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Infantile pulmonary capillary haemangiomatosis: a lethal form of pulmonary hypertension

Published online by Cambridge University Press:  15 July 2015

Eiméar McGovern ,
Paul McNally ,
Maureen O’Sullivan ,
Ethna Phelan ,
Kelli Sumner ,
D. Hunter Best  and
Colin J. McMahon
Eiméar McGovern
Affiliation:
Department of Paediatric Cardiology, Crumlin, Dublin 12, Ireland
Paul McNally
Affiliation:
Department of Pulmonology, Crumlin, Dublin 12, Ireland
Maureen O’Sullivan
Affiliation:
Department of Histopathology, Crumlin, Dublin 12, Ireland
Ethna Phelan
Affiliation:
Department of Radiology Our Lady’s Children’s Hospital, Crumlin, Dublin 12, Ireland
Kelli Sumner
Affiliation:
ARUP institute for Clinical and Experimental Pathology, Salt Lake City, Utah, United States of America
D. Hunter Best
Affiliation:
ARUP institute for Clinical and Experimental Pathology, Salt Lake City, Utah, United States of America Department of Molecular Genetics, University of Utah School of Medicine, Salt Lake City, Utah, United States of America
Colin J. McMahon*
Affiliation:
Department of Paediatric Cardiology, Crumlin, Dublin 12, Ireland
*
Correspondence to: C. McMahon, Cardiac Department, Our Lady’s Children’s Hospital, Crumlin, Dublin 12, Tel: 003531-4096160; Fax: 01-4096181; E-mail: [email protected]
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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.

Keywords

Pulmonary hypertensionpulmonary capillary haemangiomatosispulmonary veno-occlusive diseaselung transplantlethalinfant
Type
Original Articles
Information
Cardiology in the Young , Volume 26 , Issue 4 , April 2016 , pp. 663 - 668
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Copyright
© Cambridge University Press 2015 

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