Key result
Peripheral muscle weakness in patients with pulmonary arterial hypertension is at least partly caused by sarcomeric dysfunction.
Highlights sarcomeric dysfunction as a potential underlying cause of peripheral muscle weakness and exercise intolerance in patients with pulmonary arterial hypertension.
Sarcomeric dysfunction may contribute to muscle weakness in PAH; hypothesis-generating and should not yet change practice.
Despite improvements in disease targeted therapies, pulmonary arterial hypertension (PAH) is a progressive disease and PAH patients remain symptomatic [1]. Exercise intolerance is one of the main symptoms, which limit PAH patients in their daily life activities. Reduced exercise capacity is generally attributed to right ventricular dysfunction [1]. However, as with other cardiac and pulmonary diseases, PAH patients develop respiratory [2] and peripheral muscle [3, 4] weakness, which might also contribute to exercise intolerance. Indeed, exercise training improves exercise capacity in PAH patients and maximal oxygen consumption of PAH patients correlates with the functional decline of peripheral muscle strength [5, 6]. The underlying cause of the reduction in muscle strength is unclear. Some studies have reported muscle fibre atrophy and a shift towards more fast-twitch fatigable fibres in skeletal muscles of PAH patients [3, 7]; however, these are not consistent findings [4, 8]. Peripheral muscle weakness in PAH patients is at least partly caused by sarcomeric dysfunction
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Manders et al. (2015) studied Pulmonary arterial hypertension. Peripheral muscle weakness in patients with pulmonary arterial hypertension is at least partly caused by sarcomeric dysfunction.
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