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Difference between revisions of "Pulmonary hypertension"
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'''Pulmonary hypertension''' is bad stuff that arises from [[heart]] problems | [[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 embolism|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 | ====Images==== | ||
<gallery> | |||
* | 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 | ==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, | *****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. | ||
==See also== | ==See also== | ||
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*[[Congenital heart disease]]. | *[[Congenital heart disease]]. | ||
*[[Heart]]. | *[[Heart]]. | ||
*[[Hypertension]]. | |||
==References== | ==References== | ||