Long-Term Follow-Up of Patients with Tetralogy of Fallot and Implantable Cardioverter Defibrillator: The DAI-T4F (Implantable Cardioverter Defibrillator-Tetralogy of Fallot) Nationwide Registry

Victor Waldmann (1, 2) , Abdeslam Bouzeman (1, 3) , Guillaume Duthoit (4) , Linda Koutbi (5) , Francis Bessiere (6) , Fabien Labombarda (7) , Christelle Marquié (8) , Jean Baptiste Gourraud (9) , Pierre Mondoly (10) , Jean Marc Sellal (11) , Pierre Bordachar (12) , Alexis Hermida (13) , Frédéric Anselme (14) , Anouk Asselin (1) , Caroline Audinet (15) , Yvette Bernard (16) , Serge Boveda (1, 17, 18) , Paul Bru , Sok Sithikun Bun (19) , Gael Clerici (20) , Antoine da Costa (21) , Maxime de Guillebon , Pascal Defaye (22) , Nathalie Elbaz (23) , Romain Eschalier (24, 25) , Rodrigue Garcia (26) , Charles Guenancia (27) , Benoit Guy-Moyat (28) , Franck Halimi (3) , Didier Irles (29) , Laurence Iserin (2) , Fraçois Jourda (30) , Magalie Ladouceur (1, 2) , Philippe Lagrange (31) , Mikael Laredo (4) , Jacques Mansourati , Grégoire Massoullié (24) , Amel Mathiron (13) , Philippe Maury (10) , Anne Messali (32) , Kumar Narayanan (1) , Cédric Nguyen (33) , Sandro Ninni (8) , Marie-Cécile Périer (1) , Bertrand Pierre (34) , Penelope Pujadas (35) , Frédéric Sacher (12) , Pascal Sagnol (33) , Ardalan Sharifzadehgan (1, 2) , Camille Walton (6) , Pierre Winum (36) , Cyril Zakine (37) , Laurent Fauchier (34) , Raphael Martins (38, 39) , Jean Luc Pasquié (38, 40) , Jean Benoit Thambo (12) , Xavier Jouven (1, 2) , Nicolas Combes (41) , Eloi Marijon (1, 2)

1 PARCC (UMR_S 970/ U970) - Paris-Centre de Recherche Cardiovasculaire
2 HEGP - Hôpital Européen Georges Pompidou [APHP]
3 CHV - Centre Hospitalier de Versailles André Mignot
4 CHU Pitié-Salpêtrière [AP-HP]
5 TIMONE - Hôpital de la Timone [CHU - APHM]
6 Hôpital Louis Pradel [CHU - HCL]
7 CHU Caen
8 CHRU Lille - Centre Hospitalier Régional Universitaire [CHU Lille]
9 CHU Nantes - Centre Hospitalier Universitaire de Nantes
10 CHU Toulouse - Centre Hospitalier Universitaire de Toulouse
11 CHRU Nancy - Centre Hospitalier Régional Universitaire de Nancy
12 CHU Bordeaux
13 CHU Amiens-Picardie
14 CHU Rouen
15 GHBS - Groupe Hospitalier Bretagne Sud
16 CHRU Besançon - Centre Hospitalier Régional Universitaire de Besançon
17 VUB - Vrije Universiteit Brussel [Bruxelles]
18 Clinique Pasteur [Toulouse]
19 CHU - Hôpital Pasteur [Nice]
20 CHU Saint-Pierre
21 CHU ST-E - Centre Hospitalier Universitaire de Saint-Etienne [CHU Saint-Etienne]
22 CHUGA - Centre Hospitalier Universitaire [CHU Grenoble]
23 CHU Henri Mondor [Créteil]
24 CHU Clermont-Ferrand
25 UCA - Université Clermont Auvergne
26 CHU de Poitiers - Centre hospitalier universitaire de Poitiers = Poitiers University Hospital
27 CHU Dijon
28 CHU Limoges
29 Centre Hospitalier Annecy-Genevois [Saint-Julien-en-Genevois]
30 CHA - Centre hospitalier d'Auxerre
31 Clinique Saint Pierre, Perpignan
32 AP-HP - Hôpital Bichat - Claude Bernard [Paris]
33 Centre Hospitalier Chalon-sur-Saône William Morey
34 CHU Trousseau [Tours]
35 Hôpital privé les Franciscaines
36 CHU Nîmes - Centre Hospitalier Universitaire de Nîmes
37 Clinique Saint Gatien
38 PhyMedExp - Physiologie & médecine expérimentale du Cœur et des Muscles [U 1046]
39 CHU Montpellier
40 CHRU Montpellier - Centre Hospitalier Régional Universitaire [Montpellier]
41 Pasteur Clinic, Toulouse, France

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.

Domaines: Sciences du Vivant [q-bio]

Victor Waldmann, Abdeslam Bouzeman, Guillaume Duthoit, Linda Koutbi, Francis Bessiere, et al.. Long-Term Follow-Up of Patients with Tetralogy of Fallot and Implantable Cardioverter Defibrillator: The DAI-T4F (Implantable Cardioverter Defibrillator-Tetralogy of Fallot) Nationwide Registry. Circulation, In press, 142 (17), pp.1612-1622. ⟨10.1161/CIRCULATIONAHA.120.046745⟩. ⟨hal-02958808⟩