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Primary pulmonary hypertension in childhood
  1. Sheila G Haworth
  1. British Heart Foundation, Institute of Child Health, 30 Guilford Street, London WC1N 1EH, UK
  1. Professor Haworth.

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This is an opportune time to reappraise primary pulmonary hypertension. The disease is still incurable but recent laboratory studies are beginning to provide clues to its pathogenesis, and we now know that survival is improved by sustained intravenous prostacyclin treatment and by chronic anticoagulation. In 1991, before these treatments were generally available, the median survival was given as 2.8 years, based on the National Institute of Health Registry.1 In patients with a cardiac index of less than 2 l/min/m2, the median survival was 17 months, and untreated patients with a right atrial pressure exceeding 20 mm Hg lived for one month. Recent reports show a 49% survival rate at 30 months and a 25% survival rate at five years in older children and adults treated with continuous intravenous prostacyclin.2Survival is better in younger children.2 In general, the disease is similar in adults and children but there are important differences.

Key messages

  • Greater awareness of the disease should facilitate earlier diagnosis in childhood

  • Management can and should be more aggressive

  • Rarity of primary pulmonary hypertension and recent advances in basic and clinical research indicate the need for closer collaboration and a UK registry of primary pulmonary hypertension

  • During the next decade, the links between genetic predisposition and the cascade of cell signalling events leading to vessel wall pathology will clarify the pathogenesis of the disease and lead to improved treatment strategies

Primary pulmonary hypertension is diagnosed when there is no explanation for the increase in pulmonary arterial pressure. The mean pressure exceeds 25 mm Hg at rest and 30 mm Hg on exercise. The disease is progressive, leading to a gradual increase in right ventricular pressure, right heart failure, functional incapacity, and death. In 1975, a WHO committee defined primary pulmonary hypertension according to morphological criteria, as being caused either by pulmonary …

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