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. 2017 Apr-Jun;7(2):531-538.
doi: 10.1177/2045893217709024. Epub 2017 May 12.

Open label study of ambrisentan in patients with exercise pulmonary hypertension

Sergio A Segrera  1 , Laurie Lawler  1 , Alexander R Opotowsky  1 , David Systrom  1 , Aaron B Waxman  1
Affiliations

Open label study of ambrisentan in patients with exercise pulmonary hypertension

Sergio A Segrera et al. Pulm Circ. 2017 Apr-Jun.

Abstract

A growing body of evidence suggests that exercise pulmonary hypertension (ePH) is an early form of pulmonary arterial hypertension (PAH). Identifying the disease at an early, potentially more responsive phase, and initiating treatment may improve functional status and prevent progression to severe forms of PAH. This was a single-center, open-label six-month treatment trial to evaluate the effect of ambrisentan on pulmonary hemodynamics and exercise capacity in ePH utilizing invasive cardiopulmonary exercise testing (iCPET). After six months of treatment with ambrisentan, patients repeated iCPET; exercise capacity, symptoms, and pulmonary hemodynamics were reassessed. Twenty-two of 30 patients completed the treatment phase and repeat iCPET. After six months of treatment there was a significant decline in peak exercise mPAP (-5.2 ± 5.6 mmHg, P = 0.001), TPG (-7.1 ± 8.0 mmHg, P = 0.001), PVR (-0.9 ± 0.7 Woods units, P = 0.0002), and Ca-vO2 (-1.8 ± 2.3 mL/dL, P = 0.0002), with significant increases in peak PCWP (+2.9 ± 5.6 mmHg, P = 0.02), PVC (+0.8 ± 1.4 mL/mmHg, P = 0.03), and CO (+2.3 ± 1.4 L/min, P = 0.0001). A trend toward increased VO2max (+4.4 ± 2.6% predicted, P = 0.07) was observed. In addition, there were improvements in 6MWD and WHO FC after 24 weeks. Our findings suggest that treatment of ePH with ambrisentan results in improved pulmonary hemodynamics and functional status over a six-month period. Treatment of ePH may prevent the progression of vascular remodeling and development of established PAH.

Keywords: cardiopulmonary exercise testing (CPET); dyspnea; exercise; pulmonary hypertension; treatment.

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Figures

Fig. 1.
Fig. 1.
Peak exercise hemodynamics (n = 22). (a) Mean pulmonary artery pressure; (b) pulmonary capillary wedge pressure; (c) transpulmonary pressure gradient; (d) cardiac output. Box plots show median, IQR, minimum, and maximum values (± 1.5·IQR).
Fig. 2.
Fig. 2.
Peak exercise hemodynamics (cont.) (n = 22). (a) PVR = Pulmonary vascular resistance; (b) Pulmonary vascular compliance; (c) Maximum oxygen uptake. Box plots show median, IQR, minimum, and maximum values (±1.5·IQR).
See this image and copyright information in PMC

References

    1. Badesch DB, Champion HC, Sanchez MA, et al. Diagnosis and assessment of pulmonary arterial hypertension. J Am Coll Cardiol 2009; 54(Suppl. 1): S55–66. - PubMed
    1. Hoeper MM, Bogaard HJ, Condliffe R, et al. Definitions and diagnosis of pulmonary hypertension. J Am Coll Cardiol 2013; 62(Suppl. 25): D42–50. - PubMed
    1. Tolle JJ, Waxman AB, Van Horn TL, et al. Exercise-induced pulmonary arterial hypertension. Circulation 2008; 118(21): 2183–2189. - PMC - PubMed
    1. Fowler RM, Maiorana AJ, Jenkins SC, et al. Implications of exercise-induced pulmonary arterial hypertension. Med Sci Sports Exerc 2011; 43(6): 983–989. - PubMed
    1. Waxman AB, Bennett C, Janocha AJ, et al. Abnormal transpulmonary metabolite flux in exercise induced pulmonary arterial vasculopathy. Am J Respir Crit Care Med 2016; 193: A2909.