A Partial Anomalous Pulmonary Venous Connection in a Severely Symptomatic Patient, Is Surgery Always Recommended?

Partial anomalous pulmonary venous connection (PAPVC) is a rare cardiac anomaly occurring when a pulmonary vein drains into the right atrium, coronary sinus or a systemic vein creating a left-to-right shunt. Symptoms develop from right-sided fluid overload and pulmonary vascular disease. We report a rare case of a severely symptomatic patient with an incidentally discovered PAPVC in the setting of underlying severe pulmonary hypertension from multifactorial severe restrictive lung disease. Despite his worsening symptoms, a multi-disciplinary meeting decided against surgical intervention. Nine months after the decision was made, the patient showed no signs or symptoms of clinical deterioration. Prior studies recommend surgery for PAPVCs with evidence of right ventricular dilation, mild-to-moderate tricuspid regurgitation, or early stages of pulmonary vascular disease. However, our case demonstrates how decision making should consider the shunt's contribution to the overall clinical picture and underlying comorbidities. If a decision is made to defer surgical intervention, strict follow up and repeat re-evaluations for possible risk re-stratification and surgery reconsideration are warranted.