Atrial septal defect (ASD) is a major cause of left-toright intracardiac shunting. If persisting into adulthood, an ASD can lead to a larger shunt, which may eventually cause pulmonary hypertension and right ventricular failure. Large intracardiac shunts cannot be tolerated in patients with underlying lung disease such as cystic fibrosis. Although the association between an intracardiac shunt and cystic fibrosis has been reported in the literature, the impact of ASD closure on the clinical course of patients with cystic fibrosis has not been studied. Here, we report a case of ASD closure in a patient with cystic fibrosis with hypoxemia out of proportion to his lung disease. Closure of the ASD shunt resulted in significant improvement of his symptoms and pulmonary function testing.Copyright © 2017 Science International Corp.
Key WordsAtrial septal defect • Septal closure device • Cystic fibrosis when significant, can cause dyspnea on exertion, which may not be well tolerated in patients with an underlying pulmonary condition such as cystic fibrosis. It is unknown whether ASD closure could lead to symptomatic improvement in these patients. Here, we report a case of ASD closure resulting in improved pulmonary function testing in a patient with cystic fibrosis with a bidirectional shunt and worsening functional capacity.
Case PresentationOur patient was a 36-year-old man with cystic fibrosis diagnosed at birth with a dF508/dF508 genotype. His symptoms could be characterized as moderate airway disease complicated by chronic airway infections, pancreatic insufficiency, diabetes, and malnutrition. Progression of his clinical condition caused dyspnea with minimal exertion. He was referred to our cardiology service to be evaluated for hypoxemia out of proportion to his chronic pulmonary disease. Pulmonary function testing showed a forced expiratory volume in 1 s (FEV1) of 1.71 (43% predicted). Transesophageal echocardiography (TEE) showed a small ASD with a bidirectional shunt seen at rest (Figure 1). Right heart catheterization showed a pulmonary artery pressure of 26/7/16 mmHg and pulmonary vascular resistance of 1.7 Wood units. A decision was made to proceed with ASD closure, which