Pulmonary hypertension classification: Difference between revisions

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Latest revision as of 13:34, 9 June 2021

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1], Richard Channick, M.D.; Assistant Editor(s)-in-Chief: Ralph Matar; Lisa Prior, Ann Slater, R.N.; Rim Halaby, M.D. [2]; José Eduardo Riceto Loyola Junior, M.D.[3]

Overview

Pulmonary hypertension may be classified according to the mechanism leading to its development into 5 groups: pulmonary arterial hypertension, pulmonary hypertension due to left heart disease, pulmonary hypertension due to chronic lung diseases and/or hypoxia, and pulmonary hypertension due to embolic disease, and miscellaneous causes.

Classification

Pulmonary hypertension was first classified into primary and secondary in 1973 during the World Health Organization (WHO) meeting on PH in Geneva, Switzerland.^[1]
Pulmonary hypertension can be classified following different methods such as using the WHO clinical criteria, the hemodynamic findings, and the histopathological findings. The most common method of classification is using the disease mechanism, established by the World Health Organization, which is discussed below in detail.

WHO - Clinical Classification

Pulmonary hypertension was first classified into primary and secondary in 1973 during the World Health Organization (WHO) meeting on PH in Geneva, Switzerland.^[1]
The classification of the disease has been progressively updated since then and the latest version was defined in 2018, during the 6th World Symposium on Pulmonary Hypertension.
It is currently used by the U.S. Food and Drug Administration (FDA) and the European Medicines Agency (EMA) for the labeling of new drugs approved for the treatment of pulmonary hypertension.

The latest classification method categorizes pulmonary hypertension into 5 groups:
- Group I - Pulmonary arterial hypertension
- Group II - Pulmonary hypertension due to left heart disease
- Group III - Pulmonary hypertension due to chronic lung diseases and/or hypoxia
- Group IV - Pulmonary hypertension due to embolic disease
- Group V - Miscellaneous causes (e.g., sarcoidosis, lymphatic obstruction)

WHO Classification

Shown below is a table with the detailed classification of pulmonary hypertension.^[2]

Abbreviations: BMPR, bone morphogenic protein receptor type II; CAV1, caveolin-1; ENG, endoglin; HIV, human immunodeficiency virus.

Group 1. Pulmonary arterial hypertension (PAH)

1.1. Idiopathic PAH

1.2. Heritable PAH

1.2.1 BMPR2
1.2.2 ALK-1, ENG, SMAD9, CAV1, KCNK3
1.2.3 Unknown

1.3 Drug and toxin-induced

Definite (an epidemic or large multicenter epidemiological studies demonstrating an association between a drug and PAH)

Likely (a single case-control study demonstrating an association or a multiple-case series)

Possible (drugs with similar mechanisms of action as those in the definite or likely category but which have not yet been studied)

Unlikely (one in which a drug has been studied in epidemiological studies and an association with PAH has not been demonstrated)

1.4 Associated with:

1.4.1 Connective tissue disease
1.4.2 HIV infection
1.4.3 Portal hypertension
1.4.4 Congenital heart diseases
1.4.5 Schistosomiasis

1’ Pulmonary veno-occlusive disease (PVOD) and/or pulmonary capillary hemangiomatosis (PCH)

1’’ Persistent pulmonary hypertension of the newborn (PPHN)

Group 2. Pulmonary hypertension due to left heart disease

2.1 Left ventricular systolic dysfunction

2.2 Left ventricular diastolic dysfunction

2.3 Valvular disease

2.4 Congenital/acquired left heart inflow/outflow tract obstruction and congenital cardiomyopathies

Group 3. Pulmonary hypertension due to lung diseases and/or hypoxia

3.1 Chronic obstructive pulmonary disease

3.2 Interstitial lung disease

3.3 Other pulmonary diseases with mixed restrictive and obstructive pattern

3.4 Sleep-disordered breathing

3.5 Alveolar hypoventilation disorders

3.6 Chronic exposure to high altitude

3.7 Developmental lung diseases

Group 4. Chronic thromboembolic pulmonary hypertension (CTEPH)

