[A child with pulmonary hypertension: from etiological study to therapeutic decisions].

Pulmonary hypertension is a rare but devastating disease, with poor prognosis and high mortality. The disease may present as Primary Pulmonary Hypertension where no clear aetiology can be identified, or be related to a variety of underlying conditions. This is a rare disease in paediatric age. The authors present the clinical case of a 4 years old child, admitted in Paediatric Department of S. João Hospital for syncope episode. The diagnosis of pulmonary hypertension was made by echocardiogram. Ulterior studies revealed pulmonary disease (obliterans bronchiolitis). The treatment included a calcium channel blocker (diltiazem), an endothelin antagonist (bosentan) and supplemental oxygen. This is the first case of obliterans bronchiolitis diagnosed during etiological study for pulmonary hypertension, in our Pediatrics Department. In this case we could not determine the pulmonary disease precipitating event.