Researchers Share Insights From Patients Enrolled in Innovative PAH Repository

Researchers have published data on the first group of patients with pulmonary arterial hypertension (PAH) enrolled in a novel approach for data collection on the condition that allows for real-world analysis of treatment- and disease-related outcomes, including the impact of treatment on outcomes and evolving characteristics of patients with pulmonary hypertension.

Findings were published in Pulmonary Circulation.

Called the TRIO comprehensive, integrated patient data repository (TRIO CIPDR), the unique registry combines clinical data from electronic health records with pharmacy data, including PAH-specific drugs prescribed, as well as the dates prescribed, administered, and refilled. The database collects data on patients coming from Pulmonary Hypertension Association–accredited care centers and is integrated with patient outcomes, such as hospitalizations and mortality pulled from Health Insurance Portability and Accountability Act–compliant data.

“A benefit of TRIO CIPDR is that it will provide longitudinal data on treatment patterns and outcomes in patients with PAH in a contemporary setting where newer treatments are available, with stronger evidence and recommendations for combination therapy that became available after the completion of most PAH disease registries, including REVEAL,” described the researchers. “TRIO CIPDR is expected to provide novel insights into potential barriers to treatment access and information on ways economic factors may impact treatment consistency, adherence, and outcomes in patients with PAH (eg, the potential relationship between increased medication cost and failure to collect dispensed PAH treatments, and the impact of treatment interruptions on outcomes such as hospitalizations).”

In their paper, the researchers detailed findings from 940 adults with PAH enrolled in the repository between the start of 2019 and end of 2020. The comorbidities of patients generally matched those expected of patients with PAH, although the prevalence of atrial fibrillation was higher, with 34% of patients having the condition.

Thirty-eight percent of patients had idiopathic PAH and 30% had connective tissue disease–related PAH. Age at diagnosis and at enrollment, as well as time from diagnosis to enrollment, were similar between the 2 etiologies. Most (917) patients were receiving treatment for their PAH at the time of analysis, with 40% receiving monotherapy; 43%, double therapy; and 17%, triple therapy.

The researchers compared these characteristics against those seen in the Registry to Evaluate Early and Long-term PAH Disease Management (REVEAL) registry, as well as 2 similar registries to the TRIO CIPDR: the United States Pulmonary Hypertension Scientific Registry (USPHSR) and the Pulmonary Hypertension Association Registry (PHAR).

“Patient characteristics at enrollment in TRIO CIPDR had similarities and differences to REVEAL and the contemporary USPHSR and PHAR registries,” commented the researchers. “The majority of patients with PAH were female in TIRO CIPDR, REVEAL, USPHSR, and PHAR (75%, 79%, 79%, and 76%, respectively). Patients in TRIO CIPDR had a mean age of 53 years at diagnosis, which is similar to the mean age of patients at diagnosis in REVEAL and USPHSR (mean 50 and 52 years, respectively). Mean age at diagnosis in PHAR was not reported but was 56 years at enrollment.”

Median time from diagnosis to enrollment varied across the datasets, with a median time of 5, 2, and 4 years for the TRIO CIPDR, REVEAL, and USPHR registries, respectively. The drivers behind the differences are not clear, although the researchers noted that differences in diagnosis and enrollment could have played a role.

References

Farber H, Chakinala M, Cho M, et al. Characteristics of patients with pulmonary arterial hypertension from an innovative, comprehensive real-world patient data repository. Pulm Circ. Published online July 6, 2023. doi:10.1002/pul2.12258