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Kovacs, G; Avian, A; Wutte, N; Hafner, F; Moazedi-Fürst, F; Kielhauser, S; Aberer, E; Brodmann, M; Graninger, W; Foris, V; Olschewski, A; Olschewski, H.
Changes in pulmonary exercise haemodynamics in scleroderma: a 4-year prospective study.
Eur Respir J. 2017; 50(1):
Doi: 10.1183/13993003.01708-2016
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- Führende Autor*innen der Med Uni Graz
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Kovacs Gabor
- Co-Autor*innen der Med Uni Graz
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Aberer Elisabeth
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Avian Alexander
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Brodmann Marianne
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Foris Vasile
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Graninger Winfried
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Hafner Franz
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Kielhauser Sonja
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Moazedi-Fürst Florentine
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Olschewski Andrea
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Olschewski Horst
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Palfner-Wutte Nora Johanna
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- Abstract:
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Pulmonary arterial hypertension (PAH) is a feared complication of systemic sclerosis. In this prospective cohort study, we monitored the changes in resting and exercise pulmonary haemodynamics of scleroderma patients without initial PAH over a mean follow-up period of ∼4 years.All patients underwent exercise echocardiography and cardiopulmonary exercise testing at baseline and follow-up. A subgroup underwent exercise right heart catheter (RHC) investigations. The primary end-point was the echocardiographic systolic pulmonary arterial pressure at 50 W exercise (sPAP50).We included 99 patients, of whom 58 had a complete dataset. Three out of 99 patients developed RHC-confirmed PAH (0.75 cases per 100 patient-years). sPAP50 increased (p<0.001) and peak oxygen uptake (secondary end-point) decreased significantly (p=0.001) during follow-up, but there was no significant change in resting sPAP (p=0.38). In the RHC subgroup (n=28), mean (m)PAP and pulmonary vascular resistance at 50 W increased significantly (p=0.02 and p=0.002, respectively), but resting mPAP was unchanged.Scleroderma patients without PAH develop a mild but significant deterioration of pulmonary exercise haemodynamics and exercise capacity over a 4-year follow-up period, indicating a progression of pulmonary vascular disease. The manifestation rate of RHC-confirmed PAH was 0.75 cases per 100 patient-years.
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Adult -
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Austria -
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Blood Pressure -
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Cardiac Output -
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Echocardiography -
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Exercise -
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Exercise Test -
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Female -
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Humans -
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Hypertension, Pulmonary - physiopathology
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Male -
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Middle Aged -
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Pulmonary Artery - physiopathology
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Scleroderma, Systemic - complications
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Vascular Resistance -