Abstract
TRIO Health PH registry differentiates from existing registry populations (COMPERA/ASPIRE/REVEAL) in consisting of patients managed in the modern era. TRIO Health PH registry contains retrospective clinical and prescription data from eight US tertiary care centers. Descriptive and statistical analyses (ANOVA, Tukey’s studentized range procedure, Chi-square or Fisher’s exact test) were conducted for 725/1000 registry patients, enrolled July 2019-October 2020, among group 1 - pulmonary arterial hypertension (PAH) and group 4 - chronic thromboembolic pulmonary hypertension (CTEPH). Study etiologic subgroups: idiopathic (IPAH, 34%, 243), connective tissue disease (APAH-CTD, 29%, 210), Drugs & Toxins (11%, 80), congenital heart disease (APAH-CHD, 6%, 46), and CTEPH (5%, 37) At enrollment, patients had NYHA functional class II (46%, 253/554) or III (42%, 233/554) symptoms Common comorbidities were: Hypertension in Drugs & Toxins (51%, 41), CTEPH (49%, 18), IPAH (39%, 95), APAH-CTD (38%, 79); obstructive sleep apnea in APAH-CHD (17%, 8), Overweight/obesity among CTEPH (81%, 30) and Drugs & Toxins (75%, 60) Mean 6-minute walking distance (6MWD) was highest for Drugs & Toxins (381.3 m , n=73) and lowest for APAH-CTD (301.8 m, n=189). Mean pulmonary arterial pressure (mPAP) was higher in APAH-CHD (48.6 mmHg, n=41) and Drugs & Toxins (47.9 mmHg, n=75) compared to APAH-CTD (40.6 mmHg, n=196) and CTEPH (38.6 mmHg, n=33). Mean pulmonary vascular resistance (PVR) was significantly higher in IPAH (366.7 dynes-sec/m5, n=212) vs. CTEPH (136.2 dynes-sec/m5, n=33). Mean diffusing capacity for carbon monoxide (DLCO), % predicted was lowest in CTD (40.6%, n=118) and IPAH (53.8%, n=155). Overall cohort enrollment characteristics, including NYHA functional class II/III, reduced 6MWD (<400m), and elevated BNP, indicate a compromised group at intermediate risk for clinical progression. Continued evaluation will elucidate disease impact on survival, current clinical practices, and place for precision medicine.
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