Contemporary Clinical Management of Atrial Septal Defects in the Adult

Gianluca Rigatelli; Paolo Cardaioli; Ziyad M Hijazi


Expert Rev Cardiovasc Ther. 2007;5(6):1135-1146. 

In This Article

Pulmonary Arterial Hypertension

Pulmonary vascular disease may occur in up to 5-10% of patients with untreated ASDs, especially in females. In rare occasions, probably also due to recurrent embolic phenomena, high pulmonary resistance causes right-to left shunting at atrial level leading to the Eisenmenger syndrome that occurs when the ASD in not-restrictive and when there is rest cyanosis. Sudden death, heart failure, hemoptysis are the main death causes in such patients. Surgery may include heart-pulmonary transplantation and pulmonary transplantation but is of very limited importance due to high mortality and poor number of donators; moreover, there are no studies about patients with previous ASD. Although there is no trial in the pipeline for a new vasodilator in untreated ASD patients, latest generation vasodilators, such as bosentan and epoprostenol, have been reported to effectively manage such patients.[60,61]