The classification system for pulmonary hypertension was revised at the 4th World Symposium on Pulmonary Hypertension held in Dana Point, California, in 2008 1.
This system is as follows:
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group 1: pulmonary arterial hypertension
- 1.1: idiopathic pulmonary arterial hypertension
- 1.2: heritable pulmonary hypertension
- 1.3: drug- and toxin-induced pulmonary hypertension
- 1.4: pulmonary hypertension associated with
- 1.5: persistent pulmonary hypertension of the newborn
- 1.6 pulmonary veno-occlusive disease and pulmonary capillary haemangiomatosis
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group 2: pulmonary hypertension due to left-sided heart disease
- 2.1: systolic dysfunction
- 2.2: diastolic dysfunction
- 2.3: valvular disease
- group 3: pulmonary hypertension due to lung diseases or hypoxia
- group 4: pulmonary hypertension due to chronic pulmonary thromboembolism
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group 5: pulmonary hypertension due to unclear multifactorial mechanisms
- myeloproliferative disorders
- splenectomy
- systemic disorders
- metabolic disorders
- other conditions
- fibrosing mediastinitis
- tumoral obstruction
- dialysis-dependent chronic renal failure