Abstract
Background: Although the key for the successful treatment of patients with chronic thromboembolic pulmonary hypertension (CTEPH) is pulmonary endarterectomy (PEA), the patients who are not indicated for surgical intervention and/or have comorbiditiies must therefore be medically treated. Recent new medical therapies (newTx), such as bosentan and sildenafil, may thus be able to improve the outcome of Japanese patients with CTEPH.
Purpose: To clarify the improved survival in CTEPH cases administered new Tx.
Methods: Between 1986 and 2010, 202 patients were diagnosed to have CTEPH at Chiba University Hospital. Ninety-nine patients underwent PEA, while 103 patients were medically treated. 56.5% of the patients diagnosed from 2005-2010 were treated by new Tx., 19.4% of those from 1999-2004, and 8.7% of those from 1986-1998 (p<0.0001). We investigated the long-term survival from the diagnosis and prognostic factors in medically-treated CTEPH cases and also examined survival from the initiation of the new Tx.
Results: The patients diagnosed from 2005-2010 showed a significantly improved survival (5-year survival: 87.8%) compared with those from 1999-2004 (72.2%) and from 1986-1998 (56.5%) (p=0.02). The 5-year survival from the initiation of bosentan (n=15) and sildenafil (n=24) was 90.0%, 84.2%, respectively. A multivariate analysis revealed a lower pulmonary vascular resistance (p<0.0001), no comorbidities (p=0.0009), a peripheral type (p=0.02), and recent patients (p=0.02) to be significantly better prognostic factors.
Conclusion: The survival in medically treated CTEPH cases was found to have improved due to the administration of new medical therapies.
- © 2011 ERS