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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July 2017 ep 4


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.

July 2017 ep 5

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.

July 2017 ep 6

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)

July 2017 ep 7

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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Select item 28681466


  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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Select item 28709631


  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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Select item 28672919


  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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Select item 28064364


  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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Select item 28514463


  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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Ventricular arrhythmia risk stratification in patients with tetralogy of Fallot at the time of pulmonary valve replacement.

Ventricular arrhythmia risk stratification in patients with tetralogy of Fallot at the time of pulmonary valve replacement.

Rotes AS, Connolly HM, Warnes CA, Ammash NM, Phillips SD, Dearani JA, Schaff HV, Burkhart HM, Hodge DO, Asirvatham SJ, McLeod CJ. Circ Arrhythmia  Electrophysiol. 2015 Feb;8(1):110-6.

Comment from Dr. Mitchell Cohen (Phoenix), editor of congenital heart disease electrophysiology Journal Watch: The Mayo Clinic group from Rochester has once again provided the reader with a long-standing retrospective follow-up of ventricular arrhythmia risk stratification in 205 adults with tetralogy of Fallot between 1988-2010. Although the time frame spans a number of years during which surgical, catheter, and electrophysiologic based interventions evolved, a number of important findings were illustrated. The primary outcome was a combined event including ventricular tachycardia, out-of-hospital cardiac arrest, appropriate ICD therapy, and sudden cardiac death. Freedom from the combined event at 5, 10, and 15 years was 95%, 90%, and 79%, respectively. The two findings that had the greatest association with having an event were a history of ventricular arrhythmias and left ventricular dysfunction. The identification of having ventricular arrhythmias predicting ventricular arrhythmias or ICD shock is not surprising. However, the investigators identified LV dysfunction categorically defined as an LVEF <50% as a significant hazard ratio. This is in line with other previously published data from the group at Toronto Sick Kids where LV dysfunction in post-op TOF patients was a risk factor for ventricular arrhythmias as well as with a data published by Paul Khairy that LV diastolic dysfunction (LVEDP>12 mmHg) also predicted potentially life-threatening arrhythmias. We must as cardiology communities come to the increasing reality that repaired tetralogy of Fallot addressed an RV pressure/volume overload state as a child, but the LV did not inherit life-long immunity. Evidence for either LV systolic or diastolic dysfunction is of concern for ventricular arrhythmias and may merit further noninvasive or invasive electrophysiologic evaluation. Further work in our ACHD/GUCH TOF patients with LV systolic or diastolic dysfunction needs to occur. Can the LV dysfunction improve with improved surgical results, more timely attention to residual lesions, or a more aggressive prophylactic ablation set to reduce the potential for ventricular arrhythmias and maybe even sudden cardiac death?

CALM3 Mutation Associated with Long QT Syndrome.

CALM3 Mutation Associated with Long QT Syndrome.

Reed GF, Boczek NJ, Etheridge SP, Ackerman MJ. Heart Rhythm Vol. 12 Feb 2015

Comment from Dr. Mitchell Cohen (Phoenix), editor of congenital heart disease electrophysiology Journal Watch: This case presentation is interesting for the clinical cardiologist who has a patient suspicious for a diagnosis of a particular channelopathy. In the absence of a confirmatory clinically relevant commercially available test where the suspicion of true disease is high, research laboratories have begun the process of performing whole-exome sequencing.  With parental normal EKGs, the likelihood of a dominant sporadic or recessive inheritance pattern must be considered. In these situations a case-parent-trip utilizes whole exome sequencing to filter the large nodal network of potential LQT genes and identify the likely causative mutation. In this particular patient described, whole exome sequencing was used to identify a CALM3 missense mutation was identified. Genetics are moving fast and the ability to now look at the exome is new frontier for channelopathy identification in our patients.

Abnormal electroencephalogram, epileptic seizures, structural congenital heart disease and aborted sudden cardiac death in Andersen-Tawil syndrome.

Abnormal electroencephalogram, epileptic seizures, structural congenital heart disease and aborted sudden cardiac death in Andersen-Tawil syndrome. Márquez MF, Totomoch-Serra A, Burgoa JA, Méndez A, Gómez-Flores JR, Nava S, Cardenas M. Int J Cardiol. 2015 Feb 1

Comment from Dr. Mitchell Cohen (Phoenix), editor of congenital heart disease electrophysiology Journal Watch: Anderson Tawil Syndrome (ATS) is a clinical condition caused by a mutation in the LQT KCNJ2 gene. ATS is characterized by muscle weakness (periodic paralysis) and ventricular arrhythmias. The physical features of patients with ATS include micrognathia, low-set ears, widely spaced eyes, clinodactyly, and scoliosis ATS is autosomal dominant and the disease has its onset in childhood and is slowly progressive. Marquez and colleagues from Mexico City present a 7 year-old girl with ATS who also had structural heart disease with an abnormal mitral valve requiring cardiac surgery. The findings of congenital heart disease with ATS are unusual. However, the case report is worthwhile reading as pediatric cardiologists, neurologist, and emergency room physicians should be suspicious of children presenting with syncope or seizures with abnormal potassium levels, muscle weakness, periodic paralysis and atypical facial appearances.