Infantile pulmonary capillary haemangiomatosis: a lethal form of pulmonary hypertension
- Resource Type
- Authors
- Ethna Phelan; Paul McNally; D. Hunter Best; Maureen J. O'Sullivan; Kelli Sumner; Colin J. McMahon; Eimear McGovern
- Source
- Cardiology in the Young. 26:663-668
- Subject
- Male
medicine.medical_specialty
Hypertension, Pulmonary
Occlusive disease
030204 cardiovascular system & hematology
03 medical and health sciences
Pulmonary capillary haemangiomatosis
Fatal Outcome
0302 clinical medicine
Internal medicine
medicine
Humans
Lung
business.industry
Infant, Newborn
Infant
General Medicine
medicine.disease
Pulmonary hypertension
Vascular Neoplasms
Capillaries
medicine.anatomical_structure
030228 respiratory system
Pediatrics, Perinatology and Child Health
Cardiology
Etiology
Female
Pulmonary Veno-Occlusive Disease
Hemangioma
Cardiology and Cardiovascular Medicine
business
Pulmonary vasodilators
- Language
- ISSN
- 1467-1107
1047-9511
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.