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{{Pulmonary hypertension}}
{{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}}


==Overview==


==Dana Point clinical classification==
Pulmonary hypertension (PH) has been previously classified as primary (currently known as idiopathic pulmonary arterial hypertension or IPAH) and secondary in 1973.  In 1988, a clinical classification of PH into 5 groups has been developed during the 2nd World Symposium on PH in Evian, France. 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. 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).


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>
==Classification==
===Clinical Classification===
* 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>


* 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:
** 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>
** 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 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>


*'''1. Pulmonary arterial hypertension (PAH)'''
* 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).
**1.1. Idiopathic PAH
* 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.
**1.2. [[Heritable]]
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>
***1.2.1. BMPR2
***1.2.2. ALK1, endoglin (with or without [[hereditary hemorrhagic telangiectasia]])  
***1.2.3. Unknown
**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==
<span style="font-size: 80%;">''Abbreviations:'' BMPR, bone morphogenic protein receptor type II; CAV1, caveolin-1; ENG, endoglin; HIV, human immunodeficiency virus.</span>
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.


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>
1.2.1 [[BMPR2]] <br>
1.2.2 [[ALK-1]], [[Endoglin|ENG]], [[SMAD9]], [[CAV1]], [[KCNK3]] <br>
1.2.3 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>
1.4.1 [[Connective tissue disease]] <br>
1.4.2 [[HIV infection]] <br>
1.4.3 [[Portal hypertension]] <br>
1.4.4 [[Congenital heart disease]]s <br>
1.4.5 [[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>
===WHO Functional Classification===
*WHO Group I - Pulmonary arterial hypertension (PAH)
The WHO functional classification is used for the assessment of the severity of PH in order to tailor the choice of therapy.  Shown below is a table summarizing the different functional classes.<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>
*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)'''
{| style="cellpadding=0; cellspacing= 0; width: 600px;"
*'''1. Pulmonary arterial hypertension (PAH)'''  
|-
**1.1. Idiopathic (IPAH)
| 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" |'''Description'''
**1.2. Familial (FPAH)
|-
**1.3. Associated with (APAH)
| style="padding: 0 5px; font-size: 100%; background: #F5F5F5; width: 30%" align="left" | '''I''' || style="padding: 0 5px; font-size: 100%; background: #F5F5F5; width: 70%" align="left" |
***1.3.1. [[Collagen vascular disease]]
* No limitation of usual physical activity
***1.3.2. Congenital systemic-to-pulmonary shunts
* No increased dyspnea, fatigue, chest pain, or presyncope upon ordinary physical activity
***1.3.3. [[Portal hypertension]]
|-
***1.3.4. [[HIV]] infection
| style="padding: 0 5px; font-size: 100%; background: #F5F5F5; width: 30%" align="left" |'''II''' || style="padding: 0 5px; font-size: 100%; background: #F5F5F5; width: 70%" align="left" |
***1.3.5. Drugs and toxins
* Mild limitation of physical activity
***1.3.6. Other ([[thyroid disorders]], [[glycogen storage disease]], [[Gaucher disease]], [[hereditary hemorrhagic telangiectasia]], [[hemoglobinopathies]], [[myeloproliferative disorders]], [[splenectomy]])
* No discomfort at rest
**1.4. Associated with significant venous or capillary involvement
* Increased dyspnea, fatigue, chest pain, or presyncope upon normal physical activity
***1.4.1. Pulmonary veno-occlusive disease (PVOD)
|-
***1.4.2. Pulmonary capillary hemangiomatosis (PCH)
| style="padding: 0 5px; font-size: 100%; background: #F5F5F5; width: 30%" align="left" |'''III''' || style="padding: 0 5px; font-size: 100%; background: #F5F5F5; width: 70%" align="left" |
**1.5. Persistent pulmonary hypertension of the newborn
* Marked limitation of physical activity
*'''2. Pulmonary hypertension with left heart disease'''  
* No discomfort at rest
**2.1. Left-sided atrial or ventricular heart disease
* Increased dyspnea, fatigue, chest pain, or presyncope upon less than ordinary activity
**2.2. Left-sided [[valvular heart disease]]
|-
*'''3. Pulmonary hypertension associated with lung diseases and/or hypoxemia'''  
| style="padding: 0 5px; font-size: 100%; background: #F5F5F5; width: 30%" align="left" |'''IV''' || style="padding: 0 5px; font-size: 100%; background: #F5F5F5; width: 70%" align="left" |
**3.1. [[Chronic obstructive pulmonary disease]]
* Inability to perform any physical activity at rest with/without signs of right ventricular failure
**3.2. [[Interstitial lung disease]]
* Dyspnea and/or fatigue may be present at rest
**3.3. Sleep-disordered breathing
* Increased dyspnea, fatigue, chest pain, or presyncope by almost any physical activity
**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==
===Classification Based on Hemodynamical Findings===
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 literatureHowever, some physicians continue to use the older classification inappropriately.
<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: #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
|}
 
