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 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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