Group 5. Pulmonary hypertension with unclear multifactorial mechanisms

5.1 Hematologic disorders: chronic hemolytic anemia, myeloproliferative disorders, splenectomy

5.2 Systemic disorders: sarcoidosis, pulmonary histiocytosis, lymphangioleiomyomatosis

5.3 Metabolic disorders: glycogen storage disease, Gaucher disease, thyroid disorders

5.4 Others: tumor obstruction, fibrosing mediastinitis, chronic renal failure, segmental PH

Classification Based on Hemodynamical Findings

Abbreviations: PAP: Pulmonary artery pressure; PWP: pulmonary wedge pressure

Type of pulmonary hypertension	Possible clinical class	Mean PAP	PWP
Pre-capillary	Class I Class III Class IV Class V	≥ 25 mmHg	≤ 15 mmHg
Post-capillary	Class II	≥ 25 mmHg	> 15 mmHg

Classification Based on Histopathological Findings

PH is a pathological condition present in different disease states that share similar clinical manifestations and some common histopathological features. Shown below is a table that summarizes the classification of PH based on histopathology findings.^[3]

Class	Histopathological findings^[3]
Pulmonary arteriopathy	Constrictive lesions in pulmonary arteries: Medial hypertrophy Intimal thickening Adventitial thickening Complex lesions in pulmonary arteries: Plexiform lesions Dilatation lesions Arteritis
Pulmonary arteriopathy with venous-venular changes	Changes similar to pulmonary arteriopathy PLUS Changes in venules and veins
Pulmonary occlusive venopathy (with or without arteriopathy)	Changes in venules and veins: Diffuse fibrotic occlusion Intimal thickening Medial thickening Adventitial thickening Changes in the capillaries: Dilatation Congestion Changes in the interstitium Edema Fibrosis Hemosiderosis
Pulmonary microvasculopathy (with or without arteriopathy and/on venopathy)	Changes in the capillaries: Localized capillary proliferation Changes in the interstitium Edema Fibrosis Hemosiderosis
Unclassified	Non specific changes

References

↑ ^1.0 ^1.1 Hatano S, Strasser T. Primary Pulmonary Hypertension. Report on a WHO Meeting. October 15–17, 1973, Geneva: World Health Organization, 1975.
↑ Simonneau G, Gatzoulis MA, Adatia I, Celermajer D, Denton C, Ghofrani A; et al. (2013). "Updated clinical classification of pulmonary hypertension". J Am Coll Cardiol. 62 (25 Suppl): D34–41. doi:10.1016/j.jacc.2013.10.029. PMID 24355639.
↑ ^3.0 ^3.1 Pietra GG, Capron F, Stewart S, Leone O, Humbert M, Robbins IM; et al. (2004). "Pathologic assessment of vasculopathies in pulmonary hypertension". J Am Coll Cardiol. 43 (12 Suppl S): 25S–32S. doi:10.1016/j.jacc.2004.02.033. PMID 15194175.

Template:WikiDoc Sources

[WHO1973-1] 1.0 ^1.1 Hatano S, Strasser T. Primary Pulmonary Hypertension. Report on a WHO Meeting. October 15–17, 1973, Geneva: World Health Organization, 1975.

[pmid24355639-2] Simonneau G, Gatzoulis MA, Adatia I, Celermajer D, Denton C, Ghofrani A; et al. (2013). "Updated clinical classification of pulmonary hypertension". J Am Coll Cardiol. 62 (25 Suppl): D34–41. doi:10.1016/j.jacc.2013.10.029. PMID 24355639.

[pmid15194175-3] 3.0 ^3.1 Pietra GG, Capron F, Stewart S, Leone O, Humbert M, Robbins IM; et al. (2004). "Pathologic assessment of vasculopathies in pulmonary hypertension". J Am Coll Cardiol. 43 (12 Suppl S): 25S–32S. doi:10.1016/j.jacc.2004.02.033. PMID 15194175.

