The Indian Journal of Chest Diseases and Allied Sciences

Register      Login

VOLUME 56 , ISSUE 4 ( October-December, 2014 ) > List of Articles


Pulmonary Capillary Haemangiomatosis: A Rare Cause of Pulmonary Hypertension

K. Anand Babu, K. Supraja

Keywords : Pulmonary capillary haemangiomatosis, Pulmonary hypertension

Citation Information : Babu KA, Supraja K. Pulmonary Capillary Haemangiomatosis: A Rare Cause of Pulmonary Hypertension. Indian J Chest Dis Allied Sci 2014; 56 (4):259-262.

DOI: 10.5005/ijcdas-56-4-259

License: CC BY-NC 4.0

Published Online: 28-06-2022

Copyright Statement:  Copyright © 2014; The Author(s).


Pulmonary capillary haemangiomatosis (PCH) is a rare disorder of unknown aetiology, characterised by proliferating capillaries that invade the pulmonary interstitium, alveolar septae and the pulmonary vasculature. It is often mis-diagnosed as primary pulmonary hypertension and pulmonary veno-occlusive disease. Pulmonary capillary haemangiomatosis is a locally aggressive benign vascular neoplasm of the lung. We report the case of a 19-year-old female who was referred to us in the early post-partum period with severe pulmonary artery hypertension, which was diagnosed as PCH by open lung biopsy.

PDF Share
  1. Eltorky MA, Headley AS, Winer-Muram H, Garret HE, Griffin JP. Pulmonary capillary hemangiomatosis: a clinicopathologic review. Ann Thorac Surg 1994;57:772-6.
  2. Frazier AA, Franks TJ, Mohammed TLH, Ozbudak IH, Galvin JR. From the archives of the AFIP: pulmonary venoocclusive disease and pulmonary capillary hemangiomatosis. Radiographics 2007;27:867-82.
  3. Almagro P, Julià J, Sanjaume M, González G, Casalots J, Heredia JL, et al. Pulmonary capillary hemangiomatosis associated with primary pulmonary hypertension: report of 2 new cases and review of 35 cases from the literature. Medicine (Baltimore) 2002;81:417-24.
  4. Wagenvoort CA, Beetstra A, Spijker J. Capillary hemangiomatosis of the lungs. Histopathology 1978;2:401-6.
  5. Oviedo A, Abramson LP, Worthington R, Dainauskas JR, Crawford SE. Congenital pulmonary capillary hemangiomatosis: report of two cases and review of the literature. Pediatr Pulmonol 2003;36:253-6.
  6. Wirbelauer J, Hebestreit H, Marx A, Mark EJ, Speer CP. Familial pulmonary capillary hemangiomatosis early in life. Case Rep Pulmonol 2011;2011:827591.
  7. Langleben D, Heneghan JM, Batten AP, Wang NS, Fitch N, Schlesinger RD, et al. Familial pulmonary capillary hemangiomatosis resulting in primary pulmonary hypertension. Ann Intern Med 1988;109:106-9.
  8. Best DH, Sumner KL, Austin ED, Chung WK, Brown LM, Borczuk AC, et al. EIF2AK4 mutations in pulmonary capillary hemangiomatosis. Chest 2014;145:231-6.
  9. Ito K, Ichiki T, Ohi K, Egashira K, Ohta M, Taguchi K, et al. Pulmonary capillary hemangiomatosis with severe pulmonary hypertension. Circ J 2003;67:793-5.
  10. Olin J, Abman S, Grady R, Ivy D, Lovell M, Partrick D, Balasubramaniam V. A 7-year-old with pulmonary hypertension. BMJ Case Rep 2011;2011: bcr0220113843.
  11. Kawut SM, Assaad AM, Arcasoy SM, Rosenweig EB, Sonett JR, Borczuk AC. Pulmonary capillary hemangiomatosis: results of gene expression analysis. Chest 2005;128:575S-576S.
  12. Assaad AM, Kawut SM, Arcasoy SM, Rosenzweig EB, Wilt JS, Sonett JR, et al. Platelet-derived growth factor is increased in pulmonary capillary hemangiomatosis. Chest 2007; 131:850-5.
  13. Sullivan A, Chmura K, Cool CD, Keith R, Schwartz GG, Chan ED. Pulmonary capillary hemangiomatosis: an immunohistochemical analysis of vascular remodeling in a fatal case. Eur J Med Res 2006;11:187-3.
  14. Fernandez-Alonso J, Zulueta T, Reyes-Ramirez JR, Castillo- Palma MJ, Sanchez-Roman J. Pulmonary capillary hemangiomatosis as cause of pulmonary hypertension in a young woman with systemic lupus erythematosus. J Rheumatol 1999;26:231-3.
  15. Kishida Y, Kanai Y, Kuramochi S, Hosoda Y. Pulmonary veno-occlusive disease in a patient with systemic lupus erythematosus. J Rheumatol 1993;20:2161-2.
  16. Morassut PA, Walley VM, Smith CD. Pulmonary venoocclusive disease and the CREST variant of scleroderma. Canadian J Cardiol 1992;8:1055-8.
  17. Gugnani MK, Pierson C, Vanderheide R, Girgis RE. Pulmonary edema complicating prostacyclin therapy in pulmonary hypertension associated with scleroderma: a case of pulmonary capillary hemangiomatosis. Arthritis Rheum 2000;43:699-703.
  18. Jing X, Yokoi T, Nakamura Y, Nakamura M, Shan L, Tomimoto S, et al. Pulmonary capillary hemangiomatosis: a unique feature of congestive vasculopathy associated with hypertrophic cardiomyopathy. Arch Pathol Lab Med 1998;122:94-6.
  19. White CW, Sondheimer HM, Crouch EC, Wilson H, Fan LL. Treatment of pulmonary hemangiomatosis with recombinant interferon alfa-2a. N Engl J Med 1989; 320:1197-200.
  20. Ginns LC, Roberts DH, Mark EJ, Brusch JL, Marler JJ. Pulmonary capillary hemangiomatosis with atypical endotheliomatosis: successful antiangiogenic therapy with doxycycline. Chest 2003;124:2017-22.
  21. Humbert M, Maitre S, Capron F, Rain B, Musset D, Simonneau G. Pulmonary edema complicating continuous intravenous prostacyclin in pulmonary capillary hemangiomatosis. Am J Respir Crit Care Med 1998;157:1681-5.
  22. Kothari SS, Jagia P, Gupta A, Singh N, Ray R. Images in cardiovascular medicine. Pulmonary capillary hemangiomatosis. Circulation 2009;120:352-4.
PDF Share
PDF Share

© Jaypee Brothers Medical Publishers (P) LTD.