Long-Term Treatment With Sildenafil Citrate in Pulmonary Arterial Hypertension

Efficacy and safety of sildenafil treatment in pulmonary arterial hypertension: A systematic review

“…Some long-term data on sildenafil safety and efficacy are already available. The open-label, non-controlled SUPER-2 trial suggests that 3-year sildenafil monotherapy of PAH is generally well-tolerated [26]. In contrast, the STARTS-2 study suggests that, among pediatric patients, high-dose sildenafil monotherapy is associated with greater risk of mortality than is low-dose monotherapy [27].…”

Prognostic Factors in Severe Pulmonary Hypertension Patients Who Need Parenteral Prostanoid Therapy: The Impact of Late Referral

“…26 The good survival in the open-label phase of the randomized controlled study has created the wrong conclusion that oral therapy represents a definite cure for PAH, but survival is satisfactory only when other drugs are promptly added in case of clinical worsening, which is a common experience during oral monotherapy. 10,[12][13][14][15][16] In real-world clinical practice, most PAH patients are still dying on monotherapy, with only a few being escalated to parenteral prostanoids, as documented by specialty pharmacy service providers, 27 confirming a delayed use of prostanoids usually not available in the non-expert centers.…”

“…Treatment was tailored during follow-up to obtain the maximum clinical effect, and therapies were added as clinical worsening occurred. 16 All data were collected as clinical records on an Access software database (Microsoft, Redmond, WA).…”

“…The diagnosis of PH type relied on RHC and the use of an algorithm incorporating a mandatory chest X-ray image, respiratory function tests, a perfusion lung scan or computed tomography (CT) scan, an echocardiogram, and blood tests, according to the European Society of Cardiology guidelines. 16 The study excluded patients with chronic thromboembolic pulmonary hypertension (CTEPH) who were candidates for pulmonary endarterectomy. Inoperable CTEPH patients were managed as PAH patients, after Ethical Committee approval, considering the malignant nature of the disease and lack of approved therapies available for this particular sub-group.…”

“…[3][4][5] In the following years, other prostanoids (sub-cutaneous [SC] treprostinil 6 and aerosolized iloprost 7 ), and 2 classes of oral drugs, the endothelin (ET)-receptor antagonists bosentan, 8 sitaxsen-tan, 9 ambrisentan, 10 and the phosphodiesterase type 5 (PDE-5) inhibitors sildenafil 11 and tadalaifil, 12 were shown to improve exercise capacity and hemodynamics. Despite these achievements, several studies proved that after an initial period of improvement, clinical worsening is a common experience among patients treated with oral drugs, 10,[13][14][15][16] leading to a clinical need for parenteral prostanoid as an add-on therapy. In this perspective, timing for parenteral prostanoid therapy becomes a critical challenge.…”