Abstract
In patients who require urgent initiation of pulmonary arterial hypertension medications due to disease progression, it is customary to start intravenous prostacyclin therapy, typically during a hospital admission. If there are complicating factors or relative contraindications to intravenous and subcutaneous prostanoids, oral treprostinil provides another pathway to prostanoid therapy, but this usually requires a prolonged titration. We describe the case of a thirty-six-year-old male with severe pulmonary arterial hypertension and contraindication to intravenous and subcutaneous prostanoid therapy due to congenital deafness and the risk of not hearing the intravenous pump alarms. Intravenous treprostinil was initiated, titrated to high dose, and then rapidly transitioned to oral treprostinil. A rapid initiation, titration, and transition from intravenous to oral treprostinil can be safely performed under watchful supervision in order to achieve higher and more efficacious doses of oral treprostinil in a timely manner.
Highlights
Treprostinil is a tricyclic benzidine prostanoid approved for the treatment of World Health Organization (WHO) Group 1, Functional Classes II–IV pulmonary arterial hypertension (PAH)
Guidelines do not exist for the rapid initiation and titration of IV treprostinil with early rapid transition to oral treprostinil
We describe the case of a thirty-six-year-old male with severe pulmonary arterial hypertension who was successfully started on IV treprostinil, titrated to high dose, and rapidly transitioned to oral treprostinil on a background of ambrisentan and sildenafil
Summary
Treprostinil is a tricyclic benzidine prostanoid approved for the treatment of World Health Organization (WHO) Group 1, Functional Classes II–IV pulmonary arterial hypertension (PAH). It is reasonable to admit the patient to the hospital and start IV or subcutaneous prostanoid therapy. In patients with relative contraindication or complicating factors precluding intravenous prostanoids, oral treprostinil is an option, but titration typically involves a prolonged duration. We describe the case of a thirty-six-year-old male with severe pulmonary arterial hypertension who was successfully started on IV treprostinil, titrated to high dose, and rapidly transitioned to oral treprostinil on a background of ambrisentan and sildenafil. This was performed with close inpatient monitoring
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