[Primary pulmonary arterial hypertension in children under 2 years of age]

A retrospective analysis of 18 patients under two years of age who suffered from primary pulmonary hypertension was made between july 1982 november 1988 in order to define their clinical course and prognosis. Perinatal history was irrelevant. In 13 patients diagnosis was established before the first year of life and in 8 of them in the neonatal period. All patients presented the same symptomatology: Cyanosis, dyspnea and/or repeated respiratory infections. The electrocardiogram showed various degrees of enlargement of the right atrium and right ventricle as well as an uneven ST segment in the precordial leads; these could not be related to the systolic or diastolic pressures of the right ventricle. In the chest roentgenogram the most important and constant feature was the heart enlargement. Cardiac catheterization showed a mean pulmonary systolic arterial pressure of 64.46 mmHg and a mean pulmonary arterial resistance of 6.4 U/m.2 These parameters could not be related to a bad prognosis. Three patients were given vasodilators (hydralazine) with positive clinical and echocardiographic results. Five patients died (27%) two months after the diagnosis was made and three months after the onset of symptomatology, four of them died due to congestive heart failure and one had sudden death. This study shows the early onset of the disease as well as its short term high mortality.