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

Study Questions:

What is the prevalence of abnormal lung function, and what is its relation to severity of underlying cardiac defect, surgical history, and outcome in adults with congenital heart disease (ACHD)?


A retrospective review was performed at a single tertiary center. Patients underwent routine pulmonary functioning testing as part of their ACHD care. The primary measure of pulmonary function was forced vital capacity as compared with predicted values.


Lung function was normal in 53% of ACHD patients, mildly impaired in 17%, and moderately-to-severely impaired in 30%. Correlates of moderate-to-severe lung impairment included the complexity of underlying cardiac defect, enlarged cardiothoracic ratio, previous thoracotomy, lower body mass index, scoliosis, and diaphragm palsy. Over the median follow-up period of 6.7 years, patients with at least moderate lung impairment had a 1.6-fold increased risk of death as compared to patients with normal lung function.


Reduced forced vital capacity is common in ACHD patients; is associated with the complexity of the underlying cardiac defect, scoliosis, and surgical history; and is an independent predictor of mortality.


The expanding population of ACHD has allowed for a better understanding of comorbidities present in this patient population. Patients with CHD commonly have had thoracotomies in childhood and may also have associated scoliosis, placing them at risk for restrictive lung disease. Pre-existing lung disease has previously been reported to be associated with increased intensive care unit mortality in adults undergoing surgery from congenital heart defects. The current study demonstrates a relatively high prevalence of at least moderately decreased lung function in ACHD patients, which was associated with higher mortality in follow-up. Lung disease should be considered in ACHD patients, particularly to optimize management and identify patients at risk of postoperative complications at times of repeat cardiac surgery.

Clinical Topics: Cardiac Surgery, Invasive Cardiovascular Angiography and Intervention

Keywords: Heart Diseases, Prevalence, Thoracotomy, Follow-Up Studies, Body Mass Index, Scoliosis, Respiratory Insufficiency, Cardiac Surgical Procedures, Respiratory Paralysis, Diaphragm, Respiratory Function Tests, Lung Diseases

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