Difference between revisions of "Pulmonary hypertension"

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#redirect [[medical lung diseases]]
[[Image:Angiomatoid (plexiform) and dilatation lesions (4348913976).jpg|thumb|right|300px|Plexiform lesion of the lung, a finding of pulmonary hypertension. (WC/Rosen)]]
'''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:<ref name=pmid16263465>{{cite journal |author=Bush A |title=Pulmonary hypertensive diseases |journal=Paediatr Respir Rev |volume=1 |issue=4 |pages=361-7 |year=2000 |month=December |pmid=16263465 |doi=10.1053/prrv.2000.0077 |url=}}</ref>
*[[Primary pulmonary hypertension]].
*[[pulmonary embolism|Pulmonary embolic disease]] (thromboembolism, and non-thrombotic embolism).
*[[Pulmonary capillary haemangiomatosis]] (PCH).
*[[Pulmonary veno-occlusive disease]] (PVOD).
 
Notes:
*Some people consider PCH and PVOD to the be same thing.<ref name=pmid16819327>{{cite journal |author=Lantuéjoul S, Sheppard MN, Corrin B, Burke MM, Nicholson AG |title=Pulmonary veno-occlusive disease and pulmonary capillary hemangiomatosis: a clinicopathologic study of 35 cases |journal=Am. J. Surg. Pathol. |volume=30 |issue=7 |pages=850-7 |year=2006 |month=July |pmid=16819327 |doi=10.1097/01.pas.0000209834.69972.e5 |url=}}</ref>
**Both have a poor prognosis.
**Clinically they present the same way.
*PVOD is based on case reports - it is extremely rare.<ref name=pmid3342678>{{cite journal |author=Vevaina JR, Mark EJ |title=Thoracic hemangiomatosis masquerading as interstitial lung disease |journal=Chest |volume=93 |issue=3 |pages=657-9 |year=1988 |month=March |pmid=3342678 |doi= |url=}}</ref>
 
==Primary pulmonary hypertension==
*AKA ''pulmonary plexogenic arteriopathy''.<ref name=dccpad>Lie JT, Silver MD. Diagnostic criteria of cardiovascular pathology: acquired diseases. ISBN 0-397-51630-4. PP.208-9.</ref>
 
===General===
*Familial PPH may be associated with BMPR2 mutations.<ref name=omim600799>{{OMIM|/600799}}</ref>
 
===Microscopic===
*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.
 
====Images====
<gallery>
Image:Angiomatoid_(plexiform)_and_dilatation_lesions_(4348914566).jpg | Plexiform lesion of the lung. (WC/Rosen)
Image:Angiomatoid_(plexiform)_and_dilatation_lesions_(4348913976).jpg | Plexiform lesion of the lung. (WC/Rosen)
Image:Angiomatoid (plexiform) and dilatation lesions (4348915198).jpg| Plexiform lesion of the lung. (WC/Rosen)
</gallery>
 
==Pulmonary veno-occlusive disease==
*Abbreviated ''PVOD''.
 
===General===
Clinical:<ref name=PPP393-6>{{Ref PPP|393-6}}</ref>
*Gradual [[dyspnea]].
*+/-Non-productive cough.
*+/-[[Clubbing]].
 
===Microscopic===
Features:<ref name=PPP393-6>{{Ref PPP|393-6}}</ref>
*[[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.''.<ref name=pmid730121>{{cite journal |author=Wagenvoort CA, Beetstra A, Spijker J |title=Capillary haemangiomatosis of the lungs |journal=Histopathology |volume=2 |issue=6 |pages=401-6 |year=1978 |month=November |pmid=730121 |doi= |url=}}</ref>
 
===Microscopic===
Features:
*Proliferating and invasive capillaries.<ref name=pmid3770733>{{cite journal |author=Tron V, Magee F, Wright JL, Colby T, Churg A |title=Pulmonary capillary hemangiomatosis |journal=Hum. Pathol. |volume=17 |issue=11 |pages=1144-50 |year=1986 |month=November |pmid=3770733 |doi= |url=}}</ref>
*Demonstrated by CD34 immunostaining.<ref name=pmid16819327/>
*'''Dilated capillaries'''<ref>MC August 2009.</ref><ref>{{Ref PPP|396-7}}</ref> - 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''.<ref name=pmid13573570>{{cite journal |author=HEATH D, EDWARDS JE |title=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 |journal=Circulation |volume=18 |issue=4 Part 1 |pages=533-47 |year=1958 |month=October |pmid=13573570 |doi= |url=}}</ref>
*A reason for open lung biopsy in children.<ref name=pmid11426747>{{cite journal |author=Jaklitsch MT, Linden BC, Braunlin EA, Bolman RM, Foker JE |title=Open-lung biopsy guides therapy in children |journal=Ann. Thorac. Surg. |volume=71 |issue=6 |pages=1779-85 |year=2001 |month=June |pmid=11426747 |doi= |url=}}</ref>
 
===Heath-Edwards classification===
Definition:<ref name=pmid13573570>{{cite journal |author=HEATH D, EDWARDS JE |title=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 |journal=Circulation |volume=18 |issue=4 Part 1 |pages=533-47 |year=1958 |month=October |pmid=13573570 |doi= |url=}}</ref>
*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.<ref>[http://pathhsw5m54.ucsf.edu/overview/vessels.html http://pathhsw5m54.ucsf.edu/overview/vessels.html]</ref>
***Media: generalized dilation +/- '''local "dilation lesions"'''.
***Micrographs: [http://pathhsw5m54.ucsf.edu/overview/vessels.html Plexiform lesions (ucsf.edu)], [http://www.pvrireview.org/viewimage.asp?img=PVRIReview_2009_1_1_34_44882_u6.jpg 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==
*[[Medical lung diseases]].
*[[Congenital heart disease]].
*[[Heart]].
*[[Hypertension]].
 
==References==
{{reflist|2}}
 
[[Category:Pulmonary pathology]]
[[Category:Cardiovascular pathology]]

Latest revision as of 18:13, 31 January 2022

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

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

  • Plexiform lesion of the lung. (WC/Rosen)

  • Plexiform lesion of the lung. (WC/Rosen)

  • Plexiform lesion of the lung. (WC/Rosen)

Pulmonary veno-occlusive disease

  • Abbreviated PVOD.

General

Clinical:[6]

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
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