[1]

[2]

[3]

@@ Line 1: / Line 1: @@
 __NOTOC__
 {{Pulmonary hypertension}}
-{{CMG}}, Richard Channick, M.D.; '''Assistant Editor(s)-in-Chief:''' [[User:Ralph Matar|Ralph Matar]], [[User:Lisa Prior|Lisa Prior]], [[Ann Slater|Ann Slater, R.N.]]
+{{CMG}}, Richard Channick, M.D.; '''Assistant Editor(s)-in-Chief:''' [[User:Ralph Matar|Ralph Matar]]; Lisa Prior, [[Ann Slater|Ann Slater, R.N.]]; {{Rim}}; {{Jose}}
 ==Overview==
+[[Pulmonary hypertension]] may be classified according to the mechanism leading to its development into 5 groups: [[pulmonary arterial hypertension]], [[pulmonary hypertension due to left heart disease]], [[pulmonary hypertension due to chronic lung diseases and/or hypoxia]], and [[pulmonary hypertension due to embolic disease]], and [[miscellaneous]] causes.
 ==Classification==
-===Dana Point clinical classification===
+*[[Pulmonary hypertension]] was first classified into [[primary]] and [[secondary]] in 1973 during the [[World Health Organization]] (WHO) meeting on PH in Geneva, Switzerland.<ref name="WHO1973">Hatano S, Strasser T. Primary Pulmonary Hypertension. Report on a WHO Meeting. October 15–17, 1973, Geneva: World Health Organization, 1975.</ref>
-Updated Clinical Classification of Pulmonary Hypertension(Dana Point, 2008) <ref>Updated Clinical Classification of Pulmonary Hypertension doi:10.1016/j.jacc.2009.04.012 J. Am. Coll. Cardiol. 2009;54;S43-S54 Nakanishi, and Rogério Souza Gladwin, Zhi-Cheng Jing, Michael J. Krowka, David Langleben, NorifumiMarion Delcroix, Christopher P. Denton, C. Gregory Elliott, Sean P. Gaine, Mark T.Gérald Simonneau, Ivan M. Robbins, Maurice Beghetti, Richard N. Channick,</ref>
+*Pulmonary hypertension can be classified following different methods such as using the [[WHO]] clinical criteria, the hemodynamic findings, and the histopathological findings. The most common method of classification is using the disease mechanism, established by the World Health Organization, which is discussed below in detail.
+==WHO - Clinical Classification==
+* [[Pulmonary hypertension]] was first classified into [[primary]] and [[secondary]] in 1973 during the [[World Health Organization]] (WHO) meeting on PH in Geneva, Switzerland.<ref name="WHO1973">Hatano S, Strasser T. Primary Pulmonary Hypertension. Report on a WHO Meeting. October 15–17, 1973, Geneva: World Health Organization, 1975.</ref>
+* The classification of the disease has been progressively updated since then and the latest version was defined in 2018, during the 6th World Symposium on Pulmonary Hypertension.
+* It is currently used by the U.S. Food and Drug Administration (FDA) and the European Medicines Agency (EMA) for the labeling of new drugs approved for the treatment of [[pulmonary hypertension]].
-*'''1. Pulmonary arterial hypertension (PAH)'''
+*The latest classification method categorizes [[pulmonary hypertension]] into 5 groups:
-**1.1. Idiopathic PAH
+** Group I - Pulmonary arterial hypertension
-**1.2. [[Heritable]]
+** Group II - Pulmonary hypertension due to left heart disease
-***1.2.1. BMPR2
+** Group III - Pulmonary hypertension due to chronic lung diseases and/or hypoxia
-***1.2.2. ALK1, endoglin (with or without [[hereditary hemorrhagic telangiectasia]])
+** Group IV - Pulmonary hypertension due to embolic disease
-***1.2.3. Unknown
+** Group V - Miscellaneous causes (e.g., sarcoidosis, lymphatic obstruction)
