Partial anomalous pulmonary venous return: diagnostic challenges and successful correction in a patient with progressive pulmonary hypertension

Abstract

Partial anomalous pulmonary venous return (PAPVR) is a rare congenital anomaly in which one or more pulmonary veins drain into the systemic venous system, creating a left-to-right shunt and potentially causing right-sided volume overload. Frequently associated with atrial septal defects, PAPVR may present with non-specific symptoms such as exertional dyspnoea and is often misdiagnosed as pulmonary hypertension of other origins. We describe a woman with progressive exertional dyspnoea and lower extremity oedema in the setting of newly identified precapillary pulmonary hypertension. Imaging revealed severe right atrial enlargement and a left-to-right shunt from the right superior pulmonary vein draining into the superior vena cava, consistent with PAPVR. She underwent successful surgical correction with the Warden procedure resulting in functional recovery. This case highlights the importance of recognising unexplained right atrial enlargement as a clue to PAPVR and demonstrates the potential for symptomatic and physiological resolution following timely surgical intervention.

Keywords: Anatomic Variation; Pulmonary hypertension.