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(/* WHO Classification of Pulmonary Hypertension{{Cite journal | last1 = Simonneau | first1 = G. | last2 = Gatzoulis | first2 = MA. | last3 = Adatia | first3 = I. | last4 = Celermajer | first4 = D. | last5 = Denton | first5 = C. | last6 = Ghofrani | fi...)
(/* WHO Classification of Pulmonary Hypertension{{Cite journal | last1 = Simonneau | first1 = G. | last2 = Gatzoulis | first2 = MA. | last3 = Adatia | first3 = I. | last4 = Celermajer | first4 = D. | last5 = Denton | first5 = C. | last6 = Ghofrani | fi...)
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During the World Symposium on Pulmonary Hypertension (WSPH), five groups of disorders that cause pulmonary hypertension were identified: '''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). This 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), and is currently used by the U.S. Food and Drug Administration (FDA) and the European Medicines Agency (EMA) for the labelling of new drugs approved for pulmonary hypertension.
During the World Symposium on Pulmonary Hypertension (WSPH), five groups of disorders that cause pulmonary hypertension were identified: '''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). This 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), and is currently used by the U.S. Food and Drug Administration (FDA) and the European Medicines Agency (EMA) for the labelling of new drugs approved for pulmonary hypertension.


{{cquote|
{| style="cellpadding=0; cellspacing= 0; width: 600px;"
 
|-
* '''1. Pulmonary arterial hypertension (PAH)'''
| style="padding: 0 5px; font-size: 100%; background: #4682B4; color: #FFFFFF" align=center |'''1. Pulmonary arterial hypertension (PAH)'''
** 1.1. Idiopathic PAH  
|-
** 1.2. Heritable PAH
| style="padding: 0 5px; font-size: 100%; background: #F5F5F5; width: 70%" align=left colspan=2 |'''1.1. Idiopathic PAH'''
*** 1.2.1 [[BMPR2]]
|-
*** 1.2.2 [[ALK-1]], [[Endoglin|ENG]], [[SMAD9]], [[CAV1]], [[KCNK3]]
| style="padding: 0 5px; font-size: 100%; background: #F5F5F5; width: 70%" align=left colspan=2 |'''1.2. Heritable PAH''' <br>
*** 1.2.3 Unknown
1.2.1 [[BMPR2]] <br>
** 1.3 Drug and toxin induced
1.2.2 [[ALK-1]], [[Endoglin|ENG]], [[SMAD9]], [[CAV1]], [[KCNK3]] <br>
*** ''Definite'' (an epidemic or large multicenter epidemiologic studies demonstrating an association between a drug and PAH)
1.2.3 Unknown
**** [[Aminorex]]
|-
**** [[Fenfluramine]]
| style="padding: 0 5px; font-size: 100%; background: #F5F5F5; width: 70%" align=left colspan=2 |'''1.3 Drug and toxin induced''' <br>
**** [[Dexfenfluramine]]
''Definite'' (an epidemic or large multicenter epidemiologic studies demonstrating an association between a drug and PAH) <br>
**** Toxic [[rapeseed oil]]
*[[Aminorex]] <br>
**** [[Benfluorex]]
*[[Fenfluramine]] <br>
**** [[SSRI]]s
*[[Dexfenfluramine]] <br>
*** ''Likely'' (a single case-control study demonstrating an association or a multiple-case series)
*Toxic [[rapeseed oil]] <br>
**** [[Amphetamine]]s
*[[Benfluorex]] <br>
**** L-[[Tryptophan]]
*[[SSRI]]s <br>
**** [[Methamphetamine]]s
''Likely'' (a single case-control study demonstrating an association or a multiple-case series) <br>
**** [[Dasatinib]]
*[[Amphetamine]]s <br>
*** ''Possible'' (drugs with similar mechanisms of action as those in the definite or likely category but which have not yet been studied)
*L-[[Tryptophan]] <br>
**** [[Cocaine]]
*[[Methamphetamine]]s <br>
**** [[Phenylpropanolamine]]
*[[Dasatinib]] <br>
**** [[St. John's wort]]
''Possible'' (drugs with similar mechanisms of action as those in the definite or likely category but which have not yet been studied) <br>
**** [[Chemotherapeutic agent]]s
*[[Cocaine]] <br>
**** [[Interferon]] α and β
*[[Phenylpropanolamine]] <br>
**** [[Amphetamine]]-like drugs
*[[St. John's wort]] <br>
*** ''Unlikely'' (one in which a drug has been studied in epidemiologic studies and an association with PAH has not been demonstrated)
*[[Chemotherapeutic agent]]s <br>
**** [[Oral contraceptive]]s
*[[Interferon]] α and β <br>
**** [[Estrogen]]
*[[Amphetamine]]-like drugs <br>
**** [[Cigarette smoking]]
''Unlikely'' (one in which a drug has been studied in epidemiologic studies and an association with PAH has not been demonstrated) <br>
** 1.4 Associated with:
*[[Oral contraceptive]]s <br>
*** 1.4.1 [[Connective tissue disease]]
*[[Estrogen]] <br>
*** 1.4.2 [[HIV infection]]
*[[Cigarette smoking]]
*** 1.4.3 [[Portal hypertension]]
|-
*** 1.4.4 [[Congenital heart disease]]s
| style="padding: 0 5px; font-size: 100%; background: #F5F5F5; width: 70%" align=left colspan=2 |'''1.4 Associated with:''' <br>
*** 1.4.5 [[Schistosomiasis]]
1.4.1 [[Connective tissue disease]] <br>
* '''1’ Pulmonary veno-occlusive disease (PVOD) and/or pulmonary capillary hemangiomatosis (PCH)'''
1.4.2 [[HIV infection]] <br>
* '''1’’ Persistent pulmonary hypertension of the newborn (PPHN)'''
1.4.3 [[Portal hypertension]] <br>
 
1.4.4 [[Congenital heart disease]]s <br>
* '''2. Pulmonary hypertension due to left heart disease'''
1.4.5 [[Schistosomiasis]] <br>
** 2.1 [[Left ventricular systolic dysfunction]]
|-
** 2.2 [[diastolic dysfunction|Left ventricular diastolic dysfunction]]
| style="padding: 0 5px; font-size: 100%; background: #4682B4; color: #FFFFFF" align=center |'''1’ Pulmonary veno-occlusive disease (PVOD) and/or pulmonary capillary hemangiomatosis (PCH)'''
** 2.3 [[Valvular disease]]
|-
** 2.4 Congenital/acquired left heart inflow/outflow tract obstruction and congenital cardiomyopathies
| style="padding: 0 5px; font-size: 100%; background: #4682B4; color: #FFFFFF" align=center |'''1’’ Persistent pulmonary hypertension of the newborn (PPHN)'''
 
|-
* '''3. Pulmonary hypertension due to lung diseases and/or hypoxia'''
| style="padding: 0 5px; font-size: 100%; background: #4682B4; color: #FFFFFF" align=center |'''2. Pulmonary hypertension due to left heart disease'''
** 3.1 [[Chronic obstructive pulmonary disease]]
|-
** 3.2 [[Interstitial lung disease]]
| style="padding: 0 5px; font-size: 100%; background: #F5F5F5; width: 70%" align=left colspan=2 |2.1 [[Left ventricular systolic dysfunction]]
** 3.3 Other pulmonary diseases with mixed restrictive and obstructive pattern
|-
** 3.4 Sleep-disordered breathing
| style="padding: 0 5px; font-size: 100%; background: #F5F5F5; width: 70%" align=left colspan=2 |2.2 [[diastolic dysfunction|Left ventricular diastolic dysfunction]]
** 3.5 Alveolar hypoventilation disorders
|-
** 3.6 Chronic exposure to high altitude
| style="padding: 0 5px; font-size: 100%; background: #F5F5F5; width: 70%" align=left colspan=2 |2.3 [[Valvular disease]]
** 3.7 Developmental lung diseases
|-
 
| style="padding: 0 5px; font-size: 100%; background: #F5F5F5; width: 70%" align=left colspan=2 |2.4 Congenital/acquired left heart inflow/outflow tract obstruction and congenital cardiomyopathies
* '''4. Chronic thromboembolic pulmonary hypertension (CTEPH)'''
|-
 
| style="padding: 0 5px; font-size: 100%; background: #4682B4; color: #FFFFFF" align=center |'''3. Pulmonary hypertension due to lung diseases and/or hypoxia'''
* '''5. Pulmonary hypertension with unclear multifactorial mechanisms'''
|-
** 5.1 Hematologic disorders: chronic [[hemolytic anemia]], [[myeloproliferative disorder]]s, [[splenectomy]]
| style="padding: 0 5px; font-size: 100%; background: #F5F5F5; width: 70%" align=left colspan=2 |3.1 [[Chronic obstructive pulmonary disease]]
** 5.2 Systemic disorders: [[sarcoidosis]], [[Langerhans cell histiocytosis|pulmonary histiocytosis]], [[lymphangioleiomyomatosis]]
|-
** 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 |3.2 [[Interstitial lung disease]]
** 5.4 Others: tumoral obstruction, [[fibrosing mediastinitis]], [[chronic renal failure]], segmental PH|}}
|-
| 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 colspan=2 |3.4 Sleep-disordered breathing
|-
| 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: #F5F5F5; width: 70%" align=left colspan=2 |3.6 Chronic exposure to high altitude
|-
| 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: #4682B4; color: #FFFFFF" align=center |'''4. Chronic thromboembolic pulmonary hypertension (CTEPH)'''
|-
| style="padding: 0 5px; font-size: 100%; background: #4682B4; color: #FFFFFF" align=center |'''5. Pulmonary hypertension with unclear multifactorial mechanisms'''
|-
| 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 colspan=2 |5.2 Systemic disorders: [[sarcoidosis]], [[Langerhans cell histiocytosis|pulmonary histiocytosis]], [[lymphangioleiomyomatosis]]
|-
| 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; width: 70%" align=left colspan=2 |5.4 Others: tumoral obstruction, [[fibrosing mediastinitis]], [[chronic renal failure]], segmental PH
|}


::: <span style="font-size: 80%;">''Abbreviations:'' BMPR, bone morphogenic protein receptor type II; CAV1, caveolin-1; ENG, endoglin; HIV, human immunodeficiency virus.</span>
::: <span style="font-size: 80%;">''Abbreviations:'' BMPR, bone morphogenic protein receptor type II; CAV1, caveolin-1; ENG, endoglin; HIV, human immunodeficiency virus.</span>

Revision as of 14:27, 27 August 2014

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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.

Overview

Pulmonary hypertension has been previously divided into two categories: primary (currently known as idiopathic pulmonary arterial hypertension [IPAH]) and secondary. However; given the fact that some subcategories of secondary pulmonary hypertension share several similarities with primary pulmonary hypertension in terms of pathology, progression and response to therapy, the WHO (World Health Organization) has based its reclassification of pulmonary hypertension on the mechanism of the disease.

Classification

WHO Classification of Pulmonary Hypertension[1]

During the World Symposium on Pulmonary Hypertension (WSPH), five groups of disorders that cause pulmonary hypertension were identified: 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). This 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), and is currently used by the U.S. Food and Drug Administration (FDA) and the European Medicines Agency (EMA) for the labelling of new drugs approved for pulmonary hypertension.

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 epidemiologic 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 epidemiologic 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)
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
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
4. Chronic thromboembolic pulmonary hypertension (CTEPH)
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: tumoral obstruction, fibrosing mediastinitis, chronic renal failure, segmental PH
Abbreviations: BMPR, bone morphogenic protein receptor type II; CAV1, caveolin-1; ENG, endoglin; HIV, human immunodeficiency virus.

Other Classification

The Venice 2003 Revised Classification System

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.

The Venice 2003 Revised Classification system can be summarized as follows:[2]

  • 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)

Sarcoidosis, histiocytosis X, lymphangiomatosis, compression of pulmonary vessels (adenopathy, tumor, fibrosing mediastinitis

References

  1. Simonneau, G.; Gatzoulis, MA.; Adatia, I.; Celermajer, D.; Denton, C.; Ghofrani, A.; Gomez Sanchez, MA.; Krishna Kumar, R.; Landzberg, M. (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. Unknown parameter |month= ignored (help)
  2. 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.

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