Restrictive Lung Disease is an Independent Predictor of Exercise Intolerance in the Adult with Congenital Heart Disease

Abstract

Background/Objectives Following repair of congenital heart disease (CHD), adult patients are at risk for reduced exercise capacity. Restrictive lung disease (RLD) may contribute to reduced exercise capacity in this population. The aim of this study was to determine the prevalence of RLD and its impact on exercise tolerance in the adult with congenital heart disease. Methods One hundred consecutive adult patients with CHD, who underwent routine cardiopulmonary exercise testing with spirometry, were evaluated. Clinical data was obtained by retrospective chart review. Results Patients from 10 major diagnostic groups were identified. The median age for the cohort was 31 years (range 18–63) and included 43 males and 57 females. Most patients, 79%, had at least one previous surgical procedure. Based on spirometry and flow/volume loops, 50 patients were classified as normal pulmonary function, 44 patients had patterns suggestive of RLD, 4 suggestive of mixed (obstructive and restrictive), and 2 indeterminate. Risk factors associated with RLD include history of multiple thoracotomies (odds ratio=9.01, p=0.05) and history of atrial arrhythmias (odd ratio=4.25, p=0.05). Overall, 56% of the patients had abnormal exercise capacity. Spirometry suggestive of RLD was a significant risk factor for decreased exercise capacity (odds ratio=3.65, p=0.03). Patients with spirometry suggesting RLD also had lower exercise duration (p=0.004) and a higher New York Heart Association Functional Class (p=0.02). History of previous surgery and decreased heart rate reserve were also significant risk factors for decreased exercise capacity. Conclusion Abnormal spirometry suggestive of RLD is common in the adult with CHD and is a significant risk factor for decreased exercise tolerance in this population. Further studies, are needed to evaluate the relationship between RLD and exercise intolerance and its relationship to mortality in the adult with CHD.

Abnormal Spirometry in Congenital Heart Disease

“…The reported association between abnormal spirometry and mortality, in conjunction with previous reports relating abnormal FVC to lower aerobic capacity, 6 lends importance to the high prevalence of low FVC in this population. Although this is the first report to specifically highlight this connection among adults with congenital heart disease, numerous studies have reported an association between spirometric variables (eg, slow vital capacity, FEV 1 , and FVC) and overall and cardiovascular mortality both in patients with acquired heart disease and in the general population, independent of tobacco use or clinical lung disease.…”

“…6,7 Although the present report includes a large number of patients, available pulmonary function data are limited to simple spirometry without information on lung volumes or diffusing capacity, which limits mechanistic inference. Previous reports demonstrated that the pattern of pulmonary function abnormalities and underlying mechanisms may vary between defects and at different stages of disease and repair.…”

Diagnosis and Management of Noncardiac Complications in Adults With Congenital Heart Disease: A Scientific Statement From the American Heart Association

“…58 Forty-four percent of adults with CHD demonstrated a pattern on spirometry suggestive of restrictive lung disease, which is markedly higher than the 9% prevalence in the general adult population. 59 The prevalence of restrictive lung disease was highest in patients with a history of Fontan procedure (89%) and tetralogy of Fallot (TOF) repair (76%). 59 Forced vital capacity and forced expiratory volume in 1 second have been shown to be reduced in adults with CHD, a finding that is highly suggestive of abnormal pulmonary mechanics.…”

Extra-cardiac manifestations of adult congenital heart disease

“…Nearly half (44-47%) of ACHD patients have restrictive lung disease (RLD), including 89% following Fontan repair and 76% with TOF [31,32]. Predictors of RLD include multiple prior thoracotomies (possibly due to associated chest wall deformity or weakness), diaphragmatic nerve palsy, cardiomegaly, atrial arrhythmias (perhaps related to amiodarone use), scoliosis, complex CHD, and increased BMI [32,33].…”