Infantile pulmonary capillary haemangiomatosis: a lethal form of pulmonary hypertension
CARDIOLOGY IN THE YOUNG(2016)
摘要
We describe the cases of two children who both presented in infancy with recurrent severe pulmonary hypertensive crises. Exhaustive clinical work-up failed to identify an underlying aetiology. The patients had no clinical response to steroids, immunoglobulins, or pulmonary vasodilators. Post-mortem examination revealed extensive invasive pulmonary capillary haemangiomatosis. There was no evidence of pulmonary venous occlusive disease. Given the lethal nature of this condition, early consideration of referral to a lung transplant centre should be considered in selected patients.
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关键词
Pulmonary hypertension,pulmonary capillary haemangiomatosis,pulmonary veno-occlusive disease,lung transplant,lethal,infant
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