Total anomalous pulmonary venous connection natural history

Associate Editor-In-Chief:; Keri Shafer, M.D. [mailto:kshafer@bidmc.harvard.edu] Priyamvada Singh, MBBS mailto:psingh@perfuse.org

Assistant Editor-In-Chief: Kristin Feeney, B.S. mailto:kfeeney@perfuse.org

Overview
The natural history of untreated patients of TAPVC is not very favorable. It depends on the type of obstruction and amount of shunting across the atrium.

Natural History
The natural history of untreated patients of total anomalous pulmonary venous connection depends on-


 * Type of obstruction (unobstructed, obstructed)
 * Type of anatomic variant(cardiac, infracardiac or supracardiac)
 * Amount of right-to-left shunting

Untreated patients


 * Patients with severe obstruction may not be able to survive beyond few months of life.
 * 8 out of 10 patients with small interatrial connections and obstruction die as an infant.
 * The presentation of patients with unobstructed TAPVC and large interatrial shunting may vary from asymptomatic to symptoms due to progressive right heart failure