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(Circulation. 2004;110:1542-1548.)
© 2004 American Heart Association, Inc.

Congenital Heart Disease

Transplacental Fetal Treatment Improves the Outcome of Prenatally Diagnosed Complete Atrioventricular Block Without Structural Heart Disease

Edgar T. Jaeggi, MD; Jean-Claude Fouron, MD; Earl D. Silverman, MD, FRCPC; Greg Ryan, MB; Jeffrey Smallhorn, MBBS; Lisa K. Hornberger, MD

From the Divisions of Cardiology (E.T.J., J.S., L.K.H.) and Rheumatology (E.D.S.), Department of Pediatrics, The Hospital for Sick Children, and the Fetal Medicine Unit (G.R.), Mount Sinai Hospital, University of Toronto, Toronto; and the Division of Cardiology, Department of Pediatrics, Sainte-Justine Hospital, University of Montreal, Montreal (J.C.F.), Canada. Dr Hornberger is now at the University of California, San Francisco Children’s Hospital, Department of Pediatrics, Division of Cardiology, The Fetal Cardiac Program, San Francisco, Calif.

Correspondence to Edgar T. Jaeggi, MD, Director, Fetal Cardiology, The Hospital for Sick Children, 555 University Ave, Toronto, Ontario M5G 1X8, Canada. E-mail edgar.jaeggi{at}sickkids.ca

Received November 25, 2003; de novo received April 10, 2004; revision received May 17, 2004; accepted May 18, 2004.

Background— Untreated isolated fetal complete atrioventricular block (CAVB) has a significant mortality rate. A standardized treatment approach, including maternal dexamethasone at CAVB diagnosis and ß-stimulation for fetal heart rates <55 bpm, has been used at our institutions since 1997. The study presents the impact of this approach.

Methods and Results— Thirty-seven consecutive cases of fetal CAVB since 1990 were studied. Mean age at diagnosis was 25.6±5.2 gestational weeks. In 33 patients (92%), CAVB was associated with maternal anti-Ro/La autoantibodies. Patients were separated into those diagnosed between 1990 and 1996 (group 1; n=16) and those diagnosed between 1997 and 2003 (group 2; n=21). The 2 study groups were comparable in the clinical presentation at CAVB diagnosis but did differ in prenatal management (treated patients: group 1, 4/16; group 2, 18/21; P<0.0001). Overall, 22 fetuses were treated, 21 with dexamethasone and 9 with ß-stimulation for a mean of 7.5±4.5 weeks. Live-birth and 1-year survival rates of group 1 were 80% and 47%, and these improved to 95% for group 2 patients (P<0.01). The 21 patients treated with dexamethasone had a 1-year survival rate of 90%, compared with 46% without glucocorticoid therapy (P<0.02). Immune-mediated conditions (myocarditis, hepatitis, cardiomyopathy) resulting in postnatal death or heart transplantation were significantly more common in untreated anti-Ro/La antibody–associated pregnancies compared with patients treated with steroids (0/18 versus 4/9 live births; P=0.007).

Conclusions— A standardized treatment approach, including transplacental fetal administration of dexamethasone and ß-stimulation at heart rates <55 bpm, reduced the morbidity and improved the outcome of isolated fetal CAVB.

Key Words: heart block • fetus • cardiotonic agents • steroids • pregnancy

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