PURPOSE: Pulmonary arterial hypertension (PAH) is a devastating disease with a variety of treatment options available, depending upon severity and etiology. Patients who are more severely ill receive infusion therapy. There is a paucity of data in the literature on the potential for transitioning patients requiring IV/SQ prostacyclin to alternate therapies. We report our experience transitioning patients with PAH from IV/SQ prostacyclin therapy to oral and/or inhaled therapies.
METHODS: Retrospective chart review on all patients transitioned from IV/SQ therapy from 2002 to 2010.
RESULTS: A total of 49 adult patients (38 female), mean age 54.6 years (range 19-83), with PAH of all causes (23 idiopathic, 16 collagen vascular disease, 7 congenital heart disease, 1 pulmonary emboli, 1 hepatopulmonary syndrome, 1 valvular disease) were transitioned from IV/SQ prostacyclin therapy (38 by physician assessment, 6 by patient demand, 5 due to severe side effects). Of these, 14 patients were transitioned to oral endothelin receptor antagonists (ERAs), 11 to oral phosphodiesterase-5 (PD5) inhibitors, 4 to inhaled medication, 14 to a combination of ERA/PD5, 1 to ERA/inhaled, 4 to PD5/inhaled and 1 to ERA/PD5/inhaled. Six months after transition, 43 patients continued on oral/inhaled medications, 2 died of right heart failure, 1 was lost to follow-up, 1 returned to IV prostacyclin, 1 died from non-PAH cause and 1 was taken off medication completely (mitral valve-related PAH). Only 2 patients required additional noninfusion medications.
CONCLUSIONS: Of the 49 patients, 29 (59.2%) went to a single drug, 19 (38.8%) received 2 drugs and 1 went to triple-drug therapy. Forty-four patients (89.8%) were successfully transitioned off infusion therapy. Of the remaining 5, 1 patient required reinstitution of IV therapy, 1 patient was lost to follow-up and 3 patients died (2 from right heart failure, 1 accidental). Conversion from IV/SQ prostacyclin is a feasible option in select patients with PAH.
CLINICAL IMPLICATIONS: Criteria for transition will be reviewed. Pooled data from high-volume centers would be helpful for future treatment recommendations.
DISCLOSURE: The following authors have nothing to disclose: Dianne Zwicke, Mary Wenzel, Julie Cavey
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