Abstract Background Chronic thromboembolic pulmonary hypertension (CTEPH) is consequence of organized thrombotic material adhered to the pulmonary arteries associated with proliferative arteriolar disease that leads to increased pulmonary vascular resistance (PVR), right heart failure and early death if not adequately detected and treated. Furthermore, CTEPH is the only form of pulmonary hypertension (PH) that has a potentially curative treatment. This registry aimed to evaluate the comprehensive management and survival of a large cohort of patients with CTEPH in a national and European reference center for PH. Methods This registry brings together 804 patients diagnosed with CTEPH evaluated by a multidisciplinary team in the PH Unit from 1996 to 2023. In this way, it is defined whether the patient is suitable for pulmonary endarterectomy (PE); pulmonary artery balloon angioplasty (BPA) or medical treatment (MT). The characteristics of each treatment group, the clinical and hemodynamic results of each intervention, as well as long-term survival are described. A 10-year follow-up was carried out. Results From 1996 to 2023, 804 patients with CTEPH were evaluated, of which 50.2% (404) were suitable for PE. BPA started in 2013 and was performed in 201 (25%) patients, the rest received only medical treatment with pulmonary vasodilator drugs (199 patients, 25%). Before BPA was initiated in 2013, 78 PE surgery were performed, while 55 patients received MT. After 2013, 326 patients underwent to PE, 201 received BPA and 144 patients MT. In the total population, the mean age was 59.1±14 years and male sex predominated (62.8%). Patients undergoing PE were the youngest, 54.4±13.6 vs 60.9±15.1 and 65.7±13.7 years for BPA and MT respectively, p0.0001 and male sex predominated, unlike for BPA and MT; 56.9% vs 38.8% vs 37.2%, p0.0001. Surgical patients had a lower prevalence of malignancies than BPA and MT, 6% vs 15.4% vs 15.6% p0.0001. At the time of diagnosis in the three groups, functional class III/IV (WHO) was the most frequent, especially in the PE group 72.3% vs 67.7% vs 68% p0.0001. The clinical and hemodynamic results of each treatment are shown in Table 1. Hospital mortality was 4.5% and 1.5% for PE and BPA respectively. The survival of patients with CTEPH at 1, 3 and 5 years is 99%, 98% and 95% for PE; 99%, 97% and 95% for BPA and 97%, 88% and 80% for medical treatment, respectively. Long Rank p 0.0001 (Figure 1). Conclusions PE surgery is the treatment of choice in CTEPH, as demonstrated by the excellent long-term survival of patients. BPA is an option in inoperable patients, with results superior to medical treatment. Thus, comprehensive and multidisciplinary management of CTEPH in an expert center guarantees the best results for patientsTable 1 Figure 1
Coronel et al. (Sat,) studied this question.