. 2009 Aug 6;5:607–619. doi: 10.2147/vhrm.s4713

Table 1.

The Venice classification of pulmonary hypertension 2003

Group 1. Pulmonary arterial hypertension
1.1. Idiopathic pulmonary arterial hypertension
1.2. Familial pulmonary arterial hypertension
1.3. Pulmonary arterial hypertension associated with:
1.3.1. Collagen vascular disease, eg, scleroderma, systemic lupus erythematosus, rheumatoid arthritis
1.3.2. Congenital systemic-to-pulmonary shunts
1.3.3. Portal hypertension, eg, ethanol induced cirrhosis
1.3.4. HIV infection
1.3.5. Drugs and toxins, eg, fenfluramine
1.3.6. Other (thyroid disorders, glycogen storage disease, Gaucher’s disease, hereditary hemorrhagic telangiectasia, hemoglobinopathies, myeloproliferative disorders, splenectomy)
1.4. Associated with significant venous or capillary involvement
1.4.1. Pulmonary veno-occlusive disease (PVOD)
1.4.2. Pulmonary capillary hemangiomatosis (PCH)
1.5. Persistent pulmonary hypertension of the newborn
Group 2. Pulmonary hypertension with left heart disease
2.1. Left sided atrial or ventricular heart disease
2.2. Left sided valvular heart disease
Group 3. Pulmonary hypertension associated with lung disease or hypoxemia
3.1. Chronic obstructive pulmonary disease
3.2. Interstitial lung disease
3.3. Sleep-disordered breathing
3.4. Alveolar hypoventilation disorders
3.5. Chronic exposure to high altitude
3.6. Developmental abnormalities
Group 4. Pulmonary hypertension due to chronic thrombotic, embolic disease, or both
4.1. Thromboembolic obstruction of proximal pulmonary arteries
4.2. Thromboembolic obstruction of distal pulmonary arteries
4.3. Non-thrombotic pulmonary embolism (tumor, parasites or foreign material)
Group 5. Miscellaneous
Eg, sarcoidosis, pulmonary Langerhans’-cell histiocytosis, lymphangiomatosis, granulomatous disease, compression of pulmonary vessels (adenopathy, tumor or fibrosing mediastinitis)