[Primary pulmonary hypertension]

Abstract

Authors described a case of 26-year old patient with history of progressing dyspnea and repetitive syncopes. In history is intermittent hemoptysis and dyspnea from 1990. Diagnosis of thromboembolic disease was suspected. Clinical picture was dominated by dyspnea, central cyanosis, sinus tachycardia without pulmonary signs of hearth failure. On ecg there is right heart hypertrophy. Echocardiographic examination shows dilatation of right heart, systolic pressure in a. pulmonalis about 90 mmHg and tricuspidal regurgitation of the III. degree. Phlebothrombosis was not found. Complete hemocoagulation examination excluded a primary procoagulating hematologic disease. Pulmonary angiography did not confirm thromboembolic disease but found a high grade pulmonary hypertension--mean pulmonary arterial pressure of 93 mmHg. After complex pneumological examination, including HRCT, and other examinations the diagnosis of primary pulmonary hypertension was made. Patient is indicated to lung transplantation. (Fig. 4, Ref. 9.)