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Implementation of the new Chronic Thromboembolic Pulmonary Hypertension treatment algorithm: an ongoing effort from a Portuguese referral centre
Session:
CO11 - Circulação Pulmonar / Congénitos
Speaker:
Sofia Alegria
Congress:
CPC 2019
Topic:
F. Valvular, Myocardial, Pericardial, Pulmonary, Congenital Heart Disease
Theme:
21. Pulmonary Circulation, Pulmonary Embolism, Right Heart Failure
Subtheme:
21.4 Pulmonary Circulation, Pulmonary Embolism, Right Heart Failure - Treatment
Session Type:
Comunicações Orais
FP Number:
---
Authors:
Sofia Alegria; Maria José Loureiro; Filipa Ferreira; Rita Calé; Margarida S. Cabral; Helder Pereira
Abstract
<p><strong>Background:</strong> 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.</p> <p><strong>Purpose</strong>: Characterization of patients with CTEPH or chronic thromboembolic disease (CTED) and comparison of the treatment strategies according to the updated treatment algorithm.</p> <p><strong>Methods:</strong> 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 A); MT plus BPA (group B); and MT only (group C). Cox regression was used to identify predictors of all-cause mortality.</p> <p><strong>Results: </strong>58 patients were included (median age 64 years, 74% female); 17% had CTED, and the remaining had CTEPH. 50% (n=29) were submitted to PEA (group A), of which 58% had residual PH (21% underwent BPA, n=3). Among the remaining patients, 31% (n=9) underwent MT plus BPA (group B), and 69% (n=20) were treated with MT only (group C). Overall, 55% were under pulmonary vasodilator therapy, including 38% with riociguat. 67% were in functional class III/IV, the distance in the 6MWT was 328±147 meters, the NT-proBNP was 1534±2049 pg/ml, 40% had RV systolic dysfunction, the mPAP was 42±13 mmHg, and the PVR was 11±6 WU. Comparing the treatment strategies, during follow-up (median 945 days) the following differences were found (comparison between group A vs B vs C): improvement in functional class (class III-IV: 0% vs 0% vs 58%); 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); and all-cause mortality (7% vs 0% vs 35%) (p<0.02 in all). Additionally, comparing with patients in group B, patients in group A were younger (median 58 vs 68 years; p=0.032), but there was no significant difference in the prevalence of residual PH or mortality.</p> <p>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 prostanoids (p<0.05 in all).</p> <p><strong>Conclusion: </strong>Our results confirm that in patients with CTEPH / CTED, PEA is associated with functional and hemodynamic improvement, and increased survival. 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.</p>
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