Infantile pulmonary capillary haemangiomatosis: a lethal form of pulmonary hypertension. (15th July 2015)

• Record Type: Journal Article
• Authors: McGovern, Eiméar; McNally, Paul; O'Sullivan, Maureen; Phelan, Ethna; Sumner, Kelli; Best, D. Hunter; McMahon, Colin J.
• Is Part Of: Cardiology in the young. Volume 26:Number 4(2016)
• Page Start: 663
• Page End: 668
• Publication Date: 2015-07-15
• Subjects: Pulmonary hypertension, -- pulmonary capillary haemangiomatosis, -- pulmonary veno-occlusive disease, -- lung transplant, -- lethal, -- infant; Pediatric cardiology -- Periodicals; 618.9212
• DOI: 10.1017/S1047951115001006
• Languages: English
• ISSNs: 1047-9511

Abstract

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.