Unrestrictive Aortopulmonary Window

A 30-year-old woman was referred for management of an aortopulmonary window. Her history was notable for chronic atrial fibrillation treated by amiodarone and Coumadin anticoagulation. Peripheral oxygen saturation was 95%. A chest x-ray film showed cardiomegaly, bilateral prominent hilar vascularization, and interstitial edema (Figure 1). The echocardiogram showed a large, 15-mm aortopulmonary (AP) window, ≈25 mm above the aortic valve annulus between the ascending aorta and main pulmonary artery (type I) with an unrestrictive, exclusively left-right shunt and a peak gradient of 30 mm Hg (Figure 2). There was no aortic coarctation or interruption of the aortic arch, and the main pulmonary artery (3.3 cm) was dilated to a size equal to the aortic root, with dilated branch pulmonary arteries (2.3 cm each). The left atrium was severely dilated (45 cm2), as was the left ventricle (end-diastolic diameter, 7.5 cm; indexed, 5.2 cm/m2). There was moderate mitral regurgitation (regurgitation volume, 26.6 mL; proximal isovelocity surface area convergence radius, 6.8 mm) attributable to annular dilatation. The biventricular systolic function was normal. The right ventricular systolic pressure was estimated at 63.3 mm Hg by the gradient of the tricuspid regurgitation jet. Cardiac MRI confirmed these findings (Figure 3 …