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.; 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 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 | |
| 1.3 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’ 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
Complex lesions in pulmonary arteries
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| 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
Changes in the capillaries
Changes in the interstitium |
| Pulmonary microvasculopathy (with or without arteriopathy and/on venopathy) |
Changes in the capillaries
Changes in the interstitium |
| Unclassified | Non specific changes |
References
- ↑ Hatano S, Strasser T. Primary Pulmonary Hypertension. Report on a WHO Meeting. October 15–17, 1973, Geneva: World Health Organization, 1975.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 5.0 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.
- ↑ 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.
- ↑ 7.0 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.