolfhagen NJ, van Gastel V, de Klerk P, Theuns DA, Witsenburg M, Roos-Hesselink JW, Triedman JK, Bogers AJ, de Groot NM.

Int J Cardiol. 2016 Jan 6. pii: S0167-5273(16)30006-7. doi: 10.1016/j.ijcard.2016.01.042. [Epub ahead of print]

PMID: 26805391

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Select item 26803251

 

  1. Tachyarrhythmia in patients with congenital heart disease: inevitable destiny?

Teuwen CP, Taverne YJ, Houck C, Götte M, Brundel BJ, Evertz R, Witsenburg M, Roos-Hesselink JW, Bogers AJ, de Groot NM, Molhoek SG, Ramdjan TT, Helbing WA, Kammeraad JA, Dorman HG, van Opstal JM, Konings TC, Vriend JW, van der Voort P.

Neth Heart J. 2016 Jan 4. [Epub ahead of print]

PMID: 26728051

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Select item 26725480

 

  1. Outcome in 212 anti-Ro/SSA-positive pregnancies and population-based incidence of congenital heart block.

Skog A, Lagnefeldt L, Conner P, Wahren-Herlenius M, Sonesson SE.

Acta Obstet Gynecol Scand. 2016 Jan;95(1):98-105. doi: 10.1111/aogs.12785. Epub 2015 Oct 26.

PMID: 26411741

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Select item 26735234

 

  1. Image-Based Personalization of Cardiac Anatomy for Coupled Electromechanical Modeling.

Crozier A, Augustin CM, Neic A, Prassl AJ, Holler M, Fastl TE, Hennemuth A, Bredies K, Kuehne T, Bishop MJ, Niederer SA, Plank G.

Ann Biomed Eng. 2016 Jan;44(1):58-70. doi: 10.1007/s10439-015-1474-5. Epub 2015 Sep 30.

PMID: 26424476 Free PMC Article

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Select item 26694269

 

  1. Electrocardiographic intervals in foetuses with CHD.

Yilmaz B, Narayan HK, Wilpers A, Wiess C, Fifer WP, Williams IA.

Cardiol Young. 2016 Jan;26(1):84-9. doi: 10.1017/S1047951114002686. Epub 2015 Jan 20.

PMID: 25599806

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Select item 25599703

 

  1. Recognition and management of arrhythmias in adult congenital heart disease.

McLeod CJ, Warnes C.

Curr Opin Cardiol. 2016 Jan;31(1):117-23. doi: 10.1097/HCO.0000000000000251.

PMID: 26569087

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Select item 26463987

 

  1. Heart rate variability in neonates of type 1 diabetic pregnancy.

Russell NE, Higgins MF, Kinsley BF, Foley ME, McAuliffe FM.

Early Hum Dev. 2016 Jan;92:51-5. doi: 10.1016/j.earlhumdev.2015.11.003. Epub 2015 Dec 1.

PMID: 26658058

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Select item 26096717

 

  1. High interobserver variability in the assessment of epsilon waves: Implications for diagnosis of arrhythmogenic right ventricular cardiomyopathy/dysplasia.

Platonov PG, Calkins H, Hauer RN, Corrado D, Svendsen JH, Wichter T, Biernacka EK, Saguner AM, Te Riele AS, Zareba W.

Heart Rhythm. 2016 Jan;13(1):208-16. doi: 10.1016/j.hrthm.2015.08.031. Epub 2015 Aug 21.

PMID: 26304715

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Select item 26476036

 

  1. Myocardial ECV Fraction Assessed by CMR Is Associated With Type of Hemodynamic Load and Arrhythmia in Repaired Tetralogy of Fallot.

Chen CA, Dusenbery SM, Valente AM, Powell AJ, Geva T.

JACC Cardiovasc Imaging. 2016 Jan;9(1):1-10. doi: 10.1016/j.jcmg.2015.09.011. Epub 2015 Dec 9.

PMID: 26684969

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Select item 26798219

 

  1. Sports Eligibility After Risk Assessment and Treatment in Children with Asymptomatic Ventricular Pre-excitation.

Di Mambro C, Drago F, Milioni M, Russo MS, Righi D, Placidi S, Remoli R, Palmieri R, Gimigliano F, Santucci LM, Silvetti MS, Prosperi M.

Sports Med. 2016 Jan 29. [Epub ahead of print]

PMID: 26825778

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Select item 26825626

 

  1. Safety and Efficacy of Prophylactic Amiodarone in Preventing Early Junctional Ectopic Tachycardia (JET) in Children After Cardiac Surgery and Determination of Its Risk Factor.

Amrousy DE, Elshehaby W, Feky WE, Elshmaa NS.

