Skeletal muscle dysfunction in patients with idiopathic pulmonary arterial hypertension

Bauer, Ralf; Dehnert, Christoph; Schoene, P.; Filusch, Arthur; Bärtsch, Peter; Borst, Mathias M.; Katus, Hugo A.; Meyer, F. J.

doi:10.1016/j.rmed.2007.06.014

Cited by 104 publications

(94 citation statements)

References 24 publications

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“…65 More recently, Bauer et al studied forearm muscle function in 24 patients with IPAH and found abnormalities that were qualitatively similar to those in patients with LV failure. 75 In addition, the reduction in skeletal muscle strength was paralleled by respiratory muscle dysfunction in these patients. 76 And further support of this concept comes from a study that has demonstrated a marked improvement in exercise capacity in patients with severe PAH following an exercise rehabilitation program, 27 far greater than the effects from vasodilator therapy, but analogous to the observations that have been made in LV failure.…”

Section: Skeletal Muscle In Patients With Phmentioning

confidence: 87%

The Effects of Vasodilators in Pulmonary Hypertension

Rich

2009

Circ: Heart Failure

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W hen primary pulmonary hypertension (PPH) was first described in the medical literature, it was characterized from a cardiac catheterization on a young woman who had an elevated pulmonary arterial pressure of unknown origin which, after the intravenous administration of acetylcholine, promptly fell. 1 The sentinel description of this phenomenon led to the acceptance of PPH as a medical entity in which inappropriate pulmonary vasoconstriction was a central feature. 2 Since then many series of patients with PPH (now referred to as idiopathic pulmonary arterial hypertension [IPAH]) have been published which document a variable ability to respond to acute vasodilator challenge. Although, the approach to therapy has emphasized the initial classification of responder or nonresponder, most patients demonstrate only a small decrease in pulmonary pressure (PAP) in response to vasodilators. 3 However, vasodilators are widely prescribed in patients who are nonresponders, based on clinical trials that established the response to a 6-minute walk (6 MW) test as the primary end point. 4 The consistent improvement in 6 MW reflects the complexity of exercise intolerance, which may be strongly influenced not only by cardiopulmonary factors, but by peripheral factors such as the muscle ergoreflex. 5 Who is a Responder?In the current era, patients with PAH 6 who undergo acute vasoreactivity testing at the time of diagnosis are classified as responders or nonresponders based on the change in PAP from select agents. 7 The definition of a responder has changed many times, the most recent being a fall in the mean PAP of at least 10 mm Hg to a level below 40 mm Hg without any fall in cardiac output. 4 However, the response is in reality a continuum, and not an all or none phenomenon. It is notable that in 2 large series of patients who were deemed acute responders, the characteristic of those who had near perfect survival on calcium blockers (CCB) for up to 18 years had an acute fall in mean PAP of 39% and an acute fall in pulmonary vascular resistance (PVR) of 50%. 3,8 It is remarkable how similar the experiences from the 2 series are, supporting the validity of the observations. Who is a Nonresponder?The majority of patients with IPAH are nonresponders. Although this implies lack of vasoreactivity, this is clearly not the case. In an observational study of patients with IPAH who were monitored with a pulmonary artery catheter over 6 hours without an intervention, considerable variability was noted in the PAP (Ϯ8%) and PVR (Ϯ13%), suggesting that changing vasomotor tone is common in these patients. 9 In the 2 series on long-term calcium blocker therapy, the nonresponders also had an acute fall in mean PAP and PVR, but of a lesser magnitude than the responder group. In one series, the fall in PAP was 8%, and fall in PVR was 17%. 8 In the other, nonresponders were noted to have a fall in PVR of less than 20%. 3 Thus, the characterization of a patient as a nonresponder actually means a minimal response, not lack of response. Although the...

