Partial Anomalous Pulmonary Venous Return: A Cause of Pulmonary Hypertension in a Geriatric Patient

S. Hussain Khan, University of Nevada, Las Vegas
C. McLaughlin, SAMMC


Introduction: Partial anomalous pulmonary venous return (PAPVR) involves abnormal return of blood to the heart and several variants have been described. Partial anomalous pulmonary venous connection is the variant in which a pulmonary vein drains oxygenated blood from the lungs to a systemic vein leading to mixing of blood. PAPVR is a rare disorder that has been reported as 0.4-0.7% based on autopsy data. 80-90% of cases are associated with an atrial septal defect (ASD). We present a report of an elderly female presenting with dyspnea on exertion and evidence of pulmonary hypertension found to have a right upper lobe PAPVR without ASD. Case Presentation: A 72 year old woman with a history of atrial fibrillation on anticoagulation, hypertension, diastolic heart failure with preserved ejection fraction presented with progressive exertional shortness of breath with minimal exertion and leg edema for the last 2-3 months. An echocardiogram was performed with EF 51% with RVSP reported as 60 mmHg, moderate dilation of left and right atria, moderate right ventricular dilation with moderate tricuspid regurgitation. Autoimmune evaluation, CT angiography, pulmonary function testing and V/Q scan were unremarkable. Right and left heart catheterizations were performed demonstrating elevated pulmonary arterial (PA) pressures with a mean PA pressure measuring 32 mmHg and an LVEDP of 16 mmHg. During shunt evaluation oxygen saturation measurements demonstrated a step up in oxygenation from the right atrium to the right ventricle with a shunt fraction of 24%, raising concern for an ASD. She underwent further workup with TEE without evidence of intracardiac shunt, so a venous phase chest CT was performed demonstrating a right upper lobe PAPVR at the level of the azygous vein. The patient was managed with medical therapy noting improvement in her symptoms. She was evaluated for surgical intervention and it was deemed unnecessary at this time. Discussion: We present a case of congenital PAPVR diagnosed at 72 years of age presenting with pulmonary hypertension. Although rare the diagnosis of PAPVR should be considered during the evaluation of pulmonary hypertension. The primary clue to diagnosis was the step up in oxygen saturation during RHC. Further characterization can be performed with venous phase chest CT and cardiac MRI. Fortunately, not all cases require surgical intervention. Conclusion: PAPVR is a rare and silent congenital anomaly leading to pulmonary hypertension. During RHC step up in oxygen saturation can provide essential clues to diagnosis of this potentially curable condition.