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Original Research |

Echocardiography Combined with Cardiopulmonary Exercise Testing for the Prediction of Outcome in Idiopathic Pulmonary Arterial Hypertension

Roberto Badagliacca, MD, PhD; Silvia Papa, MD; Gabriele Valli, MD; Beatrice Pezzuto, MD; Roberto Poscia, MD, PhD; Giovanna Manzi, MD; Elisa Giannetta, MD, PhD; Susanna Sciomer, MD; Paolo Palange, MD; Robert Naeije, MD; Francesco Fedele, MD, FESC; Carmine Dario Vizza, MD
Author and Funding Information

Conflict of Interest Statement: RB has received fees for speaking activities and advisory boards from United Therapeutics, Dompe, GSK and Bayer. All other authors declare no conflicts of interest.

Dept. of Cardiovascular and Respiratory Science - Sapienza University of Rome, Italy

Dept. of Experimental Medicine - Sapienza University of Rome, Italy

ˆDepartment of Clinical Medicine - Sapienza University of Rome, Italy

§Department of Cardiology, Erasme University Hospital, Brussels, Belgium

Correspondence: Roberto Badagliacca, MD, PhD, Dept. of Cardiovascular and Respiratory Science, I School of Medicine, Sapienza University of RomePoliclinico Umberto I, Viale del Policlinico 155 - 00161 Rome, Italye


Copyright 2016, American College of Chest Physicians. All Rights Reserved.


Chest. 2016. doi:10.1016/j.chest.2016.07.036
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Abstract

Background  Right ventricular (RV) function is a major determinant of exercise intolerance and outcome in idiopathic pulmonary arterial hypertension (IPAH). The aim of the study was to evaluate the incremental prognostic value of echocardiography of the RV and cardiopulmonary exercise testing (CPET) on long-term prognosis in these patients.

Methods  One hundred-thirty treatment-naïve IPAH patients were enrolled and prospectively followed. Clinical worsening (CW) was defined by a reduction in 6-minute walk distance plus an increase in functional class, or non elective hospitalization for PAH, or death. Baseline evaluation included clinical, hemodynamic, echocardiographic and CPET variables. Cox regression modeling with c-statistic and bootstrapping validation methods were done.

Results  During a mean period of 528 ± 304 days, 54 patients experienced CW (53%). Among demographic, clinical and hemodynamic variables at catheterization, functional class and cardiac index were independent predictors of CW (Model-1). With addition of echocardiographic and CPET variables (Model-2), peak O2 pulse (peak VO2/heart rate) and RV fractional area change (RVFAC) independently improved the power of the prognostic model (AUC: 0.81 vs 0.66, respectively; p=0.005). Patients with low RVFAC and low O2 pulse (low RVFAC + low O2 pulse) and high RVFAC+low O2 pulse showed 99.8 and 29.4 increase in the hazard ratio, respectively (relative risk -RR- of 41.1 and 25.3, respectively), compared with high RVFAC+high O2 pulse (p=0.0001).

Conclusions  Echocardiography combined with CPET provides relevant clinical and prognostic information. A combination of low RVFAC and low O2 pulse identifies patients at a particularly high risk of clinical deterioration.


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