Pulmonary hypertension

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Pulmonary hypertension is bad stuff that arises from heart problems, an assortment of lung pathologies and some drugs.

Plexiform lesion of the lung, a finding of pulmonary hypertension. (WC/Rosen)

Hypertension, more generally, is dealt with in the hypertension article.

General classification

  • Primary, i.e. primary pulmonary hypertension, or
  • Secondary, e.g. due to congenital heart disease (like ventricular septal defect), interstitial pulmonary fibrosis.

Non-secondary pulmonary hypertension



  • Some people consider PCH and PVOD to the be same thing.[2]
    • Both have a poor prognosis.
    • Clinically they present the same way.
  • PVOD is based on case reports - it is extremely rare.[3]

Primary pulmonary hypertension

  • AKA pulmonary plexogenic arteriopathy.[4]


  • Familial PPH may be associated with BMPR2 mutations.[5]


  • Like chronic pulmonary hypertension due to congenital heart disease but without the congenital heart disease.[4]
    • Classified by Heath-Edwards classification (see below) into six grades.


Pulmonary veno-occlusive disease

  • Abbreviated PVOD.





  • Thrombosis - small veins & venules, particularily at the interlobular septae.
  • Associated with mild homogenous peripheral interstitial fibrosis.

DDx: chronic interstitial pneumonia.

Pulmonary capillary hemangiomatosis

  • Abbreviated PCH.


  • First reported in 1978 by Wagenvoort et al..[7]



  • Proliferating and invasive capillaries.[8]
  • Demonstrated by CD34 immunostaining.[2]
  • Dilated capillaries[9][10] - key feature.


  • Passive congestion (PC).
    • Differentiated by fact that PCH has multiple channels in alveolar wall (PC has only one).

Chronic pulmonary hypertension due to congenital heart disease

  • Graded using the Heath-Edwards system.[11]
  • A reason for open lung biopsy in children.[12]

Heath-Edwards classification


  • Six grades - based on intimal reaction and media of arteries and arterioles:
    • Grade 1:
      • Intima: no intimal reaction.
      • Media: hypertrophied.
    • Grade 2:
      • Intima: cellular intimal reaction.
      • Media: hypertrophied.
    • Grade 3:
      • Intima: fibrous & fibroelastic reaction + cellular intimal reaction.
      • Media: hypertrophy +/- generalized dilation.
    • Grade 4:
      • Intima: "plexiform lesions" + fibrous & fibroelastic reaction, + cellular intimal reaction.
          • Plexiform lesions = multiple channels that are dilated, associated with loss of elastic laminae; thought to arise at branch points due to aberrant WSS.[13]
      • Media: generalized dilation +/- local "dilation lesions".
      • Micrographs: Plexiform lesions (ucsf.edu), Plexiform lesions (pvrireview.org).
    • Grade 5:
      • Intima: as in Grade 4.
      • Media: generalized dilation + local "dilation lesions" + pulmonary hemosiderosis.
    • Grade 6:
      • Intima: as in Grade 4.
      • Media: generalized dilation + local "dilation lesions" + pulmonary hemosiderosis + necrotizing arteritis.


  • Bolded text - defining feature.

See also


  1. Bush A (December 2000). "Pulmonary hypertensive diseases". Paediatr Respir Rev 1 (4): 361-7. doi:10.1053/prrv.2000.0077. PMID 16263465.
  2. 2.0 2.1 Lantuéjoul S, Sheppard MN, Corrin B, Burke MM, Nicholson AG (July 2006). "Pulmonary veno-occlusive disease and pulmonary capillary hemangiomatosis: a clinicopathologic study of 35 cases". Am. J. Surg. Pathol. 30 (7): 850-7. doi:10.1097/01.pas.0000209834.69972.e5. PMID 16819327.
  3. Vevaina JR, Mark EJ (March 1988). "Thoracic hemangiomatosis masquerading as interstitial lung disease". Chest 93 (3): 657-9. PMID 3342678.
  4. 4.0 4.1 Lie JT, Silver MD. Diagnostic criteria of cardiovascular pathology: acquired diseases. ISBN 0-397-51630-4. PP.208-9.
  5. Online 'Mendelian Inheritance in Man' (OMIM) /600799
  6. 6.0 6.1 Leslie, Kevin O.; Wick, Mark R. (2004). Practical Pulmonary Pathology: A Diagnostic Approach (1st ed.). Churchill Livingstone. pp. 393-6. ISBN 978-0443066313.
  7. Wagenvoort CA, Beetstra A, Spijker J (November 1978). "Capillary haemangiomatosis of the lungs". Histopathology 2 (6): 401-6. PMID 730121.
  8. Tron V, Magee F, Wright JL, Colby T, Churg A (November 1986). "Pulmonary capillary hemangiomatosis". Hum. Pathol. 17 (11): 1144-50. PMID 3770733.
  9. MC August 2009.
  10. Leslie, Kevin O.; Wick, Mark R. (2004). Practical Pulmonary Pathology: A Diagnostic Approach (1st ed.). Churchill Livingstone. pp. 396-7. ISBN 978-0443066313.
  11. 11.0 11.1 HEATH D, EDWARDS JE (October 1958). "The pathology of hypertensive pulmonary vascular disease; a description of six grades of structural changes in the pulmonary arteries with special reference to congenital cardiac septal defects". Circulation 18 (4 Part 1): 533-47. PMID 13573570.
  12. Jaklitsch MT, Linden BC, Braunlin EA, Bolman RM, Foker JE (June 2001). "Open-lung biopsy guides therapy in children". Ann. Thorac. Surg. 71 (6): 1779-85. PMID 11426747.
  13. http://pathhsw5m54.ucsf.edu/overview/vessels.html