-**1.3. Drug- and toxin-induced
-**1.4. Associated with
-***1.4.1. [[Connective tissue diseases]]
-***1.4.2. [[HIV]] infection
-***1.4.3. [[Portal hypertension]]
-***1.4.4. [[Congenital heart diseases]]
-***1.4.5. [[Schistosomiasis]]
-***1.4.6. Chronic [[hemolytic anemia]]
-**1.5 Persistent pulmonary hypertension of the newborn
-'''1'. Pulmonary veno-occlusive disease (PVOD) and/or pulmonary capillary
-hemangiomatosis (PCH)'''
-*'''2. Pulmonary hypertension owing to left heart disease'''
-**2.1. [[Systolic dysfunction]]
-**2.2. [[Diastolic dysfunction]]
-**2.3. [[Valvular disease]]
-*'''3. Pulmonary hypertension owing to [[lung diseases]] and/or [[hypoxia]]'''
-**3.1. [[Chronic obstructive pulmonary disease]]
-**3.2. [[Interstitial lung disease]]
-**3.3. Other pulmonary diseases with mixed restrictive and obstructive pattern
-**3.4. Sleep-disordered breathing
-**3.5. Alveolar hypoventilation disorders
-**3.6. Chronic exposure to high altitude
-**3.7. Developmental abnormalities
-*'''4. Chronic thromboembolic pulmonary hypertension (CTEPH)'''
-*'''5. Pulmonary hypertension with unclear multifactorial mechanisms'''
-**5.1. Hematologic disorders: [[myeloproliferative disorders]], [[splenectomy]]
-**5.2. Systemic disorders: [[sarcoidosis]], pulmonary [[Langerhans cell histiocytosis]]: [[lymphangioleiomyomatosis]], [[neurofibromatosis]], [[vasculitis]]
-**5.3. Metabolic disorders: [[glycogen storage disease]], [[Gaucher disease]], thyroid disorders
-**5.4. Others: tumoral obstruction, [[fibrosing mediastinitis]], chronic [[renal failure]] on [[dialysis]]
-===The Venice 2003 Revised Classification system===
+===WHO Classification===
+Shown below is a table with the detailed classification of [[pulmonary hypertension]].<ref name="pmid24355639">{{cite journal| author=Simonneau G, Gatzoulis MA, Adatia I, Celermajer D, Denton C, Ghofrani A et al.| title=Updated clinical classification of pulmonary hypertension. | journal=J Am Coll Cardiol | year= 2013 | volume= 62 | issue= 25 Suppl | pages= D34-41 | pmid=24355639 | doi=10.1016/j.jacc.2013.10.029 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24355639  }} </ref>
-In 2003, the 3rd World Symposium on Pulmonary Arterial Hypertension was convened in Venice to modify the classification based on the new understanding of disease mechanisms.  The revised system developed by this group provides the current framework for understanding pulmonary hypertension.
+<span style="font-size: 80%;">''Abbreviations:'' BMPR, bone morphogenic protein receptor type II; CAV1, caveolin-1; ENG, endoglin; HIV, human immunodeficiency virus.</span>
-The system includes several improvements over the former 1998 Evian Classification system.  Risk factor descriptions were updated, and the classification of congenital systemic-to pulmonary shunts was revised.  A new classification of genetic factors in PH was recommended, but not implemented because available data were judged to be inadequate.
+{| style="cellpadding=0; cellspacing= 0; width: 600px;"
+|-
+| style="padding: 0 5px; font-size: 100%; background: #4682B4; color: #FFFFFF" align="left" |'''Group 1. Pulmonary arterial hypertension (PAH)'''
+|-
+| style="padding: 0 5px; font-size: 100%; background: #F5F5F5; width: 70%" align="left" |'''1.1. Idiopathic PAH'''
+|-
