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#redirect [[medical lung diseases]]
'''Pulmonary hypertension''' is bad stuff that arises from [[heart]] problems and an assortment of weird stuff.
 
==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 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>
*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.
 
===Pulmonary veno-occlusive disease (PVOD)===
Features:<ref>PPP PP.393-6.</ref>
*Clinical - gradual dyspnea +/- non-productive cough, +/- clubbing.
*Thrombosis - small veins & venules, particularily at the interlobular septae.
*Associated with mild homogenous peripheral interstitial fibrosis.
 
DDx: chronic interstitial pneumonia.
 
===Pulmonary capillary hemangiomatosis (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>
 
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>PPP PP.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, 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>
***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.
*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==
*[[Medical lung diseases]].
*[[Heart]].
 
==References==
{{reflist|2}}
 
[[Category:Lung pathology]]
Michael
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