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