Incidence and Prognosis of Neonatal and Late-Onset Dilated Cardiomyopathy Associated with Cardiac Neonatal Lupus

Kateri Levesque^1,2, Nathalie Morel³, Alice Maltret⁴, Gabriel Baron⁵, Mohamed Hamidou⁶, Pauline Orquevaux⁷, Jean Charles Piette⁸, Francois Barriere⁹, Jérome Lebidois⁴, Laurent Fermont⁴, Olivier Fain¹⁰, Arnaud Theulin¹¹, Francois Sassolas¹², Philippe Pézard¹³, Gaëlle Guettrot-Imbert¹⁴, Delphine Le Mercier¹⁵, Sophie Georgin-Lavialle¹⁶, Christophe Deligny¹⁷, Eric Hachulla¹⁸, Luc Mouthon¹⁹, Philippe Ravaud²⁰, Elisabeth Villain⁴, Damien Bonnet²¹ and Nathalie Costedoat-Chalumeau³, ¹Internal Medicine, CHU Ste-Justine, Montréal, QC, Canada, ²Medicine Interne, Hopital Cochin, Paris, France, ³Internal Medicine Department, Cochin Hospital, “René-Descartes Paris V” University, Paris, France, ⁴Cardiology, Groupe Hospitalier Necker - Enfants Malades, Paris, France, ⁵Hôpital Hôtel Dieu, Paris, France, ⁶Internal Medicine Department, Nantes University Hospital, Nantes, France, ⁷Hu Robert Debre, CHU Reims, Reims, France, ⁸Internal Medicine Department, University Hospital “Pitié-Salpêtrière”, “Pierre et Marie Curie Paris VI” University, Paris, France, ⁹Pediatry, CHU Nantes, Nantes, France, ¹⁰Internal Medicine, Hospital J. Verdier, Bondy, France, ¹¹Internal medicine, CHRU Strasbourg, Strasbourg, France, ¹²Cardiology, CHU Lyon, Lyon, France, ¹³Pediatric cardiologist, CHU Angers, Angers, France, ¹⁴Internal Medicine, Hopital Saint-Antoine, Paris, France, ¹⁵Obstetrics and gynecology, Hopital Necker, Paris, France, ¹⁶Internal medicine, Hopital Tenon, Paris, France, ¹⁷Rhumatologie Et Médecine Interne, Centre hospitalier Universitaire de Fort de France, Fort de France, Martinique, ¹⁸Service de Médecine Interne, Centre National de Référence des Maladies Systémiques Rares, Hôpital Claude Huriez, CHRU Lille, Lille, France, ¹⁹Internal Medicine, National Referral Center for Rare Systemic Autoimmune Diseases, Hôpital Cochin, Paris, France, ²⁰Rheumatology, Paris, France, ²¹Pediatric cardiologist, Hopital Necker, Paris, France

Meeting: 2015 ACR/ARHP Annual Meeting

Date of first publication: September 29, 2015

Keywords: Neonatal lupus and pregnancy

Session Information

Date: Monday, November 9, 2015

Title: Reproductive Issues in Rheumatic Disorders: Basic and Clinical Aspects

Session Type: ACR Concurrent Abstract Session

Session Time: 4:30PM-6:00PM

Background/Purpose:

Dilated cardiomyopathy (DCM) is a well-known complication of cardiac neonatal lupus syndrome and is associated with a high mortality rate. Its risk factors are unclear.

Methods: We analyzed the occurrence of postnatal DCM (neonatal or of late-onset) among children with a high degree congenital heart block (CHB) included in the French registry of cardiac neonatal lupus (from 1976 to 2014). The presence of postnatal DCM was defined by left ventricular systolic dysfunction with left ventricle ejection fraction <45% requiring treatment or by death related to end-stage heart failure. All mothers were anti-SSA antibodies positive.

Results:

187 children with CHB were born alive. During a median follow-up of 7 years [birth to 36 years], 35 (18.8%, one missing data) had a DCM, and 22 (11.8%) died.

DCM could be categorized in 2 different subgroups: neonatal DCM (n=13, diagnosed before day 28 of life) or late-onset DCM (n=22, diagnosed at a median age of 15.2 months [3.6 months-22.8 years]). On univariate analysis, factors associated with neonatal DCM were maternal treatment with hydroxychloroquine, in utero cardiomegaly and DCM, and hydrops. By contrast, factors associated with late-onset DCM were non-European origin and significant in utero valvulopathy. In multivariate analysis, the only factor associated with neonatal DCM was in utero DCM (p=0.0001; HR 15.99 [95%CI: 3.93-65.01]), whereas the only factor associated with late-onset DCM was non-white race origin (p=0.0147; HR 3.65 [95%CI: 1.28-10.0]).

Twenty-two (11.8%) children died: 8 in the neonatal period and 14 after the first month of life and one child with DCM had a successful heart transplantation at 2.5 years of age. Neonatal deaths were always related to DCM and occurred during the first days of life despite intensive care and the placement of pacemaker in 2 out of 8 cases. The 14 later deaths occurred at a median time of 10 days after the diagnosis of DCM [0-58months]. Eleven (79%) of those deaths were the result of DCM.

The probability of survival at 10 years of age for a neonate with CHB was 87.1%: 23.1% in the presence of neonatal DCM, 53.9% for those who developed a late-onset DCM requiring treatment versus 98.6% in those without DCM. Prenatal fluorinated steroid (p=0.27; HR 1.65 [95%CI: 0.63-4.25]) did not prevent the development of late-onset DCM.

Conclusion:

The presence of in utero DCM carries a high risk of neonatal DCM. No classical risk factors were associated with late-onset DCM. The mortality associated with DCM (either neonatal or of late-onset) was high. Maternal treatment with fluorinated steroid did not prevent the development of late-onset DCM.

Disclosure: K. Levesque, None; N. Morel, None; A. Maltret, None; G. Baron, None; M. Hamidou, None; P. Orquevaux, None; J. C. Piette, None; F. Barriere, None; J. Lebidois, None; L. Fermont, None; O. Fain, None; A. Theulin, None; F. Sassolas, None; P. Pézard, None; G. Guettrot-Imbert, None; D. Le Mercier, None; S. Georgin-Lavialle, None; C. Deligny, None; E. Hachulla, None; L. Mouthon, None; P. Ravaud, None; E. Villain, None; D. Bonnet, None; N. Costedoat-Chalumeau, None.

To cite this abstract in AMA style:

Levesque K, Morel N, Maltret A, Baron G, Hamidou M, Orquevaux P, Piette JC, Barriere F, Lebidois J, Fermont L, Fain O, Theulin A, Sassolas F, Pézard P, Guettrot-Imbert G, Le Mercier D, Georgin-Lavialle S, Deligny C, Hachulla E, Mouthon L, Ravaud P, Villain E, Bonnet D, Costedoat-Chalumeau N. Incidence and Prognosis of Neonatal and Late-Onset Dilated Cardiomyopathy Associated with Cardiac Neonatal Lupus [abstract]. Arthritis Rheumatol. 2015; 67 (suppl 10). https://acrabstracts.org/abstract/incidence-and-prognosis-of-neonatal-and-late-onset-dilated-cardiomyopathy-associated-with-cardiac-neonatal-lupus/. Accessed .

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