Last update:
07-Jul-2004

A male smoker aged 32 years was admitted because of hemoptysis and shortness of breath. His previous medical history included attacks of deep vein thrombosis, pulmonary embolism, and pulmonary hypertension. The patient was under home oxygen therapy because of development of respiratory failure and anticoagulant prophylactic therapy. On admission heart ultrasound examination revealed severe pulmonary hypertension, while ventilation-perfusion scanning and spiral CT-scan of the lungs confirmed the diagnosis of pulmonary embolism. Coagulation tests revealed reduction of activated protein C. The patient underwent thromboendarterectomy of both the pulmonary arteries and filter placement in the area of the renal veins. Despite postoperative improvement of his condition the patient was readmitted seven months later because of a new attack of pulmonary embolism. This rare case is reported of a young male patient with congenital deficiency of protein C which led to pulmonary hypertension and respiratory failure and the therapeutic dilemmas associated with his future management are discussed.

Key words: Protein C deficiency, Thromboembolic disease.