Pulmonary arterial hypertension (PAH) is a progressive and debilitating disease with poor prognosis and limited therapeutic options. While currently available PAH treatments can provide substantial clinical benefits, they are not curative. The efficacy of prostanoid treatment in PAH is well-established in clinical trials, open-label and long-term observational studies, and prostanoid therapy is considered the most effective treatment for moderate to severe PAH. However, a growing body of data demonstrates that the majority of PAH patients are not treated with a prostanoid, even at the time of their death. The logistics of parenteral administration have limited prostanoid use in clinical practice in some centres; the lure of using a simple oral therapy can be compelling for both the physician and the patient. However, deterioration on oral therapies appears inevitable and therefore, there is a need to initiate early prostanoid therapy in eligible patients and to overcome barriers to their use. Oral treatment delays patient referral to specialist PAH centres, significantly affecting the prognosis. Recent studies have demonstrated that an aggressive early approach to treatment, utilising prostanoids as first-line therapy, either as monotherapy or in combination with oral therapies, results in excellent long-term survival.
Long-term safety and survival outcomes of patients with pulmonary arterial hypertension enrolled in an open label continuation study with tadalafil 40mg daily
