UCSF

Background

Pulmonary arterial hypertension (PAH) is characterized by elevated pulmonary arterial pressures and is managed by vasodilator therapies. Current guidelines encourage PAH management in specialty care centers (SCCs), but evidence is sparse regarding improvement in clinical outcomes and correlation to vasodilator use with referral.

Research question

Is PAH management at SCCs associated with improved clinical outcomes?

Study designand methods

A single-center, retrospective study was performed at the University of Pittsburgh Medical Center (UPMC; overseeing 40 hospitals). Patients with PAH were identified between 2008 and 2018 and classified into an SCC or non-SCC cohort. Cox proportional hazard modeling was done to compare for all-cause mortality, as was negative binomial regression modeling for hospitalizations. Vasodilator therapy was included to adjust outcomes.

Results

Of 580 patients with PAH at UPMC, 455 (78%) were treated at the SCC, comprising a younger (58.8 vs 64.8 years; P < .001) and more often female (68.4% vs 51.2%; P < .001) population with more comorbidities without differences in race or income. SCC patients demonstrated improved survival (hazard ratio, 0.68; P = .012) and fewer hospitalizations (incidence ratio, 0.54; P < .001), and provided more frequent disease monitoring. Early patient referral to SCC (< 6 months from time of diagnosis) was associated with improved outcomes compared with non-SCC patients. SCC patients were more frequently prescribed vasodilators (P < .001) and carried more diagnostic PAH coding (P < .001). Vasodilators were associated with improved outcomes irrespective of location but without statistical significance when comparing between locations (P > .05).

Interpretation

The UPMC SCC demonstrated improved outcomes in mortality and hospitalizations. The SCC benefit was multifactorial, with more frequent vasodilator therapy and disease monitoring. These findings provide robust evidence for early and regular referral of patients with PAH to SCCs.