Abstract 15712: Imaging Conundrum: The Diagnosis of Unusual Partial Anomalous Pulmonary Venous Return With Dual Connections

Abstract

Case: A 68 year old male with a past medical history of hypertension, hyperlipidemia and chronic kidney disease presented for progressive dyspnea on exertion and bilateral lower extremity edema over three weeks. He was found to be in decompensated heart failure and new onset Atrial Flutter with rapid ventricular rate. Transthoracic echocardiography revealed severely impaired left ventricular systolic function with ejection fraction <20%, enlarged right ventricle, moderate right ventricular systolic dysfunction, biatrial enlargement, mild pulmonary hypertension. He was started on guideline directed medical therapy and referred for transesophageal echocardiogram (TEE) with cardioversion for new onset Atrial Flutter and possible tachycardia induced cardiomyopathy. Imaging findings: During TEE, routine doppler of right upper pulmonary vein showed markedly dilated superior vena cava (SVC) (3.6 cm) and unusual predominant outflow with brief mid late systolic inflow (Fig A and B), suggestive of partial anomalous pulmonary venous return (PAPVR). All three other pulmonary veins appeared normal. Also noted to have positive bubble study likely related to PAPVR. Subsequent right heart catheterization left to right shunt with Qp/Qs 1.6, step up noted between SVC and right atrium. CTA revealed partial anomalous right upper lobe pulmonary vein (RUPV) had dual connection to both SVC via window like communication and left atrium (Fig C), no sinus venosus defect seen. Conclusion: This case illustrates an unusual example of PAPVR with bidirectional flow, as well as the importance of thorough evaluation even on routine transesophageal echocardiograms especially for patients with right heart enlargement.