Abstract
The treatment algorithm for pulmonary arterial hypertension is applied after the diagnosis is confirmed in an expert centre. General measures and supportive therapy can be started as required. A vasoreactivity test should be then performed in patients with idiopathic, heritable, or drug-induced pulmonary arterial hypertension. In case of vasoreactivity, according to pre-defined criteria, calcium channel blocker therapy should be started and up-titrated progressively to the highest tolerated dose. In non-vasoreactive patients, initial monotherapy or double combination therapy with orally available compounds are the recommended strategies in low- or intermediate-risk patients. Initial combination therapy including intravenous prostacyclin analogues is the initial treatment of choice in high-risk patients. In case of inadequate clinical response to the initial therapy, sequential double or triple combination therapy is recommended. If maximal medical therapy does not provide an adequate response, the pulmonary arterial hypertension patient should be listed for double-lung transplantation.
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