===Classification Based on Histopathological Findings===
PH is a pathological condition present in different disease states that share similar clinical manifestation 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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Revision as of 14:59, 27 March 2018

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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]

Overview

Pulmonary hypertension (PH) has been previously classified as primary (currently known as idiopathic pulmonary arterial hypertension or IPAH) and secondary in 1973. In 1988, a clinical classification of PH into 5 groups has been developed during the 2nd World Symposium on PH in Evian, France. 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. 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 thromboembolic pulmonary hypertension (Group 4), and pulmonary hypertension due to unclear multifactorial mechanisms (Group 5).

Classification

Clinical Classification

  • PH was first classified into primary and secondary in 1973 during the World Health Organization (WHO) meeting on PH in Geneva, Switzerland.[1]
  • 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.[2] Since then, the clinical classification of PH was updated in the following meetings:
    • The 3d World Symposium on Pulmonary Hypertension (2003) in Venice, Italy[3]
    • The 4th World Symposium on Pulmonary Hypertension (2008) in Dana Point, California, USA[4]
    • The 5th World Symposium on Pulmonary Hypertension (2013) in Nice, France[5]
  • 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.

Shown below is a table summarizing the updated clinical classification of PH.[5]

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

WHO Functional Classification

The WHO functional classification is used for the assessment of the severity of PH in order to tailor the choice of therapy. Shown below is a table summarizing the different functional classes.[6]

Class Description
I
  • No limitation of usual physical activity
  • No increased dyspnea, fatigue, chest pain, or presyncope upon ordinary physical activity
II
  • Mild limitation of physical activity
  • No discomfort at rest
  • Increased dyspnea, fatigue, chest pain, or presyncope upon normal physical activity
III
  • Marked limitation of physical activity
  • No discomfort at rest
  • Increased dyspnea, fatigue, chest pain, or presyncope upon less than ordinary activity
IV
  • Inability to perform any physical activity at rest with/without signs of right ventricular failure
  • Dyspnea and/or fatigue may be present at rest
  • Increased dyspnea, fatigue, chest pain, or presyncope by almost any physical activity

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 manifestation and some common histopathological features. Shown below is a table that summarizes the classification of PH based on histopathology findings.[7]

Class Histopathological findings[7]
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

Pulmonary microvasculopathy
(with or without arteriopathy and/on venopathy)
Changes in the capillaries:
  • Localized capillary proliferation

Changes in the interstitium

Unclassified Non specific changes

References

  1. Hatano S, Strasser T. Primary Pulmonary Hypertension. Report on a WHO Meeting. October 15–17, 1973, Geneva: World Health Organization, 1975.
  2. 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.
  3. Simonneau G, Galiè N, Rubin LJ, Langleben D, Seeger W, Domenighetti G; et al. (2004). "Clinical classification of pulmonary hypertension". J Am Coll Cardiol. 43 (12 Suppl S): 5S–12S. doi:10.1016/j.jacc.2004.02.037. PMID 15194173.
  4. Simonneau G, Robbins IM, Beghetti M, Channick RN, Delcroix M, Denton CP; et al. (2009). "Updated clinical classification of pulmonary hypertension". J Am Coll Cardiol. 54 (1 Suppl): S43–54. doi:10.1016/j.jacc.2009.04.012. PMID 19555858.
  5. 5.0 5.1 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.
  6. 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.
  7. 7.0 7.1 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.

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