Source: International Congress 2016 – Pulmonary hypertension and pulmonary embolism: from the bench to the bedside
Disease area: Pulmonary vascular diseases

Abstract

EiPAH (mPAP>30mmHg at peak exercise, PVRmax>80d·s·cm-5, and PCWPmax<20mmHg in the absence of resting PAH) may represent an early form of PAH. Identifying disease and initiating treatment at an early stage may prevent progression to PAH.Methods: Single-center, open-label 6-month study to evaluate the effect of ambrisentan on exercise capacity utilizing invasive CPET (iCPET) in EiPAH. Patients were recruited after clinically indicated iCPET. After 6-months treatment patients repeated iCPET; exercise capacity, symptoms, and hemodynamics were assessed. We compared change from baseline in peak exercise values, and WHO FC.Results: 18 patients (age 60.0±2.2) completed the treatment and repeat iCPET. Baseline peak hemodynamics: mPAP=38.1±1.5mmHg, PCWP=13.6±1.0mmHg, TPG=24.1±2.1mmHg, CO=10.5±1.2L/min, PVR=181.9±20.0d·s·cm-5, and VO2max=1530±152.1mL/min, After 6 months there was significant increase in peak CO (+3.2±1.0L/min, p=0.004), and significant decline in peak mPAP (-4.5±1.3mmHg, p=0.009), TPG (-6.2±1.4mmHg, p=0.019), and PVR (-72.8±12.8d·s·cm-5, p=0.0006); no significant change in PCWP (+2.2±1.0mmHg, p=0.19) and a trend toward increase in VO2max (+93.4±170.6mL/min, p=0.078). There was improvement in WHO FC (1.9±0.1 at baseline, to 1.4±0.2) after 24 weeks (p=0.019).Conclusions: EiPAH may represent an early phase of PAH and an abnormal vascular response to exercise. Treatment of EiPAH with ambrisentan results in improved hemodynamics and WHO FC. Early intervention may prevent progression of remodeling and development of established PAH. Further study of EiPAH treatment is warranted, especially with regards to functional capacity, disease progression and QOL.


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Aaron Waxman (Boston, United States of America), Sergio Segrera, Alexander Opotowsky, Laurie Lawler, David Systrom, Aaron Waxman. Open label study of ambrisentan in patients with exercise induced pulmonary arterial hypertension (EiPAH). Eur Respir J 2016; 48: Suppl. 60, 2476

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