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INTRODUCTION:Often identified in the first 3 years of life, congenital atresia of the pulmonary vein is a rare anomaly accompanied by congenital heart disease in approximately 30% of cases1. It is associated with significant morbidity and mortality due to recurrent lower respiratory tract infection and persistent dyspnea. Treatment for such anomalies is not clear. CASE PRESENTATION:A healthy 35-year-old male presented with complaints of left sided sharp sub-sternal chest pain radiating to his left arm and neck. He denied exertional dyspnea, hemoptysis, history of recurrent pulmonary infections, epistaxis, or telangiectasias. Computed tomography angiography (CTA) of his chest revealed a large left lower lobe pulmonary arteriovenous malformation (AVM) and a prominent right coronary artery with a left main coronary artery that is not clearly discerned. A CTA coronary revealed the entire coronary system arises from the right coronary cusp and congenital atresia of the left superior pulmonary vein with dilated collateral vessels emptying into the left inferior pulmonary vein. Pulmonary angiography revealed dilated venous structure in the left lung consistent with congenital atresia of the left superior pulmonary vein with collateralized flow of the left upper lobe to the left lower lobe. This abnormal structure ultimately drained into the left atrium via the left inferior pulmonary vein. No embolization or other treatment modalities were performed by interventional radiology. Cardiac coronary angiography revealed both the left and right coronary system arose from the right coronary cusp. The patient's chest pain was self-limited and was ultimately felt to be unrelated to his congenital venous malformation.DISCUSSION: Congenital pulmonary vein atresia is the result of failure of incorporation of the pulmonary vein into the left atrium1 typically identified at birth. Our case is unique both in the lateness of presentation and the patients lack of exertional dyspnea, hemoptysis, or recurrent pulmonary infections. Treatment approaches include pneumonectomy, corrective surgery, or conservative management. Given our patients relative asymptomatic presentation we pursued a more conservative route. Hereditary hemorrhagic telangiectasias is an important differential diagnosis but was ultimately inconsistent with our patients isolated radiographic findings and clinical history. CONCLUSIONS: Our patient's atypical and delayed presentation shows one of the many anatomical variants accompanying congenital pulmonary vein atresia. His collateralized flow from the left upper lobe likely contributed to his lack of classically associated symptoms.

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