RESUMEN
We report the case of a 33- years-girl with SCA and severe PH. She developed six month before admission, non productive couph and dyspnea. Physical examination at admission revealed shortness of breath and right heart ventricular failure. Electrocardiography showed sinus rhythm and an incomplete left bundle branch block. Chest roentgenography revealed cardiomegaly with cardiothoracic index at 0.66 and pulmonary infiltrates. Laboratory tests revealed an anemia with hemoglobin of 7,1 g/dl, white blood cell count of 12500/mm, moderate renal failure [cretininemia=178 micromol/l] and hypoxemia with oxygen pressure of 60 mmHg. Hemoglobin electrophoresis revealed an heterozygous SCA. Echocardiography revealed dilatation of right heart cavities and a systolic pulmonary artery pressure of 60 mmHg. A perfusion lung scintigraphy demonstrated multiple subsegmental perfusion defects. PH is a common complication of adult patients with SCA. Appropriate therapies and strategies for prevention of PH in SCA are unkown. Further research exploring therapies such as oxygen, nitric oxide, prostacyclin and hydroxyurea are indicated