An unexpected and unusual cause of pulmonary hypertension in a patient with hypersensitivity pneumonitis: partial anomalous pulmonary venous connection causing pulmonary artery hypertension

Abstract

Partial anomalous pulmonary venous connection (PAPVC) occurs when any pulmonary vein, but not all, drains directly into the right atrium or its venous tributaries. PAPVC can very rarely present as an individual cause of pulmonary artery hypertension. Here we are presenting a case of a 41-year-old farmer with a history of exertional dyspnoea for the past three years, which increased over 6 months. Chest high-resolution computed tomography (HRCT) was suggestive of non-fibrotic hypersensitivity pneumonitis. Hence the patient was started on systemic steroids, with which the patient’s oxygen saturation improved. On 2D-ECHO, the right ventricle systolic pressure was 48+RAP. Right heart catheterization showed mean pulmonary artery pressure of 73 mmHG, PVR 8.7. On further evaluation, CTPA was done, which surprisingly revealed the left superior pulmonary vein draining into the left brachiocephalic vein.

Keywords

• Partial anomalous pulmonary venous connection
• non-fibrotic hypersensitivity pneumonitis
• pulmonary hypertension