TABLE 3.

Classification of pulmonary hypertension as advanced at the World Symposium on Primary Pulmonary Hypertension

Pulmonary arterial hypertension

Primary pulmonary hypertension

Sporadic disorder

Familial disorder

Related conditions

Collagen vascular disease

Congenital systemic-to-pulmonary shunt

Portal hypertension

HIV infection

Drugs and toxins

Anorectic agents (appetite suppressants)

Others

Persistent pulmonary hypertension of the newborn

Others

Pulmonary venous hypertension

Left-sided atrial or ventricular heart disease

Left-sided valvular heart disease

Extrinsic compression of central pulmonary veins

Fibrosing mediastinitis

Adenopathy and/or tumours

Pulmonary veno-occlusive disease

Others

Pulmonary hypertension associated with disorders of the respiratory system and/or hypoxemia

Chronic obstructive pulmonary disease

Interstitial lung disease

Sleep-disordered breathing

Alveolar hypoventilation disorders

Chronic exposure to high altitudes

Neonatal lung disease

Alveolar-capillary dysplasia

Others

Pulmonary hypertension resulting from chronic thrombotic and/or embolic disease

Thromboembolic obstruction of proximal pulmonary arteries

Obstruction of distal pulmonary arteries

Pulmonary embolism (thrombus, tumour, ova and/or parasites, foreign material)

In situ thrombosis

Sickle cell disease

Pulmonary hypertension resulting from disorders directly affecting the pulmonary vasculature

Inflammatory conditions

Schistosomiasis

Sarcoidosis

Others

Pulmonary capillary hemangiomatosis

Adapted with permission from reference 139