Fenestrated ASD closure in a child with idiopathic pulmonary hypertension and exercise desaturation

Pulmonary arterial hypertension is a progressive disorder that may result in right heart failure and death. Atrial level shunts in the presence of pulmonary hypertension may allow right-to-left mixing with maintenance of cardiac output and improved survival. However, excessive mixing at the atrial level can cause undue systemic desaturation, increased fatigue and decreased exercise tolerance even in the presence of adequate cardiac output. A 5½-year-old was diagnosed with pulmonary hypertension, a large atrial septal defect and right-to-left shunting. Medical therapy over an 18-month period was successful in decreasing pulmonary artery pressure and pulmonary vascular resistance. However, because of the size and position of the intracardiac defect, symptoms of fatigue, and severe systemic desaturation with only minor activities persisted. Fenestrated surgical closure of the defect was thus undertaken to decrease the degree of atrial mixing, but still allow atrial decompression if necessary. Subsequent hemodynamic evaluation has demonstrated continued improvement, and all previous symptoms have resolved. Repeated echocardiography has confirmed patency of the atrial fenestration with left-to-right atrial flow.