Referral Patterns and Clinical Characteristics of Patients With Pulmonary Hypertension at a Comprehensive Care Center

Pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) are life-threatening forms of pulmonary hypertension (PH) that require early recognition and referral to expert centers. In 2014, the Pulmonary Hypertension Association (PHA) launched the Pulmonary Hypertension Care Centers (PHCC) initiative to improve access to specialized evaluation and guideline-directed therapy. A decade later, real-world data evaluating referral patterns and time to diagnosis remain limited. We conducted a retrospective analysis of patients referred to Houston Methodist PHCC between January 2022 and December 2023 for suspected or confirmed PH. Of 274 referred patients, 97 were confirmed to have PAH or CTEPH and included in this study. Demographics, referral sources, presenting symptoms, diagnostic and treatment timelines, and risk stratification by REVEAL Lite 2 score were analyzed. At the time of referral, 66% patients lacked a defined group classification, 18% were labeled as PAH, and 13% as CTEPH. Following a comprehensive evaluation, 76 (28%) were confirmed as PAH and 21 (8%) as CTEPH, whereas 14% were determined not to have PH. At presentation, 58% of patients were in WHO FC III or IV, and 63% of patients with PAH were categorized as intermediate or high risk. Median time from symptom onset to diagnosis was 13 months (IQR: 4-29), to treatment 16 months (IQR: 6-35), and to PHCC referral 24 months (IQR: 10-54). 30% of patients with PAH were not receiving PH-specific therapy, and 35% were on suboptimal regimens based on their risk status. Following PHCC evaluation, dual and triple therapy use increased to 50% and 36%. Despite the establishment of the PHCC network, diagnostic delays and therapeutic gaps persist, emphasizing the need for enhanced awareness, standardized referral pathways, and stronger collaboration between community providers and PHCCs.