PURPOSE: A primary objective of the Registry to EValuate Early And Long-term Pulmonary Arterial Hypertension (PAH) Disease Management (REVEAL) is to determine outcomes in patients with PAH. Analysis of PAH-specific therapy at time of death could provide information on the aggressiveness of therapy and the adherence to recommended treatment guidelines in patients with the most severe disease. In this study, we compared functional class assessments prior to death and parenteral prostanoid administration at time of death to determine whether PAH patients were treated according to recommendations.
METHODS: Of 3518 patients enrolled in REVEAL, 2912 met the inclusion criteria: age ≥18 years at enrollment, pulmonary capillary wedge pressure ≤15 mmHg at diagnostic right heart catheterization, no active participation in a blinded clinical trial, and no history of atrial septostomy or transplant.
RESULTS: 794 patients died (median period between last functional class (FC) assessment and death, 3.76 months); of these, 342 (43%) were receiving a parenteral prostanoid (either monotherapy or combination therapy) and 65 (8%) were receiving no therapy. Among patients whose FC was assessed ≤6 months prior to death (n=482), 48% (n=233/482) were FC III and 37% (n=178/482) were FC IV. 40% of these FC IV patients (n=71/178) were not receiving a parenteral prostanoid at time of death, including 5% (n=9/178) who were untreated. 284 patients died without FC assessment within 6 months of death. 57% (n=162/284) were FC III at their last assessment; and 14% (n=41/284) were FC IV; 46% (n=19/41) of these FC IV patients received parenteral prostanoids before death.
CONCLUSIONS: At time of death, the majority of patients were most recently assessed as FC III. Even when assessed as FC IV, a substantial percentage had not been treated with parenteral prostanoids.
CLINICAL IMPLICATIONS: FC III patients with PAH require more frequent monitoring and more aggressive treatment in an attempt to prevent clinical decline. Importantly, parenteral prostanoid therapy frequently is not utilized in FC IV patients prior to their death.
DISCLOSURE: Harrison Farber: Consultant fee, speaker bureau, advisory committee, etc.: Actelion, Gilead, United Therapeutics
Dave Miller: Other: Employed by ICON Late Phase & Outcomes Research, a company that receives research support from Actelion and other pharmaceutical companies
Faith Beery: Other: Employed by ICON Late Phase & Outcomes Research, a company that receives research support from Actelion and other pharmaceutical companies
Michael McGoon: Consultant fee, speaker bureau, advisory committee, etc.: Actelion, Gilead, Lung Rx, GlaxoSmithKline, and Medtronic, Grant monies (from industry related sources): Gilead and Medtronic
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