Nathalie Morel1, Kateri Lévesque1, Alice Maltret2, Gabriel Baron3, Mohamed Hamidou4, Pauline Orquevaux5, Jean-Charles Piette6, François Barriere7, Jérôme Le Bidois2, Laurent Fermont2, Olivier Fain8, Arnaud Theulin9, François Sassolas10, Quentin Hauet11, Gaëlle Guettrot-Imbert1, Sophie Georgin-Lavialle12, Christophe Deligny13, Eric Hachulla14, Luc Mouthon1, Claire Le Jeunne1, Philippe Ravaud15, Delphine Le Mercier16, Bénédicte Romefort17, Elisabeth Villain2, Damien Bonnet2, Nathalie Costedoat-Chalumeau18. 1. AP-HP, Hôpital Cochin, Centre de référence maladies auto-immunes et systémiques rares, Paris, France. 2. APHP, Centre de référence Malformations Cardiaques Congénitales Complexes - M3C, Hôpital Necker, Paris, France; Université Paris Descartes-Sorbonne Paris Cité, Paris, France. 3. AP-HP, Hôpital Hôtel-Dieu, Centre d'Epidémiologie Clinique, Paris, France; Université Paris Descartes-Sorbonne Paris Cité, Paris, France. 4. Service de Médecine Interne, CHU Nantes, France. 5. CHU de Reims, Centre de compétence maladies auto-immunes et systémiques rares, Reims, France. 6. UPMC, AP-HP, Hôpital Pitié-Salpêtrière, Centre de référence maladies auto-immunes et systémiques rares, Paris, France. 7. AP-HM, Hôpital Timone Enfants, Unité de Réanimation Pédiatrique, Marseille, France. 8. UPMC, AP-HP, Hôpital Saint Antoine, Paris, France. 9. Rhumatologie, Centre de référence maladies auto-immunes et systémiques rares, Strasbourg, France. 10. Hôpital Louis Pradel, Lyon, France. 11. Unité Fonctionnelle de Cardiologie Pédiatrique et Congénitale, CHU Angers, France. 12. UPMC, Hôpital Tenon, Paris, France. 13. CHU Fort de France, Martinique, France. 14. Centre de référence maladies auto-immunes et systémiques rares, Lille, France. 15. AP-HP, Hôpital Hôtel-Dieu, Centre d'Epidémiologie Clinique, Paris, France; Université Paris Descartes-Sorbonne Paris Cité, Paris, France; Department of Epidemiology, Mailman School of Public Health, Columbia University, New York, USA; French Cochrane Centre, Paris, France. 16. AP-HP, Gynécologie, Hôpital Necker, Paris, France. 17. Cardiopédiatrie, CHU de Nantes, France. 18. AP-HP, Hôpital Cochin, Centre de référence maladies auto-immunes et systémiques rares, Paris, France. Electronic address: nathalie.costedoat@gmail.com.
Abstract
BACKGROUND: Dilated cardiomyopathy (DCM), a well-known complication of cardiac neonatal lupus, is associated with high mortality rate. Its risk factors remain unclear. METHODS: We analyzed occurrence of postnatal DCM among children with high-degree congenital heart block (CHB) and mothers with anti-SSA and/or anti-SSB antibodies. RESULTS: Among 187 neonates with CHB, 35 (18.8%, one missing data) had DCM and 22 (11.8%) died during a median follow-up of 7years [range: birth-36years]. On multivariate analysis, factors associated with postnatal DCM were in utero DCM (P=0.0199; HR=3.13 [95% CI: 1.20-8.16]), non-European origin (P=0.0052; HR=4.10 [95% CI: 1.81-9.28]) and pacemaker implantation (P=0.0013; HR=5.48 [95% CI: 1.94-15.47]). Postnatal DCM could be categorized in two subgroups: neonatal DCM (n=13, diagnosed at a median age of 0day [birth-4days]) and late-onset DCM (n=22, diagnosed at a median age of 15.2months [3.6months-22.8years]). Factors associated with neonatal DCM were in utero DCM, hydrops, endocardial fibroelastosis and pericardial effusion, whereas those associated with late-onset DCM were non-European origin, in utero mitral valve insufficiency, and pacemaker implantation. Fluorinated steroids showed no protective effect against late-onset DCM (P=0.27; HR=1.65 [95% CI: 0.63-4.25]). Probability of survival at 10years was 23.1% for newborns diagnosed neonatally with DCM, 53.9% for those who developed late-onset DCM, and 98.6% for those without DCM. CONCLUSION: Neonatal and late-onset DCM appear to be two different entities. None of the known risk factors associated with neonatal DCM predicted late-onset DCM. Long-term follow-up of cardiac function is warranted in all children with CHB.
BACKGROUND:Dilated cardiomyopathy (DCM), a well-known complication of cardiac neonatal lupus, is associated with high mortality rate. Its risk factors remain unclear. METHODS: We analyzed occurrence of postnatal DCM among children with high-degree congenital heart block (CHB) and mothers with anti-SSA and/or anti-SSB antibodies. RESULTS: Among 187 neonates with CHB, 35 (18.8%, one missing data) had DCM and 22 (11.8%) died during a median follow-up of 7years [range: birth-36years]. On multivariate analysis, factors associated with postnatal DCM were in utero DCM (P=0.0199; HR=3.13 [95% CI: 1.20-8.16]), non-European origin (P=0.0052; HR=4.10 [95% CI: 1.81-9.28]) and pacemaker implantation (P=0.0013; HR=5.48 [95% CI: 1.94-15.47]). Postnatal DCM could be categorized in two subgroups: neonatal DCM (n=13, diagnosed at a median age of 0day [birth-4days]) and late-onset DCM (n=22, diagnosed at a median age of 15.2months [3.6months-22.8years]). Factors associated with neonatal DCM were in utero DCM, hydrops, endocardial fibroelastosis and pericardial effusion, whereas those associated with late-onset DCM were non-European origin, in utero mitral valve insufficiency, and pacemaker implantation. Fluorinated steroids showed no protective effect against late-onset DCM (P=0.27; HR=1.65 [95% CI: 0.63-4.25]). Probability of survival at 10years was 23.1% for newborns diagnosed neonatally with DCM, 53.9% for those who developed late-onset DCM, and 98.6% for those without DCM. CONCLUSION: Neonatal and late-onset DCM appear to be two different entities. None of the known risk factors associated with neonatal DCM predicted late-onset DCM. Long-term follow-up of cardiac function is warranted in all children with CHB.