PARTIAL ANOMALOUS PULMONARY VENOUS CONNECTION: A RARE CAUSE OF PULMONARY HYPERTENSION

Abstract

SESSION TITLE: Pulmonary Vascular Disease SESSION TYPE: Fellow Case Report Posters PRESENTED ON: 10/09/2018 01:15 PM - 02:15 PM INTRODUCTION: Partial anomalous pulmonary venous connection (PAPVC) is an uncommon cause of pulmonary hypertension (PH). Isolated partial anomalous pulmonary venous connection is a rare congenital heart disease, with a prevalence of 0.1 to 0.2% in the adult population and incidence is between 0.4 – 0.7%. CASE PRESENTATION: A 69-year-old man with history of mental impairment, hypertension and asthma presented to outpatient PH clinic for evaluation of cardiomegaly and abnormal transthoracic echocardiography (TTE). Review of systems was unremarkable except for easy fatigability. Pertinent examination findings include webbed long neck, micrognatia and loud, split S2 at the base of heart. EKG revealed right bundle branch block and on TTE, the right sided chambers were mildly enlarged with mild to moderate tricuspid regurgitation and an estimated right ventricle systolic pressure (RVSP) of 44.8 mmHg. A right heart catheterization (RHC) confirmed moderate PH with a mean pulmonary artery pressure and wedge pressures measurements: 40, 20 mmHg respectively. Oxygen saturations in the IVC, SVC, RA, RV, PA, PCWP: 76%, 99%, 74%, 83%, 83%, 87% respectively. Left to right shunt was 17.3 L/min. Transesophageal echocardiogram (TEE) did not show an intracardiac shunt on color Doppler and saline contrast. CTA chest confirmed the presence of partial anomalous venous return of the right upper lobe into the superior vena cava (SVC). DISCUSSION: An anomalous pulmonary venous connection occurs in approximately 10–15% of ostium secundum atrial septal defect (ASD) and 85% of patients with sinus venosus ASD. PAPVC is more common from right pulmonary veins compared to left side. Anomalous right-sided pulmonary veins drain into the SVC, right atrium, inferior vena cava, azygos vein, portal vein, or hepatic vein. Anomalous left-sided pulmonary veins drain into the left brachiocephalic vein, coronary sinus, or hemiazygos vein. Patients with PAPVR develop pulmonary arterial hypertension (PAH) from increased venous blood return to the right side of the heart through the anomalous vein. The increase in flow and pressure within the pulmonary vascular bed and the associated shear stress lead to vascular remodeling. Clinical presentation is usually non-specific which includes fatigue, shortness of breath, signs and symptoms of right heart failure, PH. PAPVC is diagnosed by TTE, TEE, pulmonary artery angiography, left and right heart catherization. There are number of surgical corrections for PAPVC based on location of anomaly. An anomalous vein on the left side is implanted directly into the left auricular appendage or the left atrium. Right PAPVC repair consists of complete closure of the septal defect, with redirection of the anomalous pulmonary veins into the left atrium. CONCLUSIONS: Diagnosis of PAPVC is challenging with multimodality imaging and diagnostic workup. Although rare, PAPVC should be kept in differential as a cause of PH. Reference #1: MDCT of Partial Anomalous Pulmonary Venous Return (PAPVR) in Adults Mai-Lan Ho, MD et al Reference #2: Partial Anomalous Pulmonary Venous Return Evaluation of 51 Cases. Filiz SENOCAK et al Reference #3: Partial Anomalous Pulmonary Venous Connection and Pulmonary Arterial Hypertension. Sandeep Sahay et al DISCLOSURES: No relevant relationships by Sameh Aziz, source=Web Response No relevant relationships by Domingo Franco-Palacios, source=Web Response No relevant relationships by Venkateswara Kollipara, source=Web Response