Introduction: Recent studies suggest that medically treated patients with inoperable chronic thromboembolic pulmonary hypertension (CTEPH) have an improved prognosis. However, only limited data are available concerning predictors of mortality in these patients. The aim of this study was to assess, and to identify, predictors of the long-term outcome of inoperable CTEPH patients.
Methods: We analysed 84 inoperable CTEPH patients referred to our centre between 1999 and 2008. During follow-up (mean 32 months), 17 patients died and one underwent a lung transplantation. The 1-, 3- and 5-year survival rates were 93, 78 and 68%, respectively. Univariate analysis demonstrated that 6-min walking distance (6MWD), mean pulmonary artery pressure (mPAP), right atrial pressure (RAP) and pulmonary vascular resistance (PVR) were predictive factors for survival. In the multivariate analysis only 6MWD was independently related to poor survival (hazard ratio 0.995; 95% CI, 0.991-0.998; P=0.003). Kaplan-Meier curves showed that patients with an mPAP>40 mmHg, PVR>584 dyn s cm(-5) and RAP>12 mmHg had a very poor prognosis.
Conclusions: Haemodynamic parameters (mPAP, RAP, PVR) and the 6MWD at baseline are predictive factors for mortality of medically treated inoperable CTEPH patients. A subgroup of these patients with good prognostic factors, defined by their haemodynamics and clinical measures, have an improved long-term survival and outcome.