pediatrics
Aortopulmonary Window
Symptoms and Signs of Aortopulmonary Window
A Left-to-right shunts may lead to In patients with an isolated aortopulmonary window, examination findings depend on the size of the defect and the pulmonary vascular resistance (PVR). Generally the cardiac examination reveals a parasternal lift from right ventricular overload, a loud single second heart sound (S2) resulting from pulmonary hypertension, and increased peripheral pulses. With a large defect, the PVR may remain elevated in the first weeks or months of life, and the amount of pulmonary overcirculation is modest. The modest overcirculation results in a relatively soft basal systolic ejection murmur without a diastolic component and a loud single S2. As the PVR drops during the first months, the left-to-right shunting of blood into the lungs progressively increases, the systolic murmur becomes louder and longer and may extend into diastole, becoming a continuous murmur. A small aortopulmonary defect may result in a murmur typical of a Patent Ductus Arteriosus (PDA) because there is a pressure gradient from the aorta to the pulmonary artery throughout systole and diastole. If the defect is large and unrepaired, Eisenmenger Syndrome eventually occurs over years with reversal of the shunt. The clinical findings may then only reveal a parasternal lift from right ventricular hypertrophy, a loud single S2 from pulmonary hypertension, absence of any murmur, and cyanosis. 