Mortality in Adults With Congenital Heart Disease

Study Questions:

What risk factors are related to excess mortality in adults with congenital heart disease (CHD)?


A prospective survival analysis was performed at a single referral center in Spain. Survival status of each patient was verified using the Spanish National Death Index. Median age at death was estimated using a left-truncated Kaplan-Meier method. Independent risk factors for excess mortality were identified using a left-truncated Cox regression model.


A total of 3,311 adults with CHD, 50.5% males, with median age of entry of 22.5 years (interquartile range, 18-39 years), were studied over a median follow-up period of 10.5 years. During follow-up totaling 37,608 person-years, 336 (10%) patients died. The annual death rate was 0.89% and the standardized mortality ratio (SMR) was 2.64 (95% confidence interval [CI], 2.3-3.0; p < 0.001). Median age at death for the study population was 75.1 years. Survival was reduced as compared with the general population, whatever their level of complexity, repair status, or underlying CHD. Independent risk factors for excess mortality included cyanosis, single ventricle physiology, genetic disorders, ventricular dysfunction, residual hemodynamic lesions, and acquired late complications. For patients with at least one risk factor (n = 996), SMR was 6.2 (95% CI, 4.5-6.0; p < 0.001) and median age at death was 55.6 years (95% CI, 50-61). For patients with no risk factors (n = 2,315), SMR was 1.14 (95% CI, 0.9-1.5; p = 0.19) and the median age of death was 83.7 years (95% CI, 82-87).


The authors concluded that clinical parameters independently predict excess mortality in adults with CHD. Survival of patients with no risk factors did not differ from the reference population.


Several recent studies have examined the long-term survival of adults with CHD. Studies have differed as to the impact of mild disease such as atrial septal defect on long-term survival. This study identified multiple relatively generic risk factors, which predicted excess mortality. Patients with no risk factors had normal survival patterns as compared with the general population. It is interesting to note the estimated median age at death for various diagnostic categories and individual diagnoses. Defects of mild, moderate, and severe complexity had median age of death of 78, 72.6, and 49.2 years, respectively. Median age of death for tetralogy of Fallot was 63.2 years, coarctation of the aorta was 69.9 years (much higher than that historically reported in the era of late repairs), and single ventricle physiology was 48.1 years. Overall, this study is an interesting addition to existing data requiring long-term outcomes and risk factors in adults with CHD.

Clinical Topics: Arrhythmias and Clinical EP, Congenital Heart Disease and Pediatric Cardiology, Genetic Arrhythmic Conditions, Congenital Heart Disease, CHD and Pediatrics and Arrhythmias, CHD and Pediatrics and Quality Improvement

Keywords: Aortic Coarctation, Cyanosis, Genetics, Heart Defects, Congenital, Heart Septal Defects, Atrial, Hemodynamics, Mortality, Risk Factors, Survival Analysis, Tetralogy of Fallot, Ventricular Dysfunction

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