hronic thromboembolic pulmonary hypertension (CTEPH) is a relatively rare disease. Its natural history and etiology remain unclear. [1][2][3][4][5] There have been a number of reports that pulmonary thromboendarterectomy (PTE) is an effective modality for treatment in selected patients with CTEPH. [1][2][3][6][7][8][9][10][11] However, the hemodynamic benefit varies according to the location and extent of the thromboembolic occlusion, and Bergin and colleagues reported that the computed tomography (CT) angiographic extent of central disease (ie, CD score) is related to a low pulmonary vascular resistance (PVR) after surgery. 12 Our preliminary data also showed high operative mortality with CD scores of 0 (25%) and 1 (20%), compared with those with a CD score of ≥2 (7.7%), so we classified those patients with a CD score of ≤1 as having relatively peripheral type CTEPH. The cause of operative death was related to residual pulmonary hypertension as a result of failure to remove a distal obstruction. New guidelines have recommended a post-surgical estimated reduction in PVR of >50%. 10 The validity of PTE might be limited to central-type patients, especially in institutions in which the operation is performed infrequently.The prognosis of CTEPH in the medically treated group had been thought to be poor, 13-15 but Ono et al reported that oral beraprost sodium improved their survival. 16 Recently, there have been some reports about improved 6-min walk distance and pulmonary hemodynamics after epoprostenol, 17,18 sildenafil 19 and bosentan [20][21][22] in patients with CTEPH. These new drugs might improve vascular remodeling, and may offer improved survival in patients with relatively peripheral type CTEPH, in whom we predicted a poor reduction in PVR after surgery. We retrospectively tested the validity of PTE from the aspects of survival and quality of life (QOL) at long-term follow up in patients with relatively peripheral type CTEPH.
Methods
PatientsBetween April 1999 and March 2006, a total of 83 patients admitted consecutively to Chiba University Hospital were diagnosed as having CTEPH and evaluated for surgical indication. CTEPH was defined as having a mean pulmonary arterial pressure (PPA) of ≥25 mmHg with normal wedge pressure in patients who had dyspnea on exertion during a period of more than 6 months. In addition, lung