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]]; Lisa Prior, [[Ann Slater|Ann Slater, R.N.]]; {{Rim}}
+{{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.
-Pulmonary hypertension (PH) has been previously classified as primary (currently known as idiopathic pulmonary arterial hypertension or IPAH) and secondary in 1973.<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>  In 1988, a clinical classification of PH into 5 groups has been developed during the 2nd World Symposium on Pulmonary Hypertension in Evian, France.<ref>Rich S, Rubin LJ, Abenhail L, et al. Executive summary from the World Symposium on Primary Pulmonary Hypertension 1998, Evian, France, September 6-10, 1998. Geneva: The World Health Organization.</ref> The clinical classification has been updated regularly as the understanding of PH expanded; nevertheless, the main scheme of classification into the 5 main groups remained intact.  The classification of PH has been last modified in 2013 during the 5th World Symposium on Pulmonary Hypertension in Nice, France.<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> The main 5 groups of PH include pulmonary arterial hypertension (Group 1), pulmonary hypertension due to left heart disease (Group 2), pulmonary hypertension due to chronic lung disease and/or [[hypoxia]] (Group 3), chronic [[VTE|thromboembolic]] pulmonary hypertension (Group 4), and pulmonary hypertension due to unclear multifactorial mechanisms (Group 5).<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>
 ==Classification==
-===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>
-PH 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>
+*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.
-The 2-group classification of PH was replaced by a clinical 5-group classification in the 2nd World Symposium on Pulmonary Hypertension in 1998 in Evian, France.<ref>Rich S, Rubin LJ, Abenhail L, et al. Executive summary from the World Symposium on Primary Pulmonary Hypertension 1998, Evian, France, September 6-10, 1998. Geneva: The World Health Organization.</ref> Since then, the clinical classification of PH was updated in the following meetings:
+==WHO - Clinical Classification==
-* The 3d World Symposium on Pulmonary Hypertension (2003) in Venice, Italy<ref name="pmid15194173">{{cite journal| author=Simonneau G, Galiè N, Rubin LJ, Langleben D, Seeger W, Domenighetti G et al.| title=Clinical classification of pulmonary hypertension. | journal=J Am Coll Cardiol | year= 2004 | volume= 43 | issue= 12 Suppl S | pages= 5S-12S | pmid=15194173 | doi=10.1016/j.jacc.2004.02.037 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15194173  }} </ref>
+* [[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 4th World Symposium on Pulmonary Hypertension (2008) in Dana Point, California, USA<ref name="pmid19555858">{{cite journal| author=Simonneau G, Robbins IM, Beghetti M, Channick RN, Delcroix M, Denton CP et al.| title=Updated clinical classification of pulmonary hypertension. | journal=J Am Coll Cardiol | year= 2009 | volume= 54 | issue= 1 Suppl | pages= S43-54 | pmid=19555858 | doi=10.1016/j.jacc.2009.04.012 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19555858  }} </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.
-* The 5th World Symposium on Pulmonary Hypertension (2013) in Nice, France<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>
+* 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 updated clinical classification has been adopted by the Guidelines Committee of the European Society of Cardiology (ESC), European Respiratory Society (ERS), and International Society of Heart and Lung Transplantation (ISHLT).  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 PH.
+*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)
-Shown below is a table summarizing the updated clinical classification of PH.<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>
+===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>
+<span style="font-size: 80%;">''Abbreviations:'' BMPR, bone morphogenic protein receptor type II; CAV1, caveolin-1; ENG, endoglin; HIV, human immunodeficiency virus.</span>
 {| 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: #4682B4; color: #FFFFFF" align="left" |'''Group 1. Pulmonary arterial hypertension (PAH)'''
 |-
-| style="padding: 0 5px; font-size: 100%; background: #F5F5F5; width: 70%" align=left colspan=2 |'''1.1. Idiopathic 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 colspan=2 |'''1.2. Heritable PAH''' <br>
+| 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 colspan=2 |'''1.3 Drug and toxin induced''' <br>
+| 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]] <br>
+*[[Aminorex]]
-*[[Fenfluramine]] <br>
+*[[Fenfluramine]]
-*[[Dexfenfluramine]] <br>
+*[[Dexfenfluramine]]
-*Toxic [[rapeseed oil]] <br>
+*Toxic [[rapeseed oil]]
-*[[Benfluorex]] <br>
+*[[Benfluorex]]
 *[[SSRI]]s <br>
 ''Likely'' (a single case-control study demonstrating an association or a multiple-case series) <br>
