Mode of death in adults with congenital heart disease

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Abstract

An increasing number of patients with congenital heart disease (CHD) are entering adulthood. Although prior studies have focused on the causes of death in the pediatric population, the modes of death for adults with CHD have not been well defined. In a cross-sectional study performed on a population of 2,609 consecutive adults assessed at a CHD specialty clinic, there were adequate information available in 197 of 199 deceased patients. Mean age at death was 37 ± 15 years. Mortality was highest in patients with congenitally corrected transposition of the great arteries (26%), tricuspid atresia (25%), and univentricular connection (23%). Youngest mean age at death was observed in patients with tricuspid atresia (27 ± 5 years), complete transposition of the great arteries (27 ± 7 years), pulmonary atresia (27 ± 6 years), and aortic coarctation (29 ± 6 years). Sudden death (26%) was the most common cause of death followed by progressive heart failure (21%) and perioperative death (18%). Postmortem examinations were performed in 77 of 197 deceased patients (39%) and provided incremental data on the mechanism of death in 22% of autopsies. Thus, the 3 major causes of death in the growing population of adults with CHD are sudden, perioperative, and progressive heart failure.

Section snippets

Methods

In this cross-sectional study, consecutive patients referred for cardiac evaluation to the University of Toronto Congenital Cardiac Center for Adults (TCCCA), The Toronto Hospital, between 1981 and 1996 were identified from the center’s computerized database. A small proportion of the study population had been previously reported in natural history studies of specific lesion types.13, 14, 15

TCCCA serves as the adult continuity clinic in Toronto for former cardiac patients of the Hospital for

Results

The study group was comprised of 2,609 adults who fulfilled the inclusion criteria. One hundred ninety nine patients died (8%). Review of the records provided adequate details for 197 patients (99% of the deceased patients) to analyze their circumstances of death. Eighty-four of these 197 patients were women (43%) and 113 were men (57%). Their mean age at death was 37 ± 15 years (range 18 to 80). No surgery or palliative surgery was performed in 869 patients (33%), definitive surgery was

Discussion

Our study reports the largest series of adults with CHD (n = 2,609) assessed during a 15-year period at a single center. Of these, 199 patients (8%) died and adequate information was available in 99% of them. Sudden death, progressive heart failure, and perioperative death related to cardiac surgery were the most common causes of death in this study and accounted for 65% of all deaths. Autopsy contributed additional knowledge in 22% of deceased patients who underwent postmortem examination.

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    Dr. Oechslin was supported by the Faculty of Medicine, University of Zurich, and the Stiefel Zangger Foundation, Zurich, Switzerland. This study was supported in part by an operating grant from the Medical Research Council of Canada, Ottawa, Ontario, Canada. Manuscript received February 9, 2000; revised manuscript received and accepted May 25, 2000.

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