PURPOSE: With the number of FDA-approved treatments for pulmonary arterial hypertension (PAH) increasing yearly, current and reliable information about PAH management is needed. The REVEAL Registry is a multicenter, observational, U.S.-based study that should meet this need. One objective of the registry is to characterize treatment of patients diagnosed with PAH.
METHODS: All consenting patients with PAH are being enrolled at 50 sites in the US. Patients will be followed for a minimum of five years from the time of enrollment. Enrollment data include thorough clinical and treatment history, physical examination, and assessment of disease severity.
RESULTS: Among the initial 1226 patients, at the time of enrollment 8% were receiving no PAH-specific medications, 44% received only oral medications, and 31% received a prostacyclin combined with other medications. PAH-specific medications included: 9%, calcium channel blocker (CCB); 44%, prostacyclin analogue; 47%, endothelin receptor antagonist; and 46%, phosphodiesterase inhibitor. 47% of the patients were treated with monotherapy (bosentan, 13%; sildenafil, 13%; epoprostenol IV, 8%; sitaxsentan, 2%, and CCB, 4%). 36% received 2 drug combination therapy (epoprostenol IV + sildenafil, 8%; bosentan + sildenafil, 8%; bosentan + epoprostenol, 3%; bosentan + inhaled iloprost, 3%; and sildenafil + inhaled iloprost, 2%), and 9% received 3 or more PAH-specific medications. Functional class was only significantly associated with prostacyclin therapy (FC I, 24%; FC II, 40%; FC III, 50%; FC IV, 61%; P<0.001).
CONCLUSION: Eight percent of patients enrolled in the registry are not receiving any PAH-specific medication. Forty-four percent of the patients are receiving only oral medications. Combination therapy, without supporting data from clinical trials, is already frequently used to treat patients with PAH.
CLINICAL IMPLICATIONS: Despite the absence of rigorous evidence supporting multi-drug therapy, we anticipated and found a diverse array of treatment strategies. Further studies are needed to determine the optimal strategy initiating combination therapy in patients with PAH.
DISCLOSURE: Michael McGoon, No Product/Research Disclosure Information; Grant monies (from industry related sources) Robyn Barst has received research grants from CoTherix, Encysive, Myogen, Pfizer, and United Therapeutics; Employee Kathleen Feldkircher is an employee of Actelion; Consultant fee, speaker bureau, advisory committee, etc. Michael McGoon and Ramona Doyle have served as consultants for Actelion. Robyn Barst has served as a consultant or on an advisory board for GlaxoSmithKline, United Therapeutics, CoTherix, Actelion, Encysive, Pfizer, ICOS/Lilly. Ted Liou has served as a Steering Committee member for Actelion; Other David Miller has received payment from Actelion through a Master Services Agreement.