Predictors of mortality in inoperable chronic thromboembolic pulmonary hypertension

Saouti, Nabil; de Man, Frances; Westerhof, Nico; Boonstra, Anco; Twisk, Jos; Postmus, Pieter E.; Noordegraaf, Anton Vonk

doi:10.1016/j.rmed.2009.01.017

« Previous Next »Respiratory Medicine
Volume 103, Issue 7 , Pages 1013-1019, July 2009

Predictors of mortality in inoperable chronic thromboembolic pulmonary hypertension

Nabil Saouti
- Department of Pulmonary Diseases, Institute for Cardiovascular Research, VU University Medical Center, Amsterdam, The Netherlands
Frances de Man
- Department of Pulmonary Diseases, Institute for Cardiovascular Research, VU University Medical Center, Amsterdam, The Netherlands
Nico Westerhof
- Department of Pulmonary Diseases, Institute for Cardiovascular Research, VU University Medical Center, Amsterdam, The Netherlands
- Department of Physiology, Institute for Cardiovascular Research, VU University Medical Center, Amsterdam, The Netherlands
Anco Boonstra
- Department of Pulmonary Diseases, Institute for Cardiovascular Research, VU University Medical Center, Amsterdam, The Netherlands
Jos Twisk
- Clinical Epidemiology and Biostatistics, VU University Medical Center, Amsterdam, The Netherlands
Pieter E. Postmus
- Department of Pulmonary Diseases, Institute for Cardiovascular Research, VU University Medical Center, Amsterdam, The Netherlands
Anton Vonk Noordegraaf
- Department of Pulmonary Diseases, Institute for Cardiovascular Research, VU University Medical Center, Amsterdam, The Netherlands
- Corresponding author. Department of Pulmonary Diseases, VU University Medical Center, De Boelelaan 1117, PO Box 7057, 1007 MB, Amsterdam, The Netherlands. Tel.: +31 204444327; fax: +31 204444328.

Received 19 August 2008; accepted 18 January 2009. published online 23 February 2009.

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 32months), 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>40mmHg, PVR>584dyn s cm⁻⁵ and RAP>12mmHg 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