Treatments for Pulmonary Arterial Hypertension: PDE-5 Inhibitors: Sildenafil Citrate (Revatio, Viagra)

Author: Shelley Ensz. Scleroderma is highly variable. See Types of Scleroderma. Read Disclaimer


Sildenafil citrate, which is also known by the brand names Revatio and Viagra, is a treatment for pulmonary arterial hypertension (PAH). PAH sometimes occurs as part of systemic scleroderma, which is a connective tissue disease.(Also see: What is PAH?, What is Scleroderma? and PAH Treatments)

Revatio (sildenafil) is a prescription medicine used in adults to treat pulmonary arterial hypertension (PAH). When taken as directed, Revatio may improve your ability to exercise and may slow down worsening changes in your physical condition. Pfizer.

Complex pathophysiology of pulmonary hypertension (PAH) associated with systemic sclerosis (SSc): potential unfavorable effects of vasodilators. Pre–assessment of pathophysiology of pulmonary hypertension and cautious monitoring during the treatment are necessary to achieve better outcomes in patients with SSc–PAH. Journal of Scleroderma and Related Disorders, JSRD 2017; 2(2): 92 - 99.

Combination Therapies

Case Report: Improvement of peripheral artery disease with Sildenafil and Bosentan combined therapy in a patient with limited cutaneous systemic sclerosis. Sildenafil and Bosentan combined therapy was associated in our case with an improvement of the maximum walking distance without adverse effect. PMC, Medicine (Baltimore), 2017 Jun; 96(25): e6988.

Pharmacokinetic and Pharmacodynamic Comparison of Sildenafil-Bosentan and Sildenafil-Ambrisentan Combination Therapies for Pulmonary Hypertension. Incremental shuttle walking distance was superior during treatment with Sildenafil plus Ambrisentan than during treatment with Sildenafil plus Bosentan. There were no concerns about safety with either combination therapy regime. Wiley, Clin Transl Sci, 2016 Feb;9(1):29-3.

Survival with sildenafil and inhaled iloprost in a cohort with pulmonary hypertension: an observational study. In this observational study of patients with pulmonary hypertension receiving combination therapy with iloprost and sildenafil, cumulative transplant–free survival was highest in those who received iloprost monotherapy initially. PubMed, BMC Pulm Med, 2016 Jan 12;16:5. (Also see Inhaled Prostacyclin)

Adding Bosentan to Sildenafil for PAH: What's the Verdict? Despite the failure of the present trial with respect to the primary endpoint, the improvement on the 6-minute walk suggests to the practicing physician that the sildenafil/bosentan combination may be worth a trial in a patient in whom monotherapy has not provided acceptable results. Medscape, 12/16/2015. (Also see: Bosentan)


Revatio (sildenafil) should be used with caution in children.

FDA Drug Safety Communication: FDA clarifies Warning about Pediatric Use of Revatio (sildenafil) for Pulmonary Arterial Hypertension. Our 2012 warning was to raise awareness of clinical trial results showing a higher risk of mortality in pediatric patients taking a high dose of Revatio when compared to pediatric patients taking a low dose. It was not intended to suggest that Revatio should never be used in children; however, some health care professionals have interpreted this information as a contraindication, and have refused to prescribe or administer the drug. We recognize there may be situations in which the benefit-risk profile of Revatio may be acceptable in individual children. FDA.

FDA Warns About Sildenafil in Kids with pulmonary arterial hypertension (PAH). The FDA cautioned late Thursday against using sildenafil (Revatio) to treat pulmonary arterial hypertension in patients younger than 17, after an unexpectedly high rate of mortality was seen among children receiving the drug in high doses in a clinical trial. MedPageToday, 08/31/12.

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