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Transitioning Patients With Pulmonary Arterial Hypertension From Parenteral Prostacyclin: Single-Center Experience

Z. Singsank

S. Paulus

A. Zeidler

N. Niebauer

TL Freichels

V. Thohan

Dianne Zwicke


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Conference: 2014 International PHA Conference and Scientific Sessions

Release Date: 06.21.2014

Presentation Type: Abstracts

File Download: 2014 Conference Abstract - Diane Zwicke

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Background: Pulmonary arterial hypertension (PAH) is a devastating disease with various treatment options dependent on disease severity and etiology. Among patients with severe symptoms, parenteral therapy with prostacyclin is lifesaving. However, there is a paucity of data in the literature on the potential for transitioning patients requiring parenteral therapy to alternate therapies. We report our single-center experience in transitioning patients with PAH from intravenous/subcutaneous (IV/SQ) prostacyclin therapy to oral and/or inhaled therapies.

Methods: We retrospectively reviewed charts of all patients transitioned from IV/SQ therapy from 2005 through 2013.

Results: A total of 62 patients (mean age 54.6 years [range 19-83], 49 females) with PAH (24 idiopathic, 20 collagen vascular disease, 14 congenital heart disease, 2 pulmonary emboli, 1 hepatopulmonary syndrome, 1 valvular) were transitioned from IV/SQ prostacyclin therapy (47 by physician assessment, 8 by patient demand, 7 due to severe side effects). Of these patients, 15 were transitioned to oral endothelin receptor antagonists (ERAs), 14 to oral phosphodiesterase inhibitors (PD5), 18 to a combination of ERA/PD5, 4 to inhaled, 3 to ERA/inhaled, 5 to PD5/inhaled and 3 to ERA/PD5/inhaled. Six months after transition, 56 (90.3%) patients continued on oral/inhaled medical therapies and 1 patient was off all therapies. There were 3 deaths (2 right heart failure, 1 noncardiopulmonary), 1 patient lost to follow-up and 1 returned to IV prostacyclin. The two right heart failure patient deaths occurred from the discontinuance of infusion therapy by patient demand.

Conclusions: Using serial (clinical, echocardiographic, hemodynamics) assessment, the majority of patients (56/62, 90.3%) with PAH were successfully transitioned to nonparenteral therapies (53.2% single-drug, 41.9% dual-drug, 4.8% triple-drug). Conversion from parenteral prostacyclin is a feasible option is selected patients with PAH.

Type: Clinical Science