[Primary pulmonary hypertension in children]

Primary pulmonary hypertension is a clinical syndrome with severe pulmonary hypertension where other causal diseases are excluded. The condition is progressive, fatal and disappointingly resistant to therapy. In this article we present three children with the disease. Two of the patients died shortly after diagnosis, one of them during heart catheterization. The third patient showed good response to treatment with calcium channel blockers, diuretics and nightly supplementary oxygen. Quality of life and hemodynamics improved during the first six months of treatment, and remained virtually unchanged for another two years. Her condition then deteriorated rapidly, and she died three years after diagnosis. According to the literature and our experience, investigations with non-invasive methods such as oxygen saturation measurement, echocardiography, and a simple exercise test provide sufficient information to start therapy and monitor therapeutic response. Some patients show vasodilatory response to treatment with drugs, and might benefit from therapy with calcium channel blockers.