Long-Term Outcome of Simple Congenital Heart Disease

Study Questions:

What is the long-term prognosis for otherwise healthy patients with simple congenital heart disease (CHD)?


A nationwide follow-up study was performed of patients with simple CHD diagnosed between 1963 and 1973. Simple CHD was defined as isolated uncomplicated secundum atrial septal defect, patent ductus arteriosus, ventricular septal defect with normal pulmonary vascular resistance, or mild pulmonary valve stenosis verified by catheterization. The study population was followed through Danish public registries from the age of 15 years until January 2013 with respect to mortality, cause of death, morbidity, and medical follow-up.


The median age at the end of follow-up was 47.4 years (interquartile range, 43.9-50.9). The most common cause of death (40%) was sudden unexpected death. Mortality was increased compared with the general population, both overall (adjusted hazard ratio [aHR], 1.9; 95% confidence interval [CI], 1.5-2.4) and for patients (79%) without medical follow-up (aHR, 1.7; 95% CI, 1.3-2.2). The incidence of critical cardiac comorbidity was 3.9 per 1,000 patient-years. The most frequent events were adult (re)operation, and hospitalization for heart failure or ventricular tachyarrhythmia, and corresponded to an aHR of 5.7 (95% CI, 4.6-6.9) when compared with the general population.


Patients diagnosed with simple CHD in the 1960s have significantly increased long-term mortality and cardiac morbidity when compared with the general population.


Minimal follow-up is often recommended for patients with simple CHD. This study suggests significantly increased morbidity and mortality for adults with relatively simple CHD as compared with the general population. Although it is unknown as to whether closer follow-up could mitigate these risks, the study suggests that we should rethink the approach of discharge from care for patients with simple CHD. As the patients in this study underwent surgery in a different surgical era, the results may not be generalizable to current pediatric patients given differences in imaging capability as well as timing of intervention for some of these ‘simple’ defects.

Clinical Topics: Arrhythmias and Clinical EP, Cardiac Surgery, Congenital Heart Disease and Pediatric Cardiology, Heart Failure and Cardiomyopathies, Valvular Heart Disease, SCD/Ventricular Arrhythmias, Atrial Fibrillation/Supraventricular Arrhythmias, Cardiac Surgery and Arrhythmias, Cardiac Surgery and CHD and Pediatrics, Cardiac Surgery and Heart Failure, Cardiac Surgery and VHD, Congenital Heart Disease, CHD and Pediatrics and Arrhythmias, Acute Heart Failure

Keywords: Catheterization, Death, Sudden, Ductus Arteriosus, Patent, Heart Defects, Congenital, Heart Failure, Heart Septal Defects, Atrial, Heart Septal Defects, Ventricular, Morbidity, Pulmonary Valve Stenosis, Tachycardia, Vascular Resistance

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