This case series highlights the progressive nature and high mortality of primary pulmonary hypertension in children despite available pharmacotherapy.
Warrants prognostic caution and close monitoring in pediatric primary pulmonary hypertension; leaves open optimal therapies.
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.
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Quek et al. (2001) studied this question.
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