ly activity, reducing dyspnoea and fatigue, and enhancing the quality of life and muscle strength. So, the final message from the available literature reports is a strong recommendation to adopt exercise training and cardiopulmonary rehabilitation programs for any patient suffering from exertional dyspnoea and fatigue. Many questions remain unanswered regarding the optimization of the exercise training and rehabilitation programs. Ongoing rehabilitative trials in patients with PAH will help answer some of the remaining questions.KEY WORDS: pulmonary arterial hypertension; dyspnoea; exercise limitation; exercise training; pulmonary rehabilitation.
IntroductionPulmonary arterial hypertension (PAH) is a hemodynamic condition defined by a pre-capillary resting pulmonary artery pressure at or above 25 mm Hg mean value that can be the result of a variety of diseases of different causes, but similar clinical picture and virtually identical pathological changes of the distal pulmonary arteries (1, 2). During the Fifth World Symposium on pulmonary hypertension (PH) held in 2013 in Nice, the clinical classification of pulmonary hypertension (2) identified five groups of disorders according to pathological, pathophysiological and therapeutic characteristics, but the term PAH may only be applied to a subgroup of the group 1 including eight rare clinical conditions characterized by pre-capillary PH in absence of other causes of pre-capillary PH such as lung diseases, chronic pulmonary thromboembolism (CTEPH), or rare diseases related-PH with unclear multi-factorial mechanisms. Despite a number of specific drugs are available by now for PAH treatment, exertional dyspnoea is a common progressively disabling symptom, shared either by idiopathic and secondary PAH, still affecting well treated patients. Exercise training (ET), a major component of pulmonary rehabilitation programs, is an established treatment to enhance exercise limitation for patients with chronic obstructive pulmonary disease (COPD), systolic left heart failure, and a variety of chronic respiratory diseases (3
SummaryIn spite of evidence-based consensus recommendations on exercise training for patients with pulmonary arterial hypertension (PAH) its use outside specialized rehabilitation Centers has been accounted a handful of reports. Dyspnoea and fatigue are common symptoms reducing the exercise tolerance of subjects with PAH even when specific drug are correctly administered. Moreover, the mechanisms of