Unilateral Chronic Thromboembolic Pulmonary Hypertension - A Case Report of Unusual Presentation of CTEPH

Abstract

Chronic thromboembolic pulmonary hypertension categorized as group IV pulmonary according to World Health Organization, is defined as mean pulmonary arterial pressure of more than 25 mm Hg that persists inspite of more than 6 months of anticoagulation in presence of persistent pulmonary thrombi. Chronic thromboembolic pulmonary hypertension is an uncommon and potentially fatal sequelae of acute pulmonary embolism. It is usually diagnosed in late stage due to lack of early diagnosis and carry poor prognosis if definitive treatment is not given. As compared to other categories of pulmonary hypertension, chronic thromboembolic hypertension is potentially curable with pulmonary endarterectomy. The usual presentation is bilateral pulmonary artery involvement. We describe an unusual presentation of unilateral pulmonary artery involvement in a patient with Factor V Leiden mutation. Patient was successfully treated with pulmonary endarterectomy.