Abstract 17056: Report Of An Adult Living With Undiagnosed Total Anomalous Pulmonary Venous Return

Abstract

Case Presentation: 25-year-old male presented with cellulitis and abscess of the lower extremity. He was an immigrant from Guatemala. He worked in landscaping and played soccer with limited exertion. His exam was notable for cyanosis and clubbing of all digits. A II/VI systolic ejection murmur at the left upper sternal border and a mildly accentuated P2 was auscultated. ECG was notable for right axis deviation and right ventricle hypertrophy. Chest x-ray showed a widened mediastinum with perihilar vascular prominence. CT chest with contrast demonstrated all pulmonary veins forming a large, coalescing venous structure draining into the left innominate vein. Transthoracic echocardiogram confirmed total pulmonary venous return by way of a large vertical vein draining into a dilated innominate vein and a 2.7 cm ostium secundum ASD. Discussion: Total anomalous pulmonary venous return (TAPVR) is a rare, often deadly condition. Most patients die in infancy. In the rare adult patient with unrepaired TAPVR, exercise intolerance, cyanosis, clubbing of the digits, and growth retardation is often noted. A right ventricular heave with auscultation of a fixed, split second heart sound with a loud pulmonic component is common. On chest x-ray, the classic “snowman” appearance is seen with vertical vein connections as in our patient. Right heart enlargement represents the base, and an enlarged connecting vertical vein and SVC make up the upper portion. Echocardiography can demonstrate the anomalous pulmonary venous conduit coursing behind the left atrium. CT angiography can also reveal TAPVR and the patient’s specific anatomy. Amongst the few surviving adult TAPVR patients, similar characteristics include large ASDs, short anomalous veins, and absence of pulmonary venous obstruction. A large interatrial connection provides adequate systemic flow. The latter two factors reduce pulmonary vascular resistance, hence improving pulmonary flow and systemic oxygen concentrations.