title: Infantile pulmonary capillary haemangiomatosis: a lethal form of pulmonary hypertension.
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.
authors.affiliation: 1Department of Paediatric Cardiology,Crumlin,Dublin 12,Ireland.
authors.forename: Eiméar
authors.initials: E
authors.lastname: McGovern
authors.affiliation: 2Department of Pulmonology,Crumlin,Dublin 12,Ireland.
authors.forename: Paul
authors.initials: P
authors.lastname: McNally
authors.affiliation: 3Department of Histopathology,Crumlin,Dublin 12,Ireland.
authors.forename: Maureen
authors.initials: M
authors.lastname: O'Sullivan
authors.affiliation: 4Department of Radiology Our Lady's Children's Hospital,Crumlin,Dublin 12,Ireland.
authors.forename: Ethna
authors.initials: E
authors.lastname: Phelan
authors.affiliation: 5ARUP institute for Clinical and Experimental Pathology,Salt Lake City,Utah,United States of America.
authors.forename: Kelli
authors.initials: K
authors.lastname: Sumner
authors.affiliation: 5ARUP institute for Clinical and Experimental Pathology,Salt Lake City,Utah,United States of America.
authors.forename: D Hunter
authors.initials: DH
authors.lastname: Best
authors.affiliation: 1Department of Paediatric Cardiology,Crumlin,Dublin 12,Ireland.
authors.forename: Colin J
authors.initials: CJ
authors.lastname: McMahon
mesh_terms: D002196:Capillaries; D017809:Fatal Outcome; D005260:Female; D006391:Hemangioma / Q000150:complications*; D006801:Humans; D006976:Hypertension, Pulmonary / Q000209:etiology*; D007223:Infant; D007231:Infant, Newborn; D008168:Lung / Q000098:blood supply*; D008297:Male; D019043:Vascular Neoplasms / Q000150:complications*
keywords: Pulmonary hypertension; infant; lethal; lung transplant; pulmonary capillary haemangiomatosis; pulmonary veno-occlusive disease