+| style="padding: 0 5px; font-size: 100%; background: #F5F5F5; width: 70%" align="left" |'''1.2. Heritable PAH''' <br>
+.2.1 [[BMPR2]] <br>
+.2.2 [[ALK-1]], [[Endoglin|ENG]], [[SMAD9]], [[CAV1]], [[KCNK3]] <br>
+.2.3 Unknown
+|-
+| style="padding: 0 5px; font-size: 100%; background: #F5F5F5; width: 70%" align="left" |'''1.3 Drug and toxin-induced''' <br>
+''Definite'' (an epidemic or large multicenter epidemiological studies demonstrating an association between a drug and PAH) <br>
+*[[Aminorex]]
+*[[Fenfluramine]]
+*[[Dexfenfluramine]]
+*Toxic [[rapeseed oil]]
+*[[Benfluorex]]
+*[[SSRI]]s <br>
+''Likely'' (a single case-control study demonstrating an association or a multiple-case series) <br>
+*[[Amphetamine]]s
+*L-[[Tryptophan]]
+*[[Methamphetamine]]s
+*[[Dasatinib]] <br>
+''Possible'' (drugs with similar mechanisms of action as those in the definite or likely category but which have not yet been studied) <br>
+*[[Cocaine]]
+*[[Phenylpropanolamine]]
+*[[St. John's wort|St. John's Wort]]
+*[[Chemotherapeutic agent]]s
+*[[Interferon]] α and β
+*[[Amphetamine]]-like drugs <br>
+''Unlikely'' (one in which a drug has been studied in epidemiological studies and an association with PAH has not been demonstrated) <br>
+*[[Oral contraceptive]]s
+*[[Estrogen]]
+*[[Cigarette smoking]]
+|-
+| style="padding: 0 5px; font-size: 100%; background: #F5F5F5; width: 70%" align="left" |'''1.4 Associated with:''' <br>
+.4.1 [[Connective tissue disease]] <br>
+.4.2 [[HIV infection]] <br>
+.4.3 [[Portal hypertension]] <br>
+.4.4 [[Congenital heart disease]]s <br>
+.4.5 [[Schistosomiasis]] <br>
+|-
+| style="padding: 0 5px; font-size: 100%; background: #4682B4; color: #FFFFFF" align="left" |'''1’ Pulmonary veno-occlusive disease (PVOD) and/or pulmonary capillary hemangiomatosis (PCH)'''
+|-
+| style="padding: 0 5px; font-size: 100%; background: #4682B4; color: #FFFFFF" align="left" |'''1’’ Persistent pulmonary hypertension of the newborn (PPHN)'''
+|-
+| style="padding: 0 5px; font-size: 100%; background: #4682B4; color: #FFFFFF" align="left" |'''Group 2. Pulmonary hypertension due to left heart disease'''
+|-
+| style="padding: 0 5px; font-size: 100%; background: #F5F5F5; width: 70%" align="left" |2.1 [[Left ventricular systolic dysfunction]]
+|-
+| style="padding: 0 5px; font-size: 100%; background: #F5F5F5; width: 70%" align="left" |2.2 [[diastolic dysfunction|Left ventricular diastolic dysfunction]]
+|-
+| style="padding: 0 5px; font-size: 100%; background: #F5F5F5; width: 70%" align="left" |2.3 [[Valvular disease]]
+|-
+| style="padding: 0 5px; font-size: 100%; background: #F5F5F5; width: 70%" align="left" |2.4 [[Congenital heart disease|Congenital]]/acquired left heart inflow/outflow tract obstruction and congenital [[cardiomyopathies]]
+|-
+| style="padding: 0 5px; font-size: 100%; background: #4682B4; color: #FFFFFF" align="left" |'''Group 3. Pulmonary hypertension due to lung diseases and/or hypoxia'''
+|-
+| style="padding: 0 5px; font-size: 100%; background: #F5F5F5; width: 70%" align="left" |3.1 [[Chronic obstructive pulmonary disease]]
+|-
+| style="padding: 0 5px; font-size: 100%; background: #F5F5F5; width: 70%" align="left" |3.2 [[Interstitial lung disease]]
+|-
+| style="padding: 0 5px; font-size: 100%; background: #F5F5F5; width: 70%" align="left" |3.3 Other pulmonary diseases with mixed restrictive and obstructive pattern
+|-
