Aortopulmonary Window in Adults: Diagnosis and Treatment of Late-presenting Patients

Abstract

Aortopulmonary window may rarely present in adulthood. The diagnosis can usually be made by careful echocardiography alone. Even in the presence of severe pulmonary arterial hypertension, if a significant reversibility in pulmonary vascular resistance can be demonstrated with oxygen, the condition can be successfully corrected with good long-term outcomes.

Aortopulmonary window in adults: A rare entity leading to Eisenmenger syndrome

“…If the baseline PVR index is between 6 and 9 WUxm2 with a ratio of PVR to SVR of around 0.3–0.5, then vasodilator challenge (with oxygen or nitric oxide) is strongly encouraged and it is broadly accepted that these patients are surgical candidates if all of the following criteria are met: a decrease of PVR index of 20%, PVR to SVR ratio decrease of 20%, final PVR index < 6 WUxm2, and a final PVR to SVR ratio < 0.3 . A heart–lung transplant can still be an option in such patients that are not eligible for repair . Some authors however question how accurately hemodynamic measures can determine risk of death or whether or not the patient will develop persistent PVR following repair as other factors such as the type of cardiac defect or genetic predisposition of the patient can alter hemodynamic testing or have an impact on outcome after surgical correction .…”

“… 12. A heart-lung transplant can still be an option in such patients that are not eligible for repair 13,14. Some authors however question how accurately hemodynamic measures can determine risk of death or whether or not the patient will develop persistent PVR following repair as other factors such as the type of cardiac defect or genetic predisposition of the patient can alter hemodynamic testing or have an impact on outcome after surgical correction.…”

Current treatment of aortopulmonary window

“…In severe cases, acidosis with very low cardiac output may be seen. In rare cases, the APW is small and restrictive, and patients may experience only mild or no symptoms and remain undiagnosed until adulthood [6].…”

“…• APW is almost exclusively a pediatric disease. Few cases of APW with restrictive fl ow have gone undiagnosed until adulthood, but most commonly these cases are managed by pediatric cardiothoracic surgeons and cardiologists [6].…”

“…In severe cases, acidosis with very low cardiac output may be seen. In rare cases, the APW is small and restrictive, and patients may experience only mild or no symptoms and remain undiagnosed until adulthood [6].Physical examination may demonstrate a prominent second heart sound, a systolic murmur, and a widened pulse pressure. Echocardiography normally is suffi cient Aortopulmonary window is a rare abnormal congenital communication between the pulmonary artery and the ascending aorta with intact aortic and pulmonary valves.…”