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

Abstract

Aortopulmonary window is an uncommon condition, particularly so in adulthood because it is usually fatal in infancy or childhood if untreated. Very few cases of those who have survived to adulthood and been operated on successfully have been described. Our study aimed to provide clinical, investigative, surgical, and outcome details of such patients. Retrospective study of consecutive adult patients with aortopulmonary window treated at a tertiary charitable cardiovascular institute in South India between 1996 and 2006. Six adult patients successfully underwent aortopulmonary window closure. Five of the six patients had been correctly diagnosed on echocardiography, while one was only diagnosed after cardiac catheterization for unexplained pulmonary arterial hypertension. Four of the patients had large defects with severe pulmonary arterial hypertension, with pulmonary vascular resistance index (PVRI) ranging from 5.2 to 15.9 at baseline. All showed significant reversibility with oxygen administration, with PVRI on oxygen falling to between 0.6 and 2.2. These patients successfully underwent cardiopulmonary bypass. The other two patients with small lesions underwent ligation off-pump. There was no early or late mortality among these patients. All were in New York Heart Association class I, on follow-up ranging from 3 months to 8 years. 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.