Aortopulmonary window: a rare presentation with left ventricular outflow tract obstruction

Excerpt

A 17-year-old male without any previous medical history, presented with progressive dyspnea from New York Heart Association (NYHA) II to NYHA IV over 1 year. At presentation, he had central cyanosis (saturation of 68%), pan digital clubbing (Fig. 1a), respiratory rate of 30/minute, and blood pressure of 68/40 mmHg. On auscultation, ejection systolic murmur of grade IV/VI was heard in neoaortic area. Echocardiography revealed large aortopulmonary window (APW) of size 27 mm with bidirectional shunt, discrete subaortic membrane with moderate aortic regurgitation, left atrium clot, and poor left ventricular ejection fraction 15% (Fig. 1b–d, Movie 1–3). Pulmonary artery pressure could not be determined as there was no tricuspid regurgitation, and catheterization study was not done. Computed tomography confirmed a large communication between aorta and pulmonary artery with massive pulmonary artery enlargement (Fig. 1e). Volumetric rendering imaging also delineated large APW (Fig. 1f). Patient was managed in intensive care unit; however, he died after 1 day of admission due to persistent hypoxia and shock.

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