Pediatr Cardiol. 2016 Jan 27. [Epub ahead of print]

PMID: 26818850

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Select item 26818849

 

  1. Response to Letters Regarding Article, “Clinical Management of Catecholaminergic Polymorphic Ventricular Tachycardia: The Role of Left Cardiac Sympathetic Denervation”.

De Ferrari GM, Dusi V, Spazzolini C, Bos JM, Abrams DJ, Berul CI, Crotti L, Eldar M, Kharlap M, Khoury A, Krahn AD, Leenhardt A, Moir CR, Odero A, Nordkamp LO, Paul T, I Noguer FR, Shkolnikova M, Till J, Wilde AA, Ackerman MJ, Schwartz PJ.

Circulation. 2016 Jan 26;133(4):e366-7. doi: 10.1161/CIRCULATIONAHA.115.019465. No abstract available.

PMID: 26811283

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Select item 26796398

 

  1. Exercise performance in young patients with complete atrioventricular block: the relevance of synchronous atrioventricular pacing.

Gonzalez Corcia MC, Remy LS, Marchandise S, Moniotte S.

Cardiol Young. 2016 Jan 22:1-6. [Epub ahead of print]

PMID: 26796814

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Select item 26791967

 

  1. Using a Cardiac Event Recorder in Children with Potentially Arrhythmia-Related Symptoms.

Saygi M, Ergul Y, Ozyilmaz I, Sengul FS, Guvenc O, Aslan E, Guzeltas A, Akdeniz C, Tuzcu V.

Ann Noninvasive Electrocardiol. 2016 Jan 21. doi: 10.1111/anec.12339. [Epub ahead of print]

PMID: 26791967

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Select item 26796135

 

  1. Recurrent Muscle Weakness with Rhabdomyolysis, Metabolic Crises, and Cardiac Arrhythmia due to Bi-allelic TANGO2 Mutations.

Lalani SR, Liu P, Rosenfeld JA, Watkin LB, Chiang T, Leduc MS, Zhu W, Ding Y, Pan S, Vetrini F, Miyake CY, Shinawi M, Gambin T, Eldomery MK, Akdemir ZH, Emrick L, Wilnai Y, Schelley S, Koenig MK, Memon N, Farach LS, Coe BP, Azamian M, Hernandez P, Zapata G, Jhangiani SN, Muzny DM, Lotze T, Clark G, Wilfong A, Northrup H, Adesina A, Bacino CA, Scaglia F, Bonnen PE, Crosson J, Duis J, Maegawa GH, Coman D, Inwood A, McGill J, Boerwinkle E, Graham B, Beaudet A, Eng CM, Hanchard NA, Xia F, Orange JS, Gibbs RA, Lupski JR, Yang Y.

Am J Hum Genet. 2016 Jan 19. pii: S0002-9297(15)00503-0. doi: 10.1016/j.ajhg.2015.12.008. [Epub ahead of print]

PMID: 26805781

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Select item 26783281

 

  1. Absence of Change in Corrected QT Interval in Children and Adolescents Receiving Antipsychotic Treatment: A 12 Month Study.

Alda JA, Muñoz-Samons D, Tor J, Merchán-Naranjo J, Tapia-Casellas C, Baeza I, Calvo-Escalona R, Castro-Fornieles J, Martínez-Cantarero C, Andrés-Nestares P, Fernández-Avilés F, Arango C.

J Child Adolesc Psychopharmacol. 2016 Jan 18. [Epub ahead of print]

PMID: 26779966

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  1. Sphingomonas paucimobilis causing pacemaker pocket infection in a pediatric patient with a hemangioma.

Shi X, Liu R.

Am J Infect Control. 2016 Jan 15. pii: S0196-6553(15)01216-X. doi: 10.1016/j.ajic.2015.11.023. [Epub ahead of print] No abstract available.

PMID: 26781221

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Select item 26608339

 

  1. Athletes with implantable cardioverter defibrillators: can they return to competitive sports?

Prutkin JM, Ackerman MJ, Drezner JA.

Heart. 2016 Jan 15;102(2):93-4. doi: 10.1136/heartjnl-2015-309152rep. No abstract available.

PMID: 26729607

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Select item 26604136

 

  1. Multifocal atrial tachycardia caused by risperidone.

Oner T, Akdeniz C, Adaletli H.

Int J Cardiol. 2016 Jan 15;203:855-7. doi: 10.1016/j.ijcard.2015.10.234. Epub 2015 Oct 30.

PMID: 26599751

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  1. Getting to zero: impact of electroanatomical mapping on fluoroscopy use in pediatric catheter ablation.

Clark BC, Sumihara K, McCarter R, Berul CI, Moak JP.

