Difference between revisions of "Pulmonary hypertension"

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'''Pulmonary hypertension''' is bad stuff that arises from [[heart]] problems and an assortment of weird stuff.
[[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==
==General classification==
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===Non-secondary pulmonary hypertension===
===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>
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.
*[[Primary pulmonary hypertension]].
*Pulmonary embolic disease (thromboembolism, and non-thrombotic embolism).
*[[pulmonary embolism|Pulmonary embolic disease]] (thromboembolism, and non-thrombotic embolism).
*Pulmonary capillary haemangiomatosis (PCH).
*[[Pulmonary capillary haemangiomatosis]] (PCH).
*Pulmonary veno-occlusive disease (PVOD).  
*[[Pulmonary veno-occlusive disease]] (PVOD).  


Notes:
Notes:
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==Primary pulmonary hypertension==
==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>
*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>
*Like chronic pulmonary hypertension due to congenital heart disease but ''without'' the congenital heart disease.<ref name=dccpad/>
 
===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.
**Classified by ''Heath-Edwards classification'' (see below) into six grades.


==Pulmonary veno-occlusive disease (PVOD)==
====Images====
Features:<ref>{{Ref PPP|393-6}}</ref>
<gallery>
*Clinical - gradual dyspnea +/- non-productive cough, +/- clubbing.
Image:Angiomatoid_(plexiform)_and_dilatation_lesions_(4348914566).jpg | Plexiform lesion of the lung. (WC/Rosen)
*Thrombosis - small veins & venules, particularily at the interlobular septae.
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.
*Associated with mild homogenous peripheral interstitial fibrosis.


DDx: chronic interstitial pneumonia.
DDx: chronic interstitial pneumonia.


==Pulmonary capillary hemangiomatosis (PCH)==
==Pulmonary capillary hemangiomatosis==
General:
*Abbreviated ''PCH''.
*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>
 
===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:
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>
*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>
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**Grade 4:  
**Grade 4:  
***Intima: '''"plexiform lesions"''' + fibrous & fibroelastic reaction, + cellular intimal reaction.  
***Intima: '''"plexiform lesions"''' + fibrous & fibroelastic reaction, + cellular intimal reaction.  
*****Plexiform lesions = multiple channels that are dilated, assoc. with loss of elastic laminae; thought to arise at branch points due to aberant WSS.<ref>[http://pathhsw5m54.ucsf.edu/overview/vessels.html http://pathhsw5m54.ucsf.edu/overview/vessels.html]</ref>
*****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"'''.
***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)].
***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)].
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Notes:  
Notes:  
*'''Bolded''' text - defining feature.
*'''Bolded''' text - defining feature.
*It is discussed here: [http://www.pathology.or.kr/studygroup/cardiopulmonary/lecture/lenote/hka.htm http://www.pathology.or.kr/studygroup/cardiopulmonary/lecture/lenote/hka.htm].


==See also==
==See also==
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*[[Congenital heart disease]].
*[[Congenital heart disease]].
*[[Heart]].
*[[Heart]].
*[[Hypertension]].


==References==
==References==
Michael
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