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.
Pulmonary arterial hypertension (PAH) is an uncommon but serious and debilitating disorder seen in children and adults. By definition, it is an elevation of the pressure in the pulmonary artery to above a mean of 25 mmHg at rest, or 30 mmHg during exercise.
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