-*[[Amphetamine]]s <br>
+*[[Amphetamine]]s
-*L-[[Tryptophan]] <br>
+*L-[[Tryptophan]]
-*[[Methamphetamine]]s <br>
+*[[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]] <br>
+*[[Cocaine]]
-*[[Phenylpropanolamine]] <br>
+*[[Phenylpropanolamine]]
-*[[St. John's wort]] <br>
+*[[St. John's wort|St. John's Wort]]
-*[[Chemotherapeutic agent]]s <br>
+*[[Chemotherapeutic agent]]s
-*[[Interferon]] α and β <br>
+*[[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 <br>
+*[[Oral contraceptive]]s
-*[[Estrogen]] <br>
+*[[Estrogen]]
 *[[Cigarette smoking]]
 |-
-| style="padding: 0 5px; font-size: 100%; background: #F5F5F5; width: 70%" align=left colspan=2 |'''1.4 Associated with:''' <br>
+| 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>
@@ Line 63: / Line 70: @@
 .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’ 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: #4682B4; color: #FFFFFF" align=left |'''1’’ Persistent pulmonary hypertension of the newborn (PPHN)'''
+| 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: #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" |2.3 [[Valvular disease]]
 |-
-| style="padding: 0 5px; font-size: 100%; background: #F5F5F5; width: 70%" align=left colspan=2 |2.1 [[Left ventricular systolic dysfunction]]
+| 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: #F5F5F5; width: 70%" align=left colspan=2 |2.2 [[diastolic dysfunction|Left ventricular diastolic dysfunction]]
+| 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 colspan=2 |2.3 [[Valvular disease]]
+| 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 colspan=2 |2.4 [[Congenital heart disease|Congenital]]/acquired left heart inflow/outflow tract obstruction and congenital [[cardiomyopathies]]
+| 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: #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" |3.3 Other pulmonary diseases with mixed restrictive and obstructive pattern
 |-
-| style="padding: 0 5px; font-size: 100%; background: #F5F5F5; width: 70%" align=left colspan=2 |3.1 [[Chronic obstructive pulmonary disease]]
+| 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 colspan=2 |3.2 [[Interstitial lung disease]]
+| 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 colspan=2 |3.3 Other pulmonary diseases with mixed restrictive and obstructive pattern
+| style="padding: 0 5px; font-size: 100%; background: #F5F5F5; width: 70%" align="left" |3.6 Chronic exposure to high altitude
 |-
-| style="padding: 0 5px; font-size: 100%; background: #F5F5F5; width: 70%" align=left colspan=2 |3.4 Sleep-disordered breathing
+| 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: #F5F5F5; width: 70%" align=left colspan=2 |3.5 Alveolar [[hypoventilation]] disorders
+| 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: #F5F5F5; width: 70%" align=left colspan=2 |3.6 Chronic exposure to high altitude
+| 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 colspan=2 |3.7 Developmental lung diseases
+| 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: #4682B4; color: #FFFFFF" align=left |'''Group 4. Chronic thromboembolic pulmonary hypertension (CTEPH)'''
+| 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: #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" |5.3 Metabolic disorders: [[glycogen storage disease]], [[Gaucher disease]], [[thyroid]] disorders
 |-
-| style="padding: 0 5px; font-size: 100%; background: #F5F5F5; width: 70%" align=left colspan=2 |5.1 Hematologic disorders: chronic [[hemolytic anemia]], [[myeloproliferative disorder]]s, [[splenectomy]]
+| style="padding: 0 5px; font-size: 100%; background: #F5F5F5; width: 70%" align="left" |5.4 Others: [[tumor]] obstruction, fibrosing [[mediastinitis]], [[chronic renal failure]], segmental PH
+|}
+==Classification Based on Hemodynamical Findings==
+<span style="font-size: 80%;">'''Abbreviations:''' '''PAP:''' Pulmonary artery pressure; '''PWP:''' pulmonary wedge pressure </span>
+{| style="cellpadding=0; cellspacing= 0; width: 600px;"
 |-
-| style="padding: 0 5px; font-size: 100%; background: #F5F5F5; width: 70%" align=left colspan=2 |5.2 Systemic disorders: [[sarcoidosis]], [[Langerhans cell histiocytosis|pulmonary histiocytosis]], [[lymphangioleiomyomatosis]]
+| 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; width: 70%" align=left colspan=2 |5.3 Metabolic disorders: [[glycogen storage disease]], [[Gaucher disease]], [[thyroid]] disorders
+| 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; width: 70%" align=left colspan=2 |5.4 Others: [[tumor]] obstruction, fibrosing [[mediastinitis]], [[chronic renal failure]], segmental PH
+| 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
 |}
-<span style="font-size: 80%;">''Abbreviations:'' BMPR, bone morphogenic protein receptor type II; CAV1, caveolin-1; ENG, endoglin; HIV, human immunodeficiency virus.</span>
-===Functional Classification===
+==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]]
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