Isolated Partial Anomalous Pulmonary Venous Connection

Abstract

BACKGROUND: Isolated partial anomalous pulmonary venous connection (PAPVC) is a very rare variant of PAPVC, and involves the anomalous drainage of the right upper pulmonary vein into the superior vena cava (SVC) with no associated inter-atrial septal defect. CASE: We present a case of an isolated PAPVC occurring in 26-year old female patient who presented with palpitations, exertional dyspnea and easy fatigability. Physical examination revealed a dynamic precordium, a right ventricular heave, and a tricuspid regurgitation murmur. Transthoracic echocardiogram showed right atrial, ventricular, and main pulmonary artery dilatation with moderate pulmonary hypertension. Hemodynamic studies revealed an isolated PAPVC with severe pulmonary hypertension. She underwent Warden’s procedure (superior vena cava translocation with atriocaval anastomosis), which remarkably improved her symptoms. CONCLUSION: This case report shows a very rare case of isolated PAPVC presenting with heart failure symptoms and right-sided chamber enlargement. The clinical presentation is not specific and may mimic more common diseases, such as acyanotic atrial septal defect and primary pulmonary hypertension. A high index of suspicion is important in diagnosing the disease in patients with right-sided cardiac chamber dilatation.