Temporal evolution of multidisciplinary management for chronic thromboembolic pulmonary hypertension: a 30-year experience at a refererral center

Abstract

Chronic thromboembolic pulmonary hypertension (CTEPH) management has significantly evolved over the last decades. Pulmonary endarterectomy (PEA) remains the first-line and potentially curative therapy. However, technological and medical advances have enabled incorporation of new therapeutic strategies for patients who are not surgical candidates. Pulmonary angioplasty (BPA) and targeted medical therapies (MT) have broadened the therapeutic landscape, offering effective options for non-operable cases. To analyse the temporal evolution of comprehensive management of CTEPH at a high-volume reference center. We analysed a 30-year registry from our hospital, a designated as a national and European reference center for pulmonary hypertension. A total of 928 patients diagnosed between 1996 and 2025 were evaluated. This study analysed the temporal transition in therapeutic strategies, from the selection of candidates for PEA, the progressive introduction of BPA beginning in 2013, and the development of targeted medical therapies for CTEPH. Two cohorts were established: the first (C-1) covering the initial experience in CTEPH management during the period 1996-2014, and the second cohort (C-2) includes the period 2015-2025, characterized by the progressive consolidation of BPA and MT. Specific characteristics of each treatment group, as well as clinical and hemodynamic outcomes achieved after each intervention in both cohorts, were described. Long-term survival was evaluated with a 5-year follow-up. Between 1996 and 2025, a total of 928 patients with CTEPH were evaluated, with a mean age of 60.2±14.7 years, and 52.7% were female. C-1 included 200 patients (21.7% of total), of whom 111 (55.5%) underwent PEA and 81 (40.5%) received only MT. BPA program began in 2013, with 8 patients undergoing the procedure during this initial period. C-2 included 728 patients, who predominantly received interventional strategies, 49.2% underwent PEA and 38% BPA, only 8.1/ received MT alone. In both periods, interventional therapies significantly improved functional capacity, biomarkers and hemodynamic parameters (Table 1). Peri-procedural mortality decreased from 6.6% in C-1 to 2.8% in C-2 (p=0.03), reflecting the institutional learning curve. Overall survival at 1, 3 and 5 years was significantly higher in the contemporary cohort (C-2: 96%, 93% and 90%) compared to the initial cohort (C1: 92%, 85% and 78%) (Log-Rank p<0.0001) (Figure 1) Over thirty years, CTEPH management has undergone substantial transformation. PEA remains the cornerstone therapy with stable indications despite the introduction of BPA, which has become a vital complementary intervention. Both strategies yield excellent functional and hemodynamic outcomes. Furthermore, the consolidation of targeted therapies and multidisciplinary care has significantly improved long-term survival in non-surgical candidatesTable 1For image description, please refer to the figure legend and surrounding text.Figure 1:Cumulative survivalFor image description, please refer to the figure legend and surrounding text.