Abnormal Lung Function in Adults with Congenital Heart Disease: Prevalence, Relation to Cardiac Anatomy and Association with Survival
Background—Restrictive lung defects are associated with higher mortality in patients with acquired chronic heart failure. We investigated the prevalence of abnormal lung function, its relation to severity of underlying cardiac defect, surgical history and its impact on outcome across the spectrum of adult congenital heart disease (ACHD).
Methods and Results—A total of 1,188 ACHD patients (age 33.1±13.1 year) undergoing lung function testing between 2000 and 2009 were included. Patients were classified according to the severity of lung dysfunction based on predicted values of forced vital capacity. Lung function was normal in 53% of ACHD patients, mildly impaired in 17% and moderately-severely impaired the remainder (30%). Moderate-severe impairment of lung function related to complexity of underlying cardiac defect, enlarged cardiothoracic ratio, previous thoracotomy/ies, body mass index, scoliosis and diaphragm palsy. Over a median follow-up period of 6.7 years, 106 patients died. Moderate-severe impairment of lung function was an independent predictor of survival in this cohort. Patients with reduced force vital capacity of at least moderate severity had a 1.6-fold increased risk of death compared to patients with normal lung function (p 0.04).
Conclusions—A reduced forced vital capacity is prevalent in ACHD patients; its severity relates to complexity of underlying heart defect, surgical history and scoliosis. Moderate-severe impairment of lung function is an independent predictor of mortality in contemporary ACHD patients.
- Received June 30, 2012.
- Accepted December 27, 2012.
- Copyright © 2013, Circulation