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A6343 - Long-Term Outcomes of Patients with Chronic Thromboembolic Pulmonary Hypertension at a Single Japanese Pulmonary Endarterectomy Center
Author Block: H. Miwa, N. Tanabe, T. Jujo, F. Kato, R. Anazawa, K. Yamamoto, A. Naito, H. Kasai, R. Nishimura, T. Sugiura, S. Sakao, K. Tatsumi; Respirology, Graduate School of Medicine, Chiba University, Chiba, Japan.
Background: Several new treatments such as balloon pulmonary angioplasty (BPA), and selective pulmonary vasodilators for chronic thromboembolic pulmonary hypertension (CTEPH) have appeared in recent years, which have led to changes in the treatment algorithm. However, changes in survival rates and prognostic factors have not been estimated so far. Methods: Two hundred-eighty patients were diagnosed with CTEPH at Chiba University Hospital between June 1986 and June 2016. In our series, selective pulmonary vasodilators (defined as phosphodiesterase-5 inhibitors, endothelin receptor antagonist, epoprostenol, or riociguat) have been available since 1999, and BPA was first performed in 2009. Survival rate was investigated by date of treatment initiation (Group 1, 1986-1998; Group 2, 1999-2008; Group 3, 2009-2016). Survival rates were also evaluated by treatment strategy; balloon pulmonary angioplasty (BPA), pulmonary endarterectomy (PEA), and medical treatment. The BPA and Medical group was also defined as non-PEA group. Prognostic factors were evaluated in the PEA and non-PEA group. The Kaplan-Meier method was used to estimate disease-specific and overall survival, with the log-rank test for comparisons. Univariate and multivariate Cox proportional hazard models were used to examine prognostic factors. Results: Group 3 showed significantly better disease-specific survival than Groups 1 and 2 (p=0.0002, p=0.0045, respectively). The 5-year survival was 91.9% for the Group 3, 67.1% for the Group 1, and 77.0% for the Group 2, respectively. In the non-PEA group, Group 3 had better disease specific survival than Groups 1 and 2 (p=0.0001, p=0.0061, respectively). The-5 year survival was 94.9% for the Group 3, 54.6% for the Group 1, and 74.2% for the Group 2, respectively. The PEA group showed significantly better survival than the Medical group in Groups 1 and 2 (p=0.0375, p=0.0389, respectively), whereas no difference was observed among the BPA, PEA, and Medical groups in Group 3. In the non-PEA group, Group 3, low PVR, World Health Organization functional class (1-2), and selective pulmonary vasodilators were independently associated with survival. In the PEA group, multivariable analysis showed that low pulmonary vascular resistance (PVR) and Jamieson classification (type 1-2) were independently associated with better disease-specific survival. Conclusion: Survival of patients with CTEPH in recent era has significantly improved, especially in the non-PEA group. BPA and selective pulmonary vasodilators could improve survival of the non-PEA group. In the present study, no difference in survival was found between the PEA and non-PEA groups.