CLINICAL STUDY
Outcome of children with fetal, neonatal or childhood diagnosis of isolated congenital atrioventricular block
A single institution’s experience of 30 years
Edgar T. Jaeggi, MD* ,
Robert M. Hamilton, MD*,
Earl D. Silverman, MD ,
Samuel A. Zamora, MD and
Lisa K. Hornberger, MD*,*
* Division of Cardiology, The Hospital for Sick Children, University of Toronto, Canada and
Division of Rheumatology, The Hospital for Sick Children, University of Toronto, Canada
Division of Pediatric Cardiology, University Children’s Hospital, Geneva, Switzerland
Manuscript received December 12, 2000;
revised manuscript received August 30, 2001,
accepted September 7, 2001.
* Reprint requests and correspondence: Dr. Lisa K. Hornberger, Division of Cardiology, The Hospital for Sick Children, 555 University Avenue, Toronto, Ontario M5G 1X8, Canada.
hornberg{at}sickkids.on.ca
OBJECTIVES: We reviewed our institution’s experience with isolated (congenital) third-degree atrioventricular block (CAVB) to identify pre- and post-natal predictors of mortality and the requirement for pacemakers in infancy and childhood.
BACKGROUND: Because of the relative rarity of the disease, there is a paucity of data concerning the outcome of fetuses and children with isolated CAVB.
METHODS: The medical records of all cases of CAVB encountered at our institution from January 1965 to December 1998 were analyzed.
RESULTS: Of 102 cases identified, 29 were diagnosed in utero (F) at 26.1 ± 5.6 weeks gestation, 33 as neonates (N;  28 days), and 40 as children (C) at 5.7 ± 4.8 years of age. Anti-Ro and/or anti-La were present in 95% of F and 90% of N, but only in 5% of C mothers tested (p < 0.0001). Patients with CAVB having F, N and C diagnosis had a mortality of 43%, 6% and 0%, respectively, in the first two decades of life. Increased mortality risk was associated with a fetal diagnosis of CAVB (13/15 deaths; p < 0.05), fetal hydrops (6/6 cases; p < 0.0001), endocardial fibroelastosis (5/5 cases; p < 0.0001) and delivery at 32 weeks (4/6 cases; p < 0.05). Timing of pacemaker implantation differed significantly among F versus N (p < 0.05) and N versus C (p < 0.001) cases. At 20 years of age only 11% and 12% of CAVB patients with N and C diagnosis, respectively, were not paced.
CONCLUSIONS: Pre-natal diagnosis of CAVB is associated with high fetal and neonatal mortality. Among survivors, whether the diagnosis is made before or after birth, most undergo pacemaker implantation by adulthood, with earlier intervention and a significantly greater need for reintervention among those diagnosed in utero.
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Abbreviations and Acronyms
| | ACC/AHA | | American College of Cardiology/American Heart Association | | AV | | atrioventricular | | CAVB | | isolated congenital atrioventricular block | | CI | | confidence interval | | CR | | cumulative risk | | EFE | | endocardial fibroelastosis | | HF | | heart failure |
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