+| style="padding: 0 5px; font-size: 100%; background: #F5F5F5; width: 70%" align="left" |3.4 Sleep-disordered breathing
+|-
+| style="padding: 0 5px; font-size: 100%; background: #F5F5F5; width: 70%" align="left" |3.5 Alveolar [[hypoventilation]] disorders
+|-
+| style="padding: 0 5px; font-size: 100%; background: #F5F5F5; width: 70%" align="left" |3.6 Chronic exposure to high altitude
+|-
+| style="padding: 0 5px; font-size: 100%; background: #F5F5F5; width: 70%" align="left" |3.7 Developmental lung diseases
+|-
+| style="padding: 0 5px; font-size: 100%; background: #4682B4; color: #FFFFFF" align="left" |'''Group 4. Chronic thromboembolic pulmonary hypertension (CTEPH)'''
+|-
+| style="padding: 0 5px; font-size: 100%; background: #4682B4; color: #FFFFFF" align="left" |'''Group 5. Pulmonary hypertension with unclear multifactorial mechanisms'''
+|-
+| style="padding: 0 5px; font-size: 100%; background: #F5F5F5; width: 70%" align="left" |5.1 Hematologic disorders: chronic [[hemolytic anemia]], [[myeloproliferative disorder]]s, [[splenectomy]]
+|-
+| style="padding: 0 5px; font-size: 100%; background: #F5F5F5; width: 70%" align="left" |5.2 Systemic disorders: [[sarcoidosis]], [[Langerhans cell histiocytosis|pulmonary histiocytosis]], [[lymphangioleiomyomatosis]]
+|-
+| style="padding: 0 5px; font-size: 100%; background: #F5F5F5; width: 70%" align="left" |5.3 Metabolic disorders: [[glycogen storage disease]], [[Gaucher disease]], [[thyroid]] disorders
+|-
+| style="padding: 0 5px; font-size: 100%; background: #F5F5F5; width: 70%" align="left" |5.4 Others: [[tumor]] obstruction, fibrosing [[mediastinitis]], [[chronic renal failure]], segmental PH
+|}
-The Venice 2003 Revised Classification system can be summarized as follows:<ref>Proceedings of the 3rd World Symposium on Pulmonary Arterial Hypertension. Venice, Italy, June 23-25, 2003. ''J Am Coll Cardiol'' 2004 Jun 16;43(12 Suppl S):1S-90S. PMID 15194171.</ref>
+==Classification Based on Hemodynamical Findings==
-*WHO Group I - Pulmonary arterial hypertension (PAH)
-*WHO Group II - Pulmonary hypertension associated with left heart disease
-*WHO Group III - Pulmonary hypertension associated with lung diseases and/or hypoxemia
-*WHO Group IV - Pulmonary hypertension due to chronic thrombotic and/or embolic disease
-*WHO Group V - Miscellaneous
-'''Venice Clinical Classification of Pulmonary Hypertension (2003)'''
+<span style="font-size: 80%;">'''Abbreviations:''' '''PAP:''' Pulmonary artery pressure; '''PWP:''' pulmonary wedge pressure </span>
-*'''1. Pulmonary arterial hypertension (PAH)'''
-**1.1. Idiopathic (IPAH)
-**1.2. Familial (FPAH)
-**1.3. Associated with (APAH)
-***1.3.1. [[Collagen vascular disease]]
-***1.3.2. Congenital systemic-to-pulmonary shunts
-***1.3.3. [[Portal hypertension]]
-***1.3.4. [[HIV]] infection
-***1.3.5. Drugs and toxins
-***1.3.6. Other ([[thyroid disorders]], [[glycogen storage disease]], [[Gaucher disease]], [[hereditary hemorrhagic telangiectasia]], [[hemoglobinopathies]], [[myeloproliferative disorders]], [[splenectomy]])
-**1.4. Associated with significant venous or capillary involvement
-***1.4.1. Pulmonary veno-occlusive disease (PVOD)
-***1.4.2. Pulmonary capillary hemangiomatosis (PCH)
-**1.5. Persistent pulmonary hypertension of the newborn
-*'''2. Pulmonary hypertension with left heart disease'''
-**2.1. Left-sided atrial or ventricular heart disease
