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Infantile pulmonary capillary haemangiomatosis: a lethal form of pulmonary hypertension

  • Eiméar McGovern (a1), Paul McNally (a2), Maureen O’Sullivan (a3), Ethna Phelan (a4), Kelli Sumner (a5), D. Hunter Best (a5) (a6) and Colin J. McMahon (a1)...

Abstract

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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Corresponding author

Correspondence to: C. McMahon, Cardiac Department, Our Lady’s Children’s Hospital, Crumlin, Dublin 12, Tel: 003531-4096160; Fax: 01-4096181; E-mail: cmcmahon992004@yahoo.com

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Keywords

Infantile pulmonary capillary haemangiomatosis: a lethal form of pulmonary hypertension

  • Eiméar McGovern (a1), Paul McNally (a2), Maureen O’Sullivan (a3), Ethna Phelan (a4), Kelli Sumner (a5), D. Hunter Best (a5) (a6) and Colin J. McMahon (a1)...

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