J Interv Card Electrophysiol. 2016 Jan 15. [Epub ahead of print]

PMID: 26768435

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  1. Wireless Ultrasound-Guided Axillary Vein Cannulation for the Implantation of Cardiovascular Implantable Electric Devices.

Franco E, Muñoz DR, Matía R, Hernandez-Madrid A, Román AC, Sánchez I, Zamorano J, Moreno J.

J Cardiovasc Electrophysiol. 2016 Jan 9. doi: 10.1111/jce.12917. [Epub ahead of print]

PMID: 26749504

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  1. Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia (ARVC/D): Review of 16 Pediatric Cases and a Proposal of Modified Pediatric Criteria.

Deshpande SR, Herman HK, Quigley PC, Shinnick JK, Cundiff CA, Caltharp S, Shehata BM.

Pediatr Cardiol. 2016 Jan 8. [Epub ahead of print]

PMID: 26743400

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Select item 26788689

 

  1. Current approaches to the clinical assessment of syncope in pediatric population.

Bayram AK, Pamukcu O, Per H.

Childs Nerv Syst. 2016 Jan 5. [Epub ahead of print]

PMID: 26732063

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  1. Tachyarrhythmia in patients with congenital heart disease: inevitable destiny?

Teuwen CP, Taverne YJ, Houck C, Götte M, Brundel BJ, Evertz R, Witsenburg M, Roos-Hesselink JW, Bogers AJ, de Groot NM, Molhoek SG, Ramdjan TT, Helbing WA, Kammeraad JA, Dorman HG, van Opstal JM, Konings TC, Vriend JW, van der Voort P.

Neth Heart J. 2016 Jan 4. [Epub ahead of print]

PMID: 26728051

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  1. Ventricular tachycardia in a child with diabetic ketoacidosis without heart disease.

McGreevy M, Beerman L, Arora G.

Cardiol Young. 2016 Jan;26(1):206-8. doi: 10.1017/S1047951115000621. Epub 2015 Oct 8.

PMID: 26446852

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  1. Brugada syndrome: clinical and genetic findings.

Sarquella-Brugada G, Campuzano O, Arbelo E, Brugada J, Brugada R.

Genet Med. 2016 Jan;18(1):3-12. doi: 10.1038/gim.2015.35. Epub 2015 Apr 23. Review.

PMID: 25905440

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  1. Quality of life after videoscopic left cardiac sympathetic denervation in patients with potentially life-threatening cardiac channelopathies/cardiomyopathies.

Antiel RM, Bos JM, Joyce DD, Owen HJ, Roskos PL, Moir C, Ackerman MJ.

Heart Rhythm. 2016 Jan;13(1):62-9. doi: 10.1016/j.hrthm.2015.09.001. Epub 2015 Sep 1.

PMID: 26341607

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  1. Automated detection of ventricular pre-excitation in pediatric 12-lead ECG.

Gregg RE, Zhou SH, Dubin AM.

J Electrocardiol. 2016 Jan-Feb;49(1):37-41. doi: 10.1016/j.jelectrocard.2015.08.006. Epub 2015 Aug 4.

PMID: 26320370

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  1. Early Cardiac Involvement and Risk Factors for the Development of Arrhythmia in Patients With β-Thalassemia Major.

Hamed AA, Elguindy W, Elhenawy YI, Ibrahim RH.

J Pediatr Hematol Oncol. 2016 Jan;38(1):5-11. doi: 10.1097/MPH.0000000000000467.

PMID: 26583617

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  1. Tricking CARTO: Cryoablation of Supraventricular Tachycardia in Children with Minimal Radiation Exposure Using the CARTO3 System.

Ceresnak SR, Nappo L, Janson CM, Pass RH.

Pacing Clin Electrophysiol. 2016 Jan;39(1):36-41. doi: 10.1111/pace.12754. Epub 2015 Oct 30.

PMID: 26412504

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Select item 26391534

 

  1. Intermittent Preexcitation: Should We Rethink the Current Guidelines?

Cohen M.

Pacing Clin Electrophysiol. 2016 Jan;39(1):9-11. doi: 10.1111/pace.12745. Epub 2015 Sep 22. No abstract available.

PMID: 26391534

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Select item 26256551

 

  1. Intermittent versus Persistent Wolff-Parkinson-White Syndrome in Children: Electrophysiologic Properties and Clinical Outcomes.

Kiger ME, McCanta AC, Tong S, Schaffer M, Runciman M, Collins KK.

Pacing Clin Electrophysiol. 2016 Jan;39(1):14-20. doi: 10.1111/pace.12732. Epub 2015 Sep 11.

PMID: 26256551

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  1. ECGs in the ED.

Tanel RE.

Pediatr Emerg Care. 2016 Jan;32(1):60-1. doi: 10.1097/PEC.0000000000000700. No abstract available.

PMID: 26720070

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