P4674Implementation of the new chronic thromboembolic pulmonary hypertension treatment algorithm: an ongoing effort from a portuguese referral centre

Abstract

Abstract Background To date, the first-line treatment for chronic thromboembolic pulmonary hypertension (CTEPH) is pulmonary endarterectomy (PEA), although a significant number of patients will have inoperable disease or residual pulmonary hypertension (PH). Balloon pulmonary angioplasty (BPA) has provided a new therapeutic option for these patients. In addition, medical therapy (MT) also plays an important role. Purpose Characterization of a population of patients with CTEPH or chronic thromboembolic disease (CTED) and comparison of the different treatment strategies according to the updated treatment algorithm. Methods Retrospective analysis of patients with CTEPH/CTED followed in a referral centre for the treatment of PH submitted to different treatment strategies: PEA plus MT and BPA in patients with residual PH (group 1), MT plus BPA (group 2), and MT only (group 3). Cox regression was used to identify predictors of all-cause mortality. Results 58 patients were included (median age 63.5 years, 74% female); 17% had CTED, and the remaining had CTEPH 50% (n=29) were submitted to PEA (group 1), of which 58% had residual PH (21% underwent BPA, n=3). Among the remaining patients, 31% (n=9) underwent MT plus BPA (group 2), and 69% (n=20) were treated with MT only (group 3). Overall, 55% were under pulmonary vasodilator therapy, including 38% with riociguat. Most of the patients (67%) were in functional class III or IV, the distance in the 6MWT was 328±147 meters, the median NT-proBNP was 538pg/ml, 40% had RV systolic dysfunction, the mean mPAP was 42±13 mmHg, and the mean PVR was 11±6 WU. Comparing the different treatment strategies, during follow-up (median 945 days) the following differences were found (comparison between group 1 vs group 2 vs group 3): improvement in functional class (class III-IV: 0% vs 0% vs 58%); distance in 6MWT (438±83 vs 390±79 vs 281±105 meters); evolution of NT-proBNP (−984±1736 vs −198±205 vs +1177±2342); normalization of RV dimensions (89% vs 50% vs 20%); resolution of pericardial effusion (100% vs 100% vs 0%); normalization of mPAP (73% vs 71% vs 0%); PVR (median 3.4 vs 2.7 vs 10.6 WU); all-cause mortality (7% vs 0% vs 35%) (p<0.02 in all). In the overall population, the most relevant predictors of all-cause mortality were the absence of functional class improvement, baseline and follow-up NT-proBNP, baseline and follow-up SPAP by echocardiogram, and maintenance of treatment with prostanoids (p<0.05 in all). Conclusion Our results confirm that, in patients with CTEPH/CTED, PEA is associated with functional, and hemodynamic improvement and increased survival, although BPA is an alternative in patients with inoperable disease or residual PH, with similar results on short-term follow-up. Patients who are not submitted to surgical or percutaneous intervention have a poor prognosis, both in terms of morbidity and mortality.