A case series of six children with primary pulmonary hypertension.

INTRODUCTION: Primary pulmonary hypertension is an uncommon but serious disease in children. Management is difficult despite recent advances in pharmacotherapy. CLINICAL PICTURE: We reviewed patients with this condition with respect to their presenting symptoms, investigations, treatment and outcome. TREATMENT: These children were treated with individualised combinations of oxygen, diuretics and calcium antagonists. OUTCOME: In our follow-up of 5 children, 4 had died. CONCLUSION: This condition is irreversible and progressive with a high mortality rate. A better understanding of, and research into, the pathogenesis would hopefully lead to the formulation of improved therapeutic strategies for this condition.