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CLINICAL RESEARCH: CONGENITAL HEART DISEASE

Presentation and Prognosis of Complete Atrioventricular Block in Childhood, According to Maternal Antibody Status

Elisabeth Villain, MD^_*,_*, Nathalie Coastedoat-Chalumeau, MD, Eloi Marijon, MD^_*, Younes Boudjemline, MD^_*, Jean-Charles Piette, MD and Damien Bonnet, MD, PhD^_*

^_* Cardiologie Pédiatrique, Université Paris V. René Descartes, Hôpital Necker-Enfants Malades, Paris, France
Service de Médecine Interne, Centre Hospitalier Universitaire Pitié Salpêtrière, Paris, France.

Manuscript received February 27, 2006; revised manuscript received June 26, 2006, accepted July 3, 2006.

^_* Reprint requests and correspondence: Dr. Elisabeth Villain, Cardiologie pédiatrique, Hôpital Necker-Enfants Malades, 149, rue de Sèvres, 75015 Paris, France. (Email: elisabeth.villain{at}nck.aphp.fr).

OBJECTIVES: We sought to determine whether the presentation and prognosis of children with complete atrioventricular block (CAVB) were related to maternal antibody status.

BACKGROUND: Comparative studies related to the presence or absence of maternal antibodies anti-SSB/La and anti-SSA/Ro are lacking in children with isolated complete CAVB.

METHODS: From 1980 to 2004, we screened for maternal antibodies in 111 children <15 years old with CAVB. According to the presence (Ab+) or absence (Ab–) of antibodies, 2 groups of patients were retrospectively compared.

RESULTS: The study group included 56 Ab+ and 55 Ab– patients with equal gender distribution. A total of 96% Ab+ patients were diagnosed in utero or within the first month, compared with 24% Ab– patients. Progression from incomplete to complete block was shown in 23 Ab– and 2 Ab+ patients. Echocardiography showed normal heart structures in Ab– patients, but 8 Ab+ patients had ostium secundum or ductus arteriosus. Pacemaker implantation was performed in 105 patients, and age at implantation was younger in the Ab+ group. At follow-up (age 9.7 ± 6 years), all Ab– patients were alive with normal left ventricular function; dilated cardiomyopathy was diagnosed at diagnosis or during follow-up in 16 Ab+ patients, and 6 of 16 have died.

CONCLUSIONS: Patients with antibody-mediated CAVB were diagnosed and underwent pacing earlier in life and had a more severe prognosis than Ab– patients because of a high risk of dilated cardiomyopathy. The absence of antibody suggests a different pathologic mechanism than autoimmunity, and the term congenital may be not appropriate in these cases.

Abbreviations and Acronyms   CAVB = complete atrioventricular block   DCM = dilated cardiomyopathy   ECG = electrocardiogram   PM = pacemaker

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