Primary pulmonary hypertension in children living at high altitude

Eleven cases of primary pulmonary hypertension in children living at high altitudes are reported. The most common presenting symptoms were cough, dyspnea, cyanosis, and episodes of syncope. The major physical signs were related to the cardiovascular system. The electrocardiogram was consistent with marked right ventricular hypertrophy. Cardiac catheterization was performed in 5 patients, revealing moderate to severe degree of pulmonary arterial hypertension. Two patients died and their autopsy findings confirmed the diagnosis of pulmonary hypertension resulting from changes in the pulmonary vasculature. Therapy consisting of oxygen, digitalis, and reduction of environmental altitude was found to be effective.