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 |
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Drugs and
toxins |
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Other (thyroid
disorders, glycogen storage disease, Gaucher disease, hereditary hemorrhagic
telangiectasia, hemoglobinopathies, myeloproliferative disorders,
splenectomy) |
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Associated
with significant venous or capillary involvement
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Pulmonary
venoocclusive disease (PVOD) |
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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 |
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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
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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