Long-Term Follow-Up of Patients With Tetralogy of Fallot and Implantable Cardioverter Defibrillator
Authors: Waldmann Victor , Bouzeman Abdeslam , Duthoit Guillaume , Koutbi Linda , Bessiere Francis , Labombarda Fabien , Marquié Christelle , Gourraud Jean Baptiste , Mondoly Pierre , Sellal Jean Marc , Bordachar Pierre , Hermida Alexis , Anselme Frédéric , Asselin Anouk , Audinet Caroline , Bernard Yvette , Boveda Serge , Bru Paul , Bun Sok Sithikun , Clerici Gael , Da Costa Antoine , de Guillebon Maxime , Defaye Pascal , Elbaz Nathalie , Eschalier Romain , Garcia Rodrigue , Guenancia Charles , Guy-Moyat Benoit , Halimi Franck , Irles Didier , Iserin Laurence , Jourda François , Ladouceur Magalie , Lagrange Philippe , Laredo Mikael , Mansourati Jacques , Massoulié Grégoire , Mathiron Amel , Maury Philippe , Messali Anne , Narayanan Kumar , Nguyen Cédric , Ninni Sandro , Perier Marie-Cécile , Pierre Bertrand , Pujadas Penelope , Sacher Frédéric , Sagnol Pascal , Sharifzadehgan Ardalan , Walton Camille , Winum Pierre , Zakine Cyril , Fauchier Laurent , Martins Raphael , Pasquié Jean Luc , Thambo Jean Benoit , Jouven Xavier , Combes Nicolas , Marijon Eloi , Squara Fabien , Theodore Guillaume , Scarlatti Didier , Hourdain Jérome , Deharo Jean-Claude , Ollitrault Pierre , Milliez Paul , Champ-Rigot Laure , Bader Hugues , Duplantier Cécile , Milhem Antoine , Sagnard Audrey , Bertaux Géraldine , Laurent Gabriel , Badoz Marc , Bortone Agustin , Laborie Guillaume , Rollin Anne , Stephanopoli Quentin Voglimacci , Mandel Franck , Duparc Alexandre , Domain Guillaume , Albenque Jean-Paul , Cardin Christèle , Combes Stéphane , Tanese Nikita , Hasni Karim , Leclercq Christophe , Galand Vincent , Pavin Dominique , Mabo Philippe , Behar Nathalie , Clementy Nicolas , Loose Christophe , Otmani Akli , Venier Sandrine , Carabelli Adrien , Jacon Peggy , Kilani Mouna Ben , Guichard Jean Bapstist , Romeyer-Bouchard Cécile , Le Gloan Laurianne , Probst Vincent , Freysz Luc , Blangy Hugues , Chillou Christian De , Sadoul Nicolas , Khattar Pierre , Potelle Charlotte , Jean Frederic , Puie Paul , Khoueiry Ziad , Chevallier Philippe , Dulac Arnaud , Filippo Sylvie Di , Gardey Kevin , Frey Pierre , Akret Chrystelle , Dompnier Antoine , Maupain Carole , Waintraub Xavier , Hidden-Lucet Françoise , Chastre Thomas , Gandjbakhch Estelle , Badenco Nicolas , Extramiana Fabrice , Leenhardt Antoine , Zouaghi Amir , Algalarrondo Vincent , Amet Denis , Varlet Emilie , Chalbia Tej , Philibert Séverine , Ollitrault Jacky , Lavergne Thomas , Baudinaud Pierre , Mirolo Adrian , Savouré Arnaud , Godin Bénédicte , Bertrand Cathy , Fiorello Pierre , Johnson Nicolas , Lallemand Pierre-Marc , Herminda Alexis , Hermida Jean-Sylvain , Degand Bruno , Bouazzaou Rim El , Mourot Stéphane , Goussot Samuel , Jauvert Gaël , Lazarus Arnaud , Grimard Caroline , Alonso Christine , Zhao Alexandre , Thomas Olivier , Cauchemez Bruno , Moubarak Ghassan , Lellouche Nicolas , Hamon David , Enache Bogdan , Lactu Gabriel , Wiart Françoise , Geoffroy Olivier , Poindron Damien , Maltret Alice , Raimondo Cristine , Bonnet Damien , Hascoët Sébastien , Derval Nicolas , Haïssaguerre Michel , Hocini Mélèze 
Published: October 2020

Background:
Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart disease, and sudden cardiac death represents an important mode of death in these patients. Data evaluating the implantable cardioverter defibrillator (ICD) in this patient population remain scarce.

Methods:
A Nationwide French Registry including all patients with tetralogy of Fallot with an ICD was initiated in 2010 by the French Institute of Health and Medical Research. The primary time to event end point was the time from ICD implantation to first appropriate ICD therapy. Secondary outcomes included ICD-related complications, heart transplantation, and death. Clinical events were centrally adjudicated by a blinded committee.

Results:

A total of 165 patients (mean age, 42.2±13.3 years, 70.1% males) were included from 40 centers, including 104 (63.0%) in secondary prevention. During a median (interquartile range) follow-up of 6.8 (2.5–11.4) years, 78 (47.3%) patients received at least 1 appropriate ICD therapy. The annual incidence of the primary outcome was 10.5% (7.1% and 12.5% in primary and secondary prevention, respectively;
P
=0.03). Overall, 71 (43.0%) patients presented with at least 1 ICD complication, including inappropriate shocks in 42 (25.5%) patients and lead dysfunction in 36 (21.8%) patients. Among 61 (37.0%) patients in primary prevention, the annual rate of appropriate ICD therapies was 4.1%, 5.3%, 9.5%, and 13.3% in patients with, respectively, 0, 1, 2, or ≥3 guidelines-recommended risk factors. QRS fragmentation was the only independent predictor of appropriate ICD therapies (hazard ratio, 3.47 [95% CI, 1.19–10.11]), and its integration in a model with current criteria increased the 5-year time-dependent area under the curve from 0.68 to 0.81 (
P
=0.006). Patients with congestive heart failure or reduced left ventricular ejection fraction had a higher risk of nonarrhythmic death or heart transplantation (hazard ratio, 11.01 [95% CI, 2.96–40.95]).

Conclusions:
Patients with tetralogy of Fallot and an ICD experience high rates of appropriate therapies, including those implanted in primary prevention. The considerable long-term burden of ICD-related complications, however, underlines the need for careful candidate selection. A combination of easy-to-use criteria including QRS fragmentation might improve risk stratification.

Registration:

URL:
https://www.clinicaltrials.gov
; Unique identifier: NCT03837574.