Pulmonary hypertension
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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]
- 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.[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)
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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
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).
- Intima: "plexiform lesions" + fibrous & fibroelastic reaction, + cellular intimal reaction.
- 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.
- Grade 1:
Notes:
- Bolded text - defining feature.
See also
References
- ↑ Bush A (December 2000). "Pulmonary hypertensive diseases". Paediatr Respir Rev 1 (4): 361-7. doi:10.1053/prrv.2000.0077. PMID 16263465.
- ↑ 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.
- ↑ Vevaina JR, Mark EJ (March 1988). "Thoracic hemangiomatosis masquerading as interstitial lung disease". Chest 93 (3): 657-9. PMID 3342678.
- ↑ 4.0 4.1 Lie JT, Silver MD. Diagnostic criteria of cardiovascular pathology: acquired diseases. ISBN 0-397-51630-4. PP.208-9.
- ↑ Online 'Mendelian Inheritance in Man' (OMIM) /600799
- ↑ 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.
- ↑ Wagenvoort CA, Beetstra A, Spijker J (November 1978). "Capillary haemangiomatosis of the lungs". Histopathology 2 (6): 401-6. PMID 730121.
- ↑ Tron V, Magee F, Wright JL, Colby T, Churg A (November 1986). "Pulmonary capillary hemangiomatosis". Hum. Pathol. 17 (11): 1144-50. PMID 3770733.
- ↑ MC August 2009.
- ↑ Leslie, Kevin O.; Wick, Mark R. (2004). Practical Pulmonary Pathology: A Diagnostic Approach (1st ed.). Churchill Livingstone. pp. 396-7. ISBN 978-0443066313.
- ↑ 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.
- ↑ 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.
- ↑ http://pathhsw5m54.ucsf.edu/overview/vessels.html