-**2.2. Left-sided [[valvular heart disease]]
-*'''3. Pulmonary hypertension associated with lung diseases and/or hypoxemia'''
-**3.1. [[Chronic obstructive pulmonary disease]]
-**3.2. [[Interstitial lung disease]]
-**3.3. Sleep-disordered breathing
-**3.4. Alveolar hypoventilation disorders
-**3.5. Chronic exposure to high altitude
-**3.6. Developmental abnormalities
-*'''4. Pulmonary hypertension owing to chronic thrombotic and/or embolic disease'''
-**4.1. Thromboembolic obstruction of proximal pulmonary arteries
-**4.2. Thromboembolic obstruction of distal pulmonary arteries
-**4.3. Nonthrombotic [[pulmonary embolism]] ([[tumor]], [[parasites]], foreign material)
-*'''5. Miscellaneous'''
-[[Sarcoidosis]], [[histiocytosis X]], [[lymphangiomatosis]], compression of pulmonary [[vessels]] ([[adenopathy]], [[tumor]], [[fibrosing mediastinitis]]
-===Previous terminology===
+{| style="cellpadding=0; cellspacing= 0; width: 600px;"
+|-
+| style="padding: 0 5px; font-size: 100%; background: #4682B4; color: #FFFFFF" align="left" |'''Type of pulmonary hypertension''' || style="padding: 0 5px; font-size: 100%; background: #4682B4; color: #FFFFFF" align="left" |'''Possible clinical class''' || style="padding: 0 5px; font-size: 100%; background: #4682B4; color: #FFFFFF" align="left" |'''Mean PAP''' || style="padding: 0 5px; font-size: 100%; background: #4682B4; color: #FFFFFF" align="left" |'''PWP'''
+|-
+| style="padding: 0 5px; font-size: 100%; background: #F5F5F5;" align="left" |'''Pre-capillary''' || style="padding: 0 5px; font-size: 100%; background: #F5F5F5;" align="left" |Class I <br>Class III <br>Class IV <br>Class V || style="padding: 0 5px; font-size: 100%; background: #F5F5F5;" align="left" |≥ 25 mmHg || style="padding: 0 5px; font-size: 100%; background: #F5F5F5;" align="left" |≤ 15 mmHg
+|-
+| style="padding: 0 5px; font-size: 100%; background: #F5F5F5;" align="left" |'''Post-capillary''' || style="padding: 0 5px; font-size: 100%; background: #F5F5F5;" align="left" |Class II || style="padding: 0 5px; font-size: 100%; background: #F5F5F5;" align="left" |≥ 25 mmHg || style="padding: 0 5px; font-size: 100%; background: #F5F5F5;" align="left" |> 15 mmHg
+|}
-The terms primary and secondary pulmonary hypertension (PPH and SPH) were formerly used to classify the disease.  This led to the assumption that only the primary disease should be treated, and the secondary variety should be ignored in favor of treating only the underlying illness.  In fact all forms of pulmonary arterial hypertension are treatable. Unfortunately, this classification system still persists in the minds of many physicians, and probably leads to many patients with being denied treatment.  This approach to pulmonary arterial hypertension may also contribute to underdiagnosis.  It is estimated that there are about 100,000 patients with PAH in the US, but only 15-20,000 have been diagnosed.  Many others have been misdiagnosed as [[COPD]], [[asthma]], or [[congestive heart failure]].
-The term primary pulmonary hypertension (PPH) has now been replaced with idiopathic pulmonary arterial hypertension (IPAH) in much of the medical literature.  However, some physicians continue to use the older classification inappropriately.
+==Classification Based on Histopathological Findings==
