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Disease Classification

Pulmonary Arterial Hypertension (PAH) Classification

The New York Heart Association (NYHA) Functional Assessment measures symptom severity associated with pulmonary arterial hypertension (PAH) and the WHO Group Classification of Pulmonary Hypertension (Venice version) helps categorize PAH by type. Both classification systems are below.

New York Heart Association (NYHA) Functional Assessment1

Class I: Patients with pulmonary hypertension but without resulting limitation of physical activity. Ordinary physical activity does not cause undue dyspnea or fatigue, chest pain, or near syncope.

Class II: Patients with pulmonary hypertension resulting in slight limitation of physical activity. They are comfortable at rest. Ordinary physical activity causes undue dyspnea or fatigue, chest pain, or near syncope.

Class III: Patients with pulmonary hypertension resulting in marked limitation of physical activity. They are comfortable at rest. Less-than-ordinary physical activity causes undue dyspnea or fatigue, chest pain, or near syncope.

Class IV: Patients with pulmonary hypertension with inability to carry out any physical activity without symptoms. These patients manifest signs of right heart failure. Dyspnea and/or fatigue may even be present at rest. Discomfort is increased by any physical activity.

Third World Symposium on Pulmonary Arterial Hypertension (Venice, 2003)
Updated and modified the Evian Classification based on a new understanding of disease mechanisms.

WHO Group Classification of Pulmonary Hypertension²

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 (e.g., anorexigens, rapeseed oil, L-typotophan, methamphetamine, and cocaine)

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

1.4.2 Pulmonary-capillary hemanglomatosis

1.5 Persisted pulmonary hypertension of the newborn

1. American Heart Association. 1994 Revisions to Classification of Functional Capacity and Objective Assessment of Patients with Diseases of the Heart. Available at http://www.americanheart.org/presenter.jhtml?identifier=1712. Accessed October 3, 2007.
2. Simonneau G, Galie N, Rubin LJ, et al. Clinical classification of pulmonary hypertension. J Am Coll Cardiol. 2004;43(12 suppl S):5S-12S.

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