Chronic thromboembolic pulmonary artery hypertension with deep vein thrombosis due to protein s deficiency

A 30 year old male presented with the complaint of progressive dyspnoea. Cardiovascular examination showed tachycardia and loud second sound in the pulmonary area. ECG showed T wave inversion in lead III and chest X-ray showed dilatation of the pulmonary artery. Echocardiography showed mildly dilated right atrium/right ventricle, mild tricuspid regurgitation and moderate pulmonary arterial hypertension. Venous Doppler of the bilateral lower limbs showed deep vein thrombosis of both the lower limbs. HRCT (high resolution CT) showed pleural thickening in the apical segment of the left upper lobe and scattered ground glass attenuation in the apical basal segment of both the lower lobes, which were suggestive of thromboembolism. CECT (contrast enhanced CT) of chest showed pulmonary artery thrombosis of the left lower lobe segmental and interlobar artery. Protein S activity was 25 %(normal range: 77-143%), protein C activity was 82 %(normal range: 70-130%) and antithrombin III was 119(normal range: 80-120).