Elsevier

Respiratory Medicine

Volume 103, Issue 7, July 2009, Pages 1013-1019
Respiratory Medicine

Predictors of mortality in inoperable chronic thromboembolic pulmonary hypertension

https://doi.org/10.1016/j.rmed.2009.01.017Get rights and content
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Summary

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.

Keywords

Chronic thromboembolic pulmonary hypertension
Prognostic factors
Survival

Abbreviations

6MWD
6-min walking distance
AMC
associated medical conditions
CTEPH
chronic thromboembolic pulmonary hypertension
ERA
endothelin receptor antagonist
mPAP
mean pulmonary artery pressure
NT-proBNP
N-terminal pro brain natriuretic peptide
PDE-5 inhibitor
phosphodiesterase-5 inhibitor
PVR
pulmonary vascular resistance
PEA
pulmonary endarterectomy
RAP
right atrial pressure
ROC
receiver operating characteristics
RVP
right ventricular pressure
SvO2
mixed venous oxygen saturation

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