Isolated Partial Anomalous Pulmonary Venous Connection: Development of Volume Overload and Elevated Estimated Pulmonary Pressure in Adults

OBJECTIVE: Partial anomalous pulmonary venous connection (PAPVC) is one of the rare congenital cardiac diseases with a prevalence of 0.4–0.7% of autopsies. The prevalence of a partial anomalous pulmonary venous connection is 0.2% in computed tomography (CT) among adults. We chose to study the association between isolated PAPVC and volume overload, estimated systolic pulmonary artery pressure in a tertiary care center for cardiovascular diseases. METHODS: CT report database was searched for keywords of partial anomalous pulmonary venous connection, pulmonary hypertension, dilated right atrium (RA), and right ventricle (RV). Both pediatric and adult population were considered. All the dedicated studies of non-coronary cardiac evaluation, pulmonary arteries, and thorax were included in the study. Echocardiography was performed in all the subjects. In adults, abnormalities searched were features of volume overload of RA and RV and estimated systolic pulmonary pressure of 45 mmHg. Biphasic studies were performed, and upper abdomen was included in the CT studies. RESULTS: Among the 110 subjects, 54 (49%) had isolated PAPVC. Of 54, 26 patients had volume overload of RA/RV or elevated estimated systolic pulmonary artery pressure. There is a significant association between drainage of anomalous veins to superior vena cava (SVC) and age >18 years (Chi-squared test P = 0.003). Among patients with isolated PAPVC, 18 had anomalous drainage to the SVC. Among isolated PAPVC cases, 38 were of the age >18 years. We found statistically significant association (P = 0.02) between isolated PAPVC in adults and pulmonary hypertension. CONCLUSION: Isolated PAPVC has association with the development of pulmonary hypertension in adults, approaching statistically significant p value. Because isolated PAPVC is a clinically significant independent risk factor, it should be actively treated to prevent the development of pulmonary hypertension later in life, which may result in severe clinical consequences.