Hispanic patients with pulmonary arterial hypertension: Preliminary results from a contemporary national registry

Objective: To analyze data prospectively collected from Mexican-mestizo patients who participated in randomized clinical trials between 2000-2016 in Mexico City. Results: Only data from 100 patients is presented. Idiopathic (iPAH, 60%), connective tissue disease PAH (CTD, 15%) and congenital heart disease-PAH. The latter was divided in corrected(12%) and non-corrected patients(n=13). 2% of iPAH were vasoresponders. Median age at RHC was 29 years, but iPAH and CTD tended to be older. BMI was 23.84 with no differnece between groups. Thyroid disorders were most frequent co-morbidity. In RHC, mPAP was signficantly higher in congenital-PAH group than other types. iPAH had lowest median cardiac output. 22% of patients were diagnosed before 2000, but only 6.1% treated with nifedipine before 1998. All patients received PAH pharmacotherapy, but time from diagnostic RHC to first use of drug was significantly different between and within groups. Endothelin receptor antagonists were most frequent drug used, followed by PDE-5 inhibitor. Except for CTD-PAH, significant improvement in functional class was found for all groups within 1-year after treatment. 30-53% received combination therapy. Corrected congenital-PAH had longest median time until addition of second drug, followed by iPAH and CTD. 10-40% had a drug class switch due to lack of supply, end of randomized or extended open-label trial phase. Cumulative survival from drug start to death (or censored at last oupatient visit) for 1,3,5 and 10 years were as follows: iPAH excluding vasoresponders (100%,95%,93%,87%), CTD (77%,66%,55%,0%) and corrected-congenital PAH (100%,92%,83%,75%). All Eisenmenger9s remain alive.