Congenital heart disease
Incidence and Predictors of Sudden Cardiac Arrest in Adults With Congenital Heart Defects Repaired Before Adult Life

https://doi.org/10.1016/j.amjcard.2012.02.057Get rights and content

Many adult survivors of repaired congenital heart disease (CHD) are at premature risk of death. Sudden cardiac arrest (SCA) is 1 of the leading causes of death but little is known about determinants for SCA in adults with repaired lesions. We sought to determine incidence and risk factors for SCA in a study population of 936 adults with previously repaired CHD who had completed follow-up at a single tertiary center during a mean period of 9 ± 7 years. Mean age at first examination in our institution was 21 ± 7 years. Diagnostic categories included tetralogy of Fallot (216), coarctation of the aorta (157), transposition complexes (99), single ventricle (55), and other CHD (409). During a total follow-up of 8,387 person-years, 22 patients (2.6 per 1,000 person-years) presented with SCA. Incidence of SCA varied widely between specific lesions; the highest incidence was observed in transposition complexes (10 per 1,000 person-years). Independent predictors of SCA were retrospectively identified using multivariate Cox proportional hazard modeling. Age at initial examination and severely impaired subaortic ventricular systolic function were independent risk factors for SCA (severe subaortic ventricular systolic dysfunction, adjusted hazard ratio 29, 95% confidence interval 11 to 72, p <0.001). SCA occurred in 23% of patients with severe subaortic ventricular systolic dysfunction versus 0.7% of patients with nonsevere decreased subaortic ventricular function (p <0.001). In conclusion, severe subaortic ventricular systolic dysfunction is a dominant multivariate predictor of SCA in an unselected population of adult survivors after surgery for CHD. Our data support the consideration of primary prevention strategies in these patients.

Section snippets

Methods

Clinical records of 3,197 patients referred to the adult CHD unit at La Paz University Hospital for cardiac evaluation from January 1990 through January 2010 were retrospectively evaluated. For the present study, 1,289 patients (40%) who had a reparative or palliative intervention for CHD at <20 years of age were identified. The study group included all patients who were followed serially at our institution with a clinical visit within the previous 2 years or before an end point. A

Results

In total 936 patients (73%) were eligible for inclusion and comprised the study population. Distribution of diagnostic categories and duration of follow-up for the entire sample and separately by CHD category are presented in Table 1. Although the mean follow-up period ranged from 5 ± 5 years in patients with atrial septal defects to 11 ± 8 years in patients with repaired aortic stenosis, differences in follow-up were not statistically significant between diagnostic categories. Further details

Discussion

To our knowledge, this is the first longitudinal cohort study to analyze predictors of SCA in a large population of adult survivors after operation for CHD at <20 years of age. Results indicated that severe systemic ventricular dysfunction has an outstanding role as a dominant multivariate predictor of SCA for all combined diagnoses in this heterogenous population.

Until recently, limited data existed on risk factors for SCA during long-term follow-up after surgery in the total adult CHD

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