Hipertensión pulmonar idiopática en pediatría: Caso clínico y revisión de la literatura

Abstract

Idiopathic pulmonary hypertension is a rare disease in the pediatric age. Symptoms are subtle in first stages delaying diagnosis and resulting in worst outcomes. Therefore pediatricians should be aware of the initial symptoms. We report the case of a 13 years oíd girl, who started with progressive dyspnea 9 months befare diagnosis. She was admitted to our hospital because of productive cough, fever and shortness ofbreath. Chest X ray showed enlargement ofright ventricle and pulmonary artery. Echocardiography was suggestive of pulmonary hypertension and the diagnosis was confirmed by cardiac catheterization. Work up far secondary causes of pulmonary hypertension was negative and pulmonary hypertension was considered idiopathic. Treatment with oxygen, diuretics, L-arginine, sildenafil, iloprost and acenocumarol was started with good clinical response.