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.

Case Report: Sarcoidosis–associated pulmonary hypertension (SAPH) treated with sildenafil. Development of SAPH significantly worsens prognosis in sarcoidosis patients and here, we report a case of a patient with severe SAPH treated with sildenafil. PubMed, Adv Respir Med. (Also see Sarcoidosis)

Combination Therapies

Comparison Of The Efficacy Of Sildenafil Alone Versus Sildenafil Plus Bosentan In Newborns With Persistent Pulmonary Hypertension. The combined use of sildenafil and bosentan is more effective than sildenafil alone for control of pulmonary hypertension in resource limited centres. PubMed, J Ayub Med Coll Abbottabad, Jul-Sep 2018;30(3):333-336.

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).


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.

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