A63-05 When Veins Take a Wrong Turn: Partial Anomalous Pulmonary Venous Return as a Cause of Pulmonary Arterial Hypertension

Abstract Background Partial anomalous venous return (PAPVR) is part of a spectrum of congenital cardiovascular malformations in which one or more pulmonary veins return to the right atrium or systemic venous circulation rather than draining into the left atrium. While 9-43% with chronic left to right shunting develop pulmonary hypertension (PH) due to pulmonary over circulation, co-morbid lung disease can accelerate the development of severe pre-capillary PH and right heart failure due to its myriad of imparts on pulmonary vascular remodeling. We describe a rare case of a patient with PAPVR developing pre-capillary PH with subsequent improvement following initiation of pulmonary artery vasodilatory therapies. Case Description A 66-year-old female with chronic obstructive pulmonary disease (COPD) presented to the Pulmonary Vascular Disease clinic for further evaluation of progressive dyspnea and exertional hypoxia that seemed disproportionate to the severity of her COPD. Initial work up with trans-thoracic echocardiogram demonstrated right ventricular (RV) systolic pressure of 80 mmHg with severe RV systolic dysfunction. Initial right heart catheterization (RHC) revealed a right atrial pressure (RAP) of 11 mmHg, RV pressure of 90/10 mmHg, mean pulmonary artery pressure (mPAP) 64.7 mmHg, pulmonary capillary wedge pressure (PCWP) of 12 mmHg, pulmonary vascular resistance (PVR) of 10.74 Woods Units. Contrasted CT chest imaging revealed a partial anomalous pulmonary vein draining from the left upper lobe into the left brachiocephalic vein. In addition to diuretics, she was initiated on tadalafil and ambrisentan with good tolerance of both. As part of a clinical trial evaluating the use of early, upfront parenteral remodulin therapy to achieve rapid right ventricular (RV) remodeling, she was initiated on parenteral remodulin with further improvement in her functional status and echocardiographic RV function. Hemodynamics and echocardiography improved as shown in table 1. Discussion This rare presentation of pre-capillary PH highlights the importance of maintaining a broad differential and systematic approach to the evaluation of patients with pre-capillary PH. Patients with PAPVR rarely develop severe RV failure (5% of all PAVPR cases) due to milder severity of their left to right shunting. However, with her co-morbid COPD and it’s well-known association with pre-capillary PH, the chronic left to right shunt presented an additional contributing factor for the development of PH. Despite the presence of COPD, she demonstrated a good response to oral and parenteral pulmonary vasodilators. Our case demonstrates the importance of phenotyping and appropriate management of pulmonary vascular disease in the setting of comorbid lung disease. This abstract is funded by: None