Vascular > PAH

WHO Classification of pulmonary hypertesnion:

From: Thorax 1999; Peacock AJ. Primary pulmonary hypertension. 54: 1107-1118

1. Pulmonary arterial hypertension:
    1.1 Primary pulmonary hypertension
        (a) Sporadic
        (b) Familial

    1.2 Related to:
        (a) Collagen vascular disease
        (b) Congenital systemic to pulmonic shunts
        (c) Portal hypertension
        (d) HIV infection
        (e) Drugs: Fenfluramine, Dexfenfluramine, Amphetamines, L-trytophan

2. Pulmonary venous hypertension:
    2.1 Left sided atrial or ventricular disease
    2.2 Left sided valvular heart disease
    2.3 Extrinsic compression of the central pulmonary veins
        (a) Fibrosing mediastinitis
        (b) Adenopathy/tumor
    2.4 Pulmonary veno-occlusive disease

3. Pulmonary hypertension associated with disorders of the respiratory system and/or hypoxemia:
    3.1 COPD
    3.2 Interstitial lung disease
    3.3 Sleep disordered breathing
    3.4 Alveolar hypoventilation disorders
    3.5 Chronic exposure to high altitude
    3.6 Neonatal lung disease
    3.7 Alveolar-capillary dysplasia

4. Pulmonary hypertension due to chronic thrombotic and/or embolic disease
    4.1 Thromboembolic obstruction of proximal pulmonary arteries
    4.2 Obstruction of distal pulmonary arteries:
        (a) Pulmonary embolism
        (b) In situ thrombus
        (c) Sickle cell disease

5. Pulmonary hypertension due to disorders directly affecting the pulmonary vasculature:
    5.1 Inflammatory
        (a) Shistosomiasis
        (b) Sarcoid
        (c) Other
    5.2 Pulmonary capillary hemangiomatosis