Klasifikasi Hipertensi Pulmonal
Revised Clinical Classification of Pulmonary Hypertension
Pulmonary
arterial hypertension (PAH)
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|
Idiopathic
(IPAH) |
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Familial
(FPAH) |
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Associated with
(APAH)
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Collagen
vascular disease |
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|
Congenital
systemic-to-pulmonary shunts |
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|
Portal
hypertension |
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|
Human
immunodeficiency virus (HIV) infection |
| |
|
Drugs and
toxins |
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|
Other (thyroid
disorders, glycogen storage disease, Gaucher disease, hereditary hemorrhagic
telangiectasia, hemoglobinopathies, myeloproliferative disorders,
splenectomy) |
|
| |
|
Associated
with significant venous or capillary involvement
| |
|
Pulmonary
venoocclusive disease (PVOD) |
| |
|
Pulmonary
capillary hemangiomatosis (PCH)
|
|
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|
Persistent
pulmonary hypertension of the newborn
|
|
|
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Pulmonary
hypertension with left heart disease
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|
Left-sided
arterial or ventricular heart disease |
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|
Left-sided
valvular heart disease
|
|
|
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|
Pulmonary
hypertension associated with lung disease and/or hypoxemia
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|
Chronic
obstructive pulmonary disease |
| |
|
Interstitial
lung disease |
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Sleep-disordered
breathing |
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Alveolar
hypoventilation disorders |
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Chronic
exposure to high altitude |
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Developmental
abnormalities
|
|
|
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|
Pulmonary
hypertension caused by chronic thrombotic and/or embolic disease
| |
|
Thromboembolic
obstruction of proximal pulmonary arteries |
| |
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Thromboembolic
obstruction of distal pulmonary arteries |
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|
Nonthrombotic
pulmonary embolism (tumor, parasites, foreign material)
|
|
|
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Miscellaneous
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|
Sarcoidosis,
histiocytosis X, lymphangiomatosis, compression of pulmonary vessels
(adenopathy, tumor, fibrosing mediastinitis)
|
|
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From Simmoneau G, Galie N, Rubin LJ, et al: Clinical classification of
pulmonary hypertension. J Am Coll Cardiol 43:5S, 2004.
WHO Functional Classification of Pulmonary Hypertension
| 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 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 syncope. |
| Class III—Patients with pulmonary hypertension resulting in
marked limitation of physical activity. They are comfortable at rest. Less than
ordinary activity causes undue dyspnea or fatigue, chest pain, or
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. |
Referensi: Braunwald's Heart Disease 8E (Saunders) 2007