Findings among individuals with an isolated aortopulmonary window vary based on the size of the defect and the pulmonary vascular resistance. Cardiac examination typically indicates a parasternal lift resulting from right ventricular overload, a loud single second heart sound induced by pulmonary hypertension, and increased peripheral pulses.[2]
When the defect is larger, pulmonary vascular resistance may continue to be elevated in the initial weeks or months of life, and there is a modest amount of pulmonary overcirculation. A rather faint basal systolic ejection murmur without a diastolic element and a loud single second heart sound develop due to mild overcirculation. As the pulmonary vascular resistance decreases throughout the first few months, the left-to-right shunting of blood into the lungs increases, and the systolic murmur becomes more intense and longer, eventually extending into diastole and becoming a continuous murmur.[2]
Corrective heart surgery, which is normally performed in the first year of life, is the definitive intervention for an aortopulmonary window. If the patient's symptoms don't allow for corrective surgery, medical therapy of congestive heart failure is the second choice. Permanent alterations in the pulmonary vasculature can be prevented with early intervention.[2]
