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Skeletal muscle dysfunction in patients with idiopathic pulmonary arterial hypertension

Lookup NU author(s): Dr Ralf Bauer


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Dyspnea and exercise limitation are common in patients with idiopathic pulmonary arterial hypertension (IPAH). Recently, a reduction in inspiratory and expiratory muscle strength has been observed in IPAH. However, it has not been investigated whether this respiratory muscle weakness might be part of a general muscle dysfunction as observed in congestive left heart failure. Therefore, in 24 consecutive IPAH patients (16 female; age 58.7 +/- 16.2; WHO class II-III; systolic pulmonary artery pressure during echocardiography at rest (sPAP) 65.0 +/- 20.6 mmHg, and 6-min-walk test (6-MWT) 473.6 +/- 127.7m), the maximal isometric forearm muscle strength (best of three hand grip manoeuvres), maximal. inspiratory and expiratory mouth occlusion pressures (Pimax, Pemax) were prospectively evaluated. The isometric forearm muscle strength was significantly tower in IPAH patients (281.7 +/- 102.6 N) than in matched 24 healthy controls (397.1 +/- 116.8 N; p = 0.03). In IPAH patients, there was a correlation between maximal isometric forearm muscle strength and 6-MV,1T (r = 0.67; p = 0.0007) and both, Pimax (r = 0.69; p = 0.0003) and Pemax (r = 0.63; p = 0.01), respectively. There was no correlation between forearm muscle strength and sPAP (r = 0.30; p = 0.16). The present skeletal muscle dysfunction is a novel finding in patients with IPAH. The correlation with respiratory muscle dysfunction and severity of disease might indicate a generalised "myopathy" in IPAH. (C) 2007 Elsevier Ltd. All rights reserved.

Publication metadata

Author(s): Bauer R, Dehnert C, Schoene P, Filusch A, Bartsch P, Borst MM, Katus HA, Meyer FJ

Publication type: Article

Publication status: Published

Journal: Respiratory Medicine

Year: 2007

Volume: 101

Issue: 11

Pages: 2366-2369

ISSN (print): 0954-6111

ISSN (electronic): 1532-3064

Publisher: Elsevier


DOI: 10.1016/j.rmed.2007.06.014


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