+PH is a pathological condition present in different disease states that share similar clinical manifestations and some common histopathological features.  Shown below is a table that summarizes the classification of PH based on histopathology findings.<ref name="pmid15194175">{{cite journal| author=Pietra GG, Capron F, Stewart S, Leone O, Humbert M, Robbins IM et al.| title=Pathologic assessment of vasculopathies in pulmonary hypertension. | journal=J Am Coll Cardiol | year= 2004 | volume= 43 | issue= 12 Suppl S | pages= 25S-32S | pmid=15194175 | doi=10.1016/j.jacc.2004.02.033 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15194175  }} </ref>
+{| style="cellpadding=0; cellspacing= 0; width: 600px;"
+|-
+| style="padding: 0 5px; font-size: 100%; background: #4682B4; color: #FFFFFF" align="left" |'''Class''' || style="padding: 0 5px; font-size: 100%; background: #4682B4; color: #FFFFFF" align="left" |'''Histopathological findings'''<ref name="pmid15194175">{{cite journal| author=Pietra GG, Capron F, Stewart S, Leone O, Humbert M, Robbins IM et al.| title=Pathologic assessment of vasculopathies in pulmonary hypertension. | journal=J Am Coll Cardiol | year= 2004 | volume= 43 | issue= 12 Suppl S | pages= 25S-32S | pmid=15194175 | doi=10.1016/j.jacc.2004.02.033 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15194175  }} </ref>
+|-
+| style="padding: 0 5px; font-size: 100%; background: #F5F5F5;" align="left" |'''Pulmonary arteriopathy''' || style="padding: 0 5px; font-size: 100%; background: #F5F5F5;" align="left" |Constrictive lesions in pulmonary arteries:
+* Medial hypertrophy
+* Intimal thickening
+* Adventitial thickening
+Complex lesions in pulmonary arteries:
+* Plexiform lesions
+* Dilatation lesions
+* [[Arteritis]]
+|-
+| style="padding: 0 5px; font-size: 100%; background: #F5F5F5;" align="left" |'''Pulmonary arteriopathy with venous-venular changes''' || style="padding: 0 5px; font-size: 100%; background: #F5F5F5;" align="left" | Changes similar to pulmonary arteriopathy <br> PLUS<br> Changes in venules and veins
+|-
+| style="padding: 0 5px; font-size: 100%; background: #F5F5F5; width:50%" align="left" |'''Pulmonary occlusive venopathy''' <br> (with or without arteriopathy) || style="padding: 0 5px; font-size: 100%; background: #F5F5F5;" align="left" |Changes in venules and veins:
+* Diffuse fibrotic occlusion
+* Intimal thickening
+* Medial thickening
+* Adventitial thickening
+Changes in the capillaries:
+* Dilatation
+* Congestion
+Changes in the interstitium
+* [[Edema]]
+* [[Fibrosis]]
+* [[Hemosiderosis]]
+|-
+| style="padding: 0 5px; font-size: 100%; background: #F5F5F5;" align="left" |'''Pulmonary microvasculopathy''' <br> (with or without arteriopathy and/on venopathy)|| style="padding: 0 5px; font-size: 100%; background: #F5F5F5;" align="left" |Changes in the capillaries:
+* Localized capillary proliferation
+Changes in the interstitium
+* [[Edema]]
+* [[Fibrosis]]
+* [[Hemosiderosis]]
+|-
+| style="padding: 0 5px; font-size: 100%; background: #F5F5F5;" align="left" |'''Unclassified''' || style="padding: 0 5px; font-size: 100%; background: #F5F5F5;" align="left" | Non specific changes
+|}
 ==References==
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+[[Category:Medicine]]
 [[Category:Cardiology]]
 [[Category:Pulmonology]]
-[[Category:Disease]]
+[[Category:Emergency medicine]]
-[[Category:Mature chapter]]
+[[Category:Up-To-Date]]