Difference between revisions of "Pulmonary hypertension"

From Libre Pathology
Jump to navigation Jump to search
Line 29: Line 29:
*Like chronic pulmonary hypertension due to [[congenital heart disease]] but ''without'' the congenital heart disease.<ref name=dccpad/>
*Like chronic pulmonary hypertension due to [[congenital heart disease]] but ''without'' the congenital heart disease.<ref name=dccpad/>
**Classified by ''Heath-Edwards classification'' (see below) into six grades.
**Classified by ''Heath-Edwards classification'' (see below) into six grades.
====Images====
<gallery>
Image:Angiomatoid_(plexiform)_and_dilatation_lesions_(4348913976).jpg | Plexiform lesions of the lung. (WC/Rosen)
Image:Angiomatoid (plexiform) and dilatation lesions (4348915198).jpg| Plexiform lesions of the lung. (WC/Rosen)
</gallery>


==Pulmonary veno-occlusive disease==
==Pulmonary veno-occlusive disease==

Revision as of 07:28, 17 November 2014

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

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

Causes:[1]

Notes:

  • 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]

General

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

Microscopic

  • 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.

Images

Pulmonary veno-occlusive disease

  • Abbreviated PVOD.

General

Clinical:[6]

  • Gradual dyspnea.
  • +/-Non-productive cough.
  • +/- Clubbing.

Microscopic

Features:[6]

  • 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.

General

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

Microscopic

Features:

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

DDx:

  • 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

Definition:[11]

  • 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.

Notes:

  • Bolded text - defining feature.

See also

References

  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