Table 5.

Therapy according to the clinical classification.

Group (clinical classification)	Therapeutic strategy
1. Pulmonary arterial hypertension	Specific therapy (idiopathic and heritable forms)
	Primary therapy (forms associated to other diseases)
	Supportive therapy (oral anticoagulant, oxygen, diuretics)
	Surgical therapy (lung transplantation, atrial septostomy)
2. Pulmonary hypertension due to left heart disease	Primary therapy
	Supportive therapy (diuretics, oxygen)
3. Pulmonary hypertension due to lung diseases and/or hypoxaemia	Primary therapy
	Supportive therapy (diuretics, oxygen)
	Specific therapy (in selected patients)
	Surgical therapy (lung transplantation)
4. Chronic thromboembolic pulmonary hypertension	Supportive therapy (oral anticoagulant)
	Surgical therapy (thromboendoarterectomy)
	Specific therapy (in selected patients)
5. PH with unclear and/or multifactorial mechanisms	Primary therapy
	Supportive therapy (oxygen)