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Section: Skeletal Muscle In Patients With Phmentioning

confidence: 87%

The Effects of Vasodilators in Pulmonary Hypertension

Rich

2009

Circ: Heart Failure

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“…More recently, several reports suggested muscle dysfunction in patients with pulmonary arterial hypertension [14][15][16]. Mechanisms are still unclear, but it can be speculated that inactivity of the skeletal muscles, together with a decreased cardiac output leading to a reduced oxygen transport to the skeletal muscles, trigger morphological changes, such as muscle atrophy, fibre type switching and reduced aerobic capacity [18].…”

Section: Training Improved Endurance Capacitymentioning

confidence: 99%

“…Respiratory muscle dysfunction was found in two studies, both by voluntary and nonvoluntary techniques [14,15]. More recently, forearm muscle dysfunction has been reported in these patients [16]. In a pilot study, examining voluntary strength of the respiratory, forearm and quadriceps muscles in iPAH patients, specific quadriceps muscle dysfunction has been found [17].…”

mentioning

confidence: 95%

Effects of exercise training in patients with idiopathic pulmonary arterial hypertension

Man¹,

Handoko²,

Groepenhoff³

et al. 2009

Eur Respir J

205

202

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We determined the physiological effects of exercise training on exercise capacity and quadriceps muscle function in patients with idiopathic pulmonary arterial hypertension (iPAH).In total, 19 clinically stable iPAH patients (New York Heart Association II-III) underwent a supervised exercise training programme for the duration of 12 weeks. Maximal capacity, endurance capacity and quadriceps function were assessed at baseline and after 12 weeks. In 12 patients, serial quadriceps muscle biopsies were obtained.6-min walk distance and peak exercise capacity did not change after training. However, endurance capacity improved significantly after training, demonstrated by a shift of the anaerobic threshold to a higher workload (from 32¡5 to 46¡6 W; p50.003) together with an increase in exercise endurance time (p,0.001). Moreover, exercise training increased quadriceps strength by 13% (p50.005) and quadriceps endurance by 34% (p50.001).Training enhanced aerobic capacity of the quadriceps, by increasing capillarisation (1.36¡0.10 to 1.78¡0.13 capillaries per muscle fibre; p,0.001) and oxidative enzyme activity, especially of the type-I (slow) muscle fibres. No changes were found in cross-sectional area and fibre type distribution.Exercise training in iPAH improves exercise endurance and quadriceps muscle function, which is also reflected by structural changes of the quadriceps.

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“…In addition, muscle strength may be reduced in patients with pulmonary hypertension compared with control subjects [17]. A delayed recovery in TLCO could be considered the consequence of major surgery in the early period after PEA and of the vessels remodelling before surgery in the nonobstructed segments of the lungs in the later follow-up.…”

Section: Determinants Of Exercise Capacity After Peamentioning

confidence: 99%

Pulmonary arterial compliance and exercise capacity after pulmonary endarterectomy

Ghio

Morsolini²,

Corsico

et al. 2014

Eur Respir J

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Patients with chronic thromboembolic pulmonary hypertension (CTEPH), despite successful pulmonary endarterectomy (PEA), can continue to suffer from a limitation in exercise capacity. The objective of this study was to assess whether pulmonary arterial compliance is a predictor of exercise capacity after PEA.Right heart haemodynamics, treadmill incremental exercise test, spirometry, carbon monoxide transfer factor, arterial blood gas and echocardiographic examinations were retrospectively analysed in a population of CTEPH patients who underwent PEA at a single centre. Baseline and 3-month haemodynamic data were available in 296 patients; 5-year follow-up data were available in 68 patients.In a multivariable model the following parameters were found to be independent predictors of exercise capacity after surgery: age, sex, pulmonary arterial compliance, tricuspid annular plane excursion, arterial oxygen tension and carbon monoxide transfer factor (p,0.0001); the model showed good discrimination (Harrell's c50.84) and calibration (shrinkage coefficient50.91). Poor exercise capacity at 3 months was loosely associated with higher death rate during subsequent survival (Harrell's c50.61).In conclusion, after successful PEA, reduced pulmonary arterial compliance is an important determinant of exercise capacity in association with the age and sex of the patients, and the extent of recovery of both cardiac and respiratory function. However, exercise capacity does not explain a large proportion of the effect of surgery on subsequent survival. @ERSpublications Pulmonary arterial compliance is an important determinant of exercise capacity after pulmonary endarterectomy

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Skeletal muscle dysfunction in patients with idiopathic pulmonary arterial hypertension

Cited by 104 publications

References 24 publications

The Effects of Vasodilators in Pulmonary Hypertension

The Effects of Vasodilators in Pulmonary Hypertension

Effects of exercise training in patients with idiopathic pulmonary arterial hypertension

Pulmonary arterial compliance and exercise capacity after pulmonary endarterectomy

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