Abnormal Lung Function in Adults With Congenital Heart Disease: Prevalence, Relation to Cardiac Anatomy, and Association With Survival

Abstract

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, its surgical history, and its impact on outcome across the spectrum of adult congenital heart disease. Methods and Results— A total of 1188 patients with adult congenital heart disease (age, 33.1±13.1 years) 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 patients with adult congenital heart disease, mildly impaired in 17%, and moderately to severely impaired in the remainder (30%). Moderate to 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 to 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 with patients with normal lung function ( P =0.04). Conclusions— A reduced forced vital capacity is prevalent in patients with adult congenital heart disease; its severity relates to the complexity of the underlying heart defect, surgical history, and scoliosis. Moderate to severe impairment of lung function is an independent predictor of mortality in contemporary patients with adult congenital heart disease.

Lung Function, Inflammation, and Endothelin‐1 in Congenital Heart Disease–Associated Pulmonary Arterial Hypertension

“…The findings of the present study, however, are in conflict with a study that demonstrated that Eisenmenger patients have near normal lung volumes, but that study had no controls—either healthy or CHD patients without PAH 42. In addition, unselected CHD patients have been reported as having reduced FVC, which may be related to features such as scoliosis (which was excluded from the present study) and previous cardiothoracic surgery 14, 43. The present study demonstrates that lung restriction occurs in CHD‐APAH compared with CHD and healthy controls without scoliosis.…”

“…The present study demonstrates that lung restriction occurs in CHD‐APAH compared with CHD and healthy controls without scoliosis. While thoracotomy was associated with lung restriction, sternotomy was not, which is in keeping with previous studies 14. However, patients who had not had previous cardiothoracic surgery also had lower lung volumes than predicted values, suggesting that the presence of APAH may have a separate effect on lung restriction in keeping with lung restriction that has been described in IPAH patients 8, 9, 13, 44, 45…”

“…Some of these abnormalities correlate with exertion dyspnea and reduction in exercise tolerance 10, 11. While some of these abnormalities have also been described in CHD‐APAH patients,9, 12, 13 a lack of adequate controls makes the significance unclear as abnormal lung function has also been described in CHD patients without APAH 14. These might relate to coexisting scoliosis or previous cardiothoracic surgery that is frequent in this group and may be found in CHD patients both with and without APAH.…”

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]. CCHD and increased CHD complexity are further associated with worsened lung function [33].…”

“…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]. CCHD and increased CHD complexity are further associated with worsened lung function [33]. RLD can also be seen in CHD associated with lung underdevelopment (as in scimitar syndrome-partial anomalous pulmonary venous return with hypoplastic right lung) or related to impaired pulmonary blood flow due to congenital pulmonic stenosis or TOF [34].…”