Objective: To examine health-related quality of life (HR-QoL) of adults with congenital heart disease (CHD).
Design: The HR-QoL of a large consecutive series of adults with CHD was prospectively studied using the Medical Outcome Study-Short Form 36.
Results: Except for very young men who report normal health, young adults describe poor health and exercise limitations relative to their peers in the community. In addition, middle-aged men report fatigue, psychological distress, impaired social functioning, and disability because of emotional difficulties. Among structurally defined subgroups, palliated and repaired cyanotic groups describe poor health. Patients with unoperated and palliated cyanotic lesions and men with repaired cyanotic defects or unoperated shunts also report exercise intolerance. Current cyanosis emerged as an important influence on HR-QoL. Patients with acquired cyanosis report poor health, exercise intolerance, fatigue, psychological difficulties, and social impairment. Chronic cyanosis was associated with only patient reports of poor health, exercise limitations, exercise-related disability, and social difficulties. Although adults with repaired cyanotic defects report poor health, no other HR-QoL deficits were reported by acyanotic patient groups. Pulmonary hypertension and rhythm disorders also influenced specific HR-QoL domains. Although gross structural and pathophysiologic factors influenced perceived health and exercise ratings, these variables were relatively poor predictors of self-reported health status and exercise capacity.
Conclusions: Many adults with CHD do not meet normative expectations for perceived health status and exercise tolerance. The principal determinants of self-reported health status and perceived exercise tolerance in adults with CHD are not known. Gender differences in outcome require further investigation.