Congenital aortopulmonary window

Congenital aortopulmonary window (also known as aortopulmonary septal defect, aortopulmonary fenestration, or aorticopulmonary window) is a rare congenital heart defect characterized by an abnormal communication (opening) between the ascending aorta and the pulmonary artery in the presence of two separate semilunar valves. This distinguishes it from truncus arteriosus, where there is a single outflow valve. The defect allows oxygenated blood from the aorta to shunt into the pulmonary artery (left-to-right shunt), leading to excessive blood flow to the lungs and volume overload of the left heart chambers. Clinical presentation typically occurs in the neonatal or early infantile period. Affected infants may develop signs of congestive heart failure, including tachypnea (rapid breathing), poor feeding, failure to thrive, recurrent respiratory infections, and a continuous or systolic heart murmur. If left untreated, the persistent high-pressure left-to-right shunt can lead to irreversible pulmonary vascular disease (Eisenmenger syndrome), which carries a very poor prognosis. Associated cardiac anomalies are found in approximately 50% of cases and may include interrupted aortic arch, tetralogy of Fallot, ventricular septal defect, patent ductus arteriosus, and anomalous origin of a coronary artery. Diagnosis is established through echocardiography, often supplemented by cardiac catheterization or CT/MRI angiography. The standard treatment is early surgical repair, ideally performed in infancy before pulmonary vascular disease develops. Surgical closure—either by direct patch closure or division of the communication—has excellent outcomes when performed early, with low operative mortality and good long-term survival. Without surgical intervention, mortality in infancy and early childhood is high. Long-term follow-up after repair is recommended to monitor for residual defects, pulmonary artery stenosis, or aortic regurgitation.

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