Hostname: page-component-84c44f86f4-d74w9 Total loading time: 0 Render date: 2025-10-14T12:21:14.722Z Has data issue: false hasContentIssue false
  • English
  • Français

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: cmcmahon992004@yahoo.com
Get access Rights & Permissions [Opens in a new window]

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

Information

Type
Original Articles
Information
Cardiology in the Young , Volume 26 , Issue 4 , April 2016 , pp. 663 - 668
Check for updates
Copyright
© Cambridge University Press 2015 

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

Article purchase

Temporarily unavailable

References

1. Steinhorn, RH. Neonatal Pulmonary Hypertension. Pediatr Crit Care Med 2010; 11: S79–S84.Google Scholar
2. Hwang, JH. Neonatal pulmonary hypertension. Korean J Perinatol 2013; 24: 1–10.CrossRefGoogle Scholar
3. Waagenvort, CA, Beetra, A, Spiker, J. Capillary haemangiomatosis of the lung. Histopathology 1978; 2: 201–206.Google Scholar
4. Eltorky, MA, Headley, S, Winer-Muram, H, Garrett, HE, Griffin, JP. Pulmonary capillary haemangiomatosis: a clinicopathologic review. Ann Thorac Surg 1994; 57: 772–776.Google Scholar
5. Ito, K, Ichiki, T, Ohi, K, et al. Pulmonary capillary hemangiomatosis with severe pulmonary hypertension. Circ J 2003; 67: 793–795.Google Scholar
6. Bartyik, K, Bede, O, Tiszlavicz, L, et al. Pulmonary capillary haemangiomatosis in children and adolescents: report of a new case and review of the literature. Eur J Pediatr 2004; 163: 731–737.Google Scholar
7. Oviedo, A, Abramson, LP, Worthington, R, et al. Congenital pulmonary capillary haemangiomatosis: report of two cases and review of the literature. Pediatr Pulmonol 2003; 36: 471–475.CrossRefGoogle ScholarPubMed
8. Takiguchi, Y, Urama, T, Hiroshima, K, et al. Stable pulmonary capillary haemangiomatosis without symptomatic pulmonary hypertension. Thorax 2001; 56: 815–817.CrossRefGoogle ScholarPubMed
9. Frazier, AA, Franks, TJ, Mohammed, TLH et al. From the Archives of the AFIP. Pulmonary veno-occlusive disease and pulmonary capillary hemangiomatosis. Radiographics 2007; 27: 867–882.Google Scholar
10. Kothari, SS, Jagia, P, Gupta, A, et al. Pulmonary capillary hemangiomatosis. Circulation 2009; 120: 352–354.Google Scholar
11. Dufour, B, Maitre, S, Humbert, M, et al. High-resolution CT of the chest in patients with pulmonary capillary haemangiomatosis or pulmonary veno-occlusive disease. AJR 1998; 171: 1321–1324.Google Scholar
12. Aiello, VD, Thomaz, AM, Pozzan, G, Lopes, AA. Capillary hemangiomatosis like-lesions in lung biopsies from children with congenital heart defects. Pediatr Pulmonol 2014; 49: E82–E85.Google Scholar
13. Khan, MS, Heinle, JS, Samayoa, AX, et al. Is lung transplantation survival better in infants? Analysis of over 80 infants. J Heart Lung Transplant 2013; 32: 44–49.Google Scholar
14. Conrad, C, Cornfield, D. Pediatric lung transplantation: promise being realised. Curr Opin Pediatr 2014; 26: 334–342.Google Scholar
15. Mallory, GB, Spray, TL. Paediatric lung transplantation. Eur Respir J 2004; 24: 839–845.Google Scholar
16. White, CW, Sondheimer, HM, Crouch, EC, Wilson, H, Fan, LL. Treatment of pulmonary hemangiomatosis with recombinant interferon alpha-2a. N Engl J Med 1989; 320: 1197–1200.Google Scholar
17. White, CW. Treatment of haemangiomatosis with recombinant interferon alpha. Semin Haematol 1990; 27: 15–22.Google Scholar
18. White, CW, Wolf, SJ, Korones, DL, et al. Treatment of childhood angiomatous diseases with recombinant interferon alpha 2a. J Pediatr 1991; 118: 59–66.Google Scholar
19. Ginns, LC, Roberts, DH, Mark, EJ, et al. Pulmonary capillary hemangiomatosis with atypical endotheliomatosis: successful antiangiogenic therapy with doxycycline. Chest 2003; 124: 2017–2022.Google Scholar
20. Ishii, H, Iwabuchi, K, Kameya, T, et al. Pulmonary capillary haemangiomatosis. Histopathology 1996; 29: 275–278.Google Scholar
21. Best, DH, Sumner, KL, Austin, ED, et al. EIF2AK4 mutations in pulmonary capillary haemangiomatosis. Chest 2014; 145: 231–236.Google Scholar
22. Eyries, M, Montani, D, Girerd, B, et al. EIF2AK4 mutations cause pulmonary veno-occlusive disease, a recessive form of pulmonary hypertension. Nat Genet 2014; 46: 65–69.Google Scholar
23. Lantuéjoul, S, Sheppard, MN, Corrin, B, Burke, MM, Nicholson, AG. Pulmonary veno-occlusive disease and pulmonary capillary haemangiomatosis: a clinicopathological study of 35 cases. Am J Surg Pathol 2006; 30: 850–857.Google Scholar