Evaluation of Pulmonary Hypertension in a Patient of Cryptogenic Organizing Pneumonia (COP) Unveils a Rare Congenital Anomaly

Abstract

INTRODUCTION: Partial anomalous pulmonary venous return (PAPVR) is an uncommon congenital anomaly and a rare cause of adult onset pulmonary hypertension. We present a case of pulmonary hypertension and right ventricular (RV) failure initially thought to be secondary to COP which unveiled PAPVR as the more likely major contributing factor. CASE PRESENTATION: A 21 year old African American female initially presented with symptoms of pneumonia to another hospital and was treated with antibiotics without clinical improvement. A CT scan of the chest revealed bilateral patchy ground glass opacities predominantly in lower lobes. Subsequent CT-guided lung biopsy was consistent with COP. She was discharged with oxygen and prednisone but presented 3 months later, off prednisone, with progressive shortness of breath, fatigue, multiple joint pains and Raynaud's phenomenon. Serologies for connective tissue diseases were negative. Pulmonary function testing revealed moderate restriction. She was started on high dose steroids. Echocardiogram showed normal left ventricular function and severely dilated and reduced right ventricular function. Pulmonary artery systolic pressure was elevated (41mm Hg). Given the patient young age and degree of right ventricle dysfunction was deemed > for World Health Organization (WHO) group 3 pulmonary hypertension from COP, a complete evaluation was performed including a right heart catheterization. It revealed elevated right atrial pressure, normal wedge pressure; mild pulmonary hypertension (mean PA) 30 mm Hg, transpulmonary gradient 25 mmHg, reduced cardiac output (2.34 Liters/min), and moderately elevated pulmonary vascular resistance of 6.57 Woods units. Serial saturations suggested a possible step up at the SVC as compared with IVC. This > for World Health Organization (WHO) group 3 pulmonary hypertension from COP, a complete evaluation was performed including a right heart catheterization. It revealed elevated right atrial pressure, normal wedge pressure; mild pulmonary hypertension (mean PA) 30 mm Hg, transpulmonary gradient 25 mmHg, reduced cardiac output (2.34 Liters/min), and moderately elevated pulmonary vascular resistance of 6.57 Woods units. Serial saturations suggested a possible step up at the SVC as compared with IVC. This > pulmonary hypertension and RV failure lead to a cardiac MRI which revealed PAPVR (left superior pulmonary vein draining into left brachiocephalic vein). As patient was acutely ill, it was decided to defer closure until the COP was treated with high dose steroids. At 3 months follow up her COP radiological signs have improved but the patient remains functional class 3 (six minute walk test 240 meters). She was started on phosphodiesterase type 5 inhibitor to improve her predominately WHO group 1 pulmonary hypertension in preparation for correction of PAPVR. DISCUSSION: The persistence of high pulmonary arterial systolic pressure despite treatment of COP signifies PAPVR as the main contributing factor for pulmonary hypertension in this case. CONCLUSIONS: Right ventricular failure and pulmonary hypertension > pulmonary hypertension and RV failure lead to a cardiac MRI which revealed PAPVR (left superior pulmonary vein draining into left brachiocephalic vein). As patient was acutely ill, it was decided to defer closure until the COP was treated with high dose steroids. At 3 months follow up her COP radiological signs have improved but the patient remains functional class 3 (six minute walk test 240 meters). She was started on phosphodiesterase type 5 inhibitor to improve her predominately WHO group 1 pulmonary hypertension in preparation for correction of PAPVR. DISCUSSION: The persistence of high pulmonary arterial systolic pressure despite treatment of COP signifies PAPVR as the main contributing factor for pulmonary hypertension in this case. CONCLUSIONS: Right ventricular failure and pulmonary hypertension > of hypoxic lung disease should trigger a thorough evaluation for other WHO group etiologies of pulmonary hypertension. Further, it is unknown whether there exists any pathophysiological connection PAPVR and COP.