Partial anomalous pulmonary venous connection natural history, complications and prognosis

Partial anomalous pulmonary venous connection Microchapters
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-In-Chief: Priyamvada Singh, M.B.B.S. [2], Keri Shafer, M.D. [3]; Assistant Editor(s)-In-Chief: Kristin Feeney, B.S. [4]

Overview

Congenital heart disease is the most common birth defect but partial anomalous pulmonary venous connection is a relatively rare form of congenital heart disease. As partial anomalous pulmonary venous connection is asymptomatic in early childhood and adolescence, many people can live well into adulthood before health complications may impact the quality of life.

Natural History and Complications

There are few symptoms in infancy or childhood. After many years of excess pulmonary venous return, right atrial and right ventricular (RV) dilation may occur as a result of volume overload in the adult. RV dilation and enlargement is in turn associated with arrhythmias, cor pulmonale or right-sided heart failure, and, in some patients, the development of pulmonary hypertension.