Pulmonary hypertension classification
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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 (PH) has been previously classified as primary (currently known as idiopathic pulmonary arterial hypertension or IPAH) and secondary. In 1988, a clinical classification of PH into 5 groups has been developed during the 2nd World Symposium on Pulmonary Hypertension in Evian.[1] The clinical classification has been updated regularly as the understanding of PH expanded; nevertheless, the main scheme of classification into the 5 groups remains valid. The classification of PH has been last modified in 2013 during the 5th World Symposium on Pulmonary Hypertension in Nice.[2] 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).[2]
Classification
WHO Classification of Pulmonary Hypertension[3]
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.
| Group 1. Pulmonary arterial hypertension (PAH) | |
| 1.1. Idiopathic PAH | |
| 1.2. Heritable PAH 1.2.1 BMPR2 | |
| 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’ 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 | |
- 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:[4]
- 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)
- 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
References
- ↑ 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.
- ↑ 2.0 2.1 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.
- ↑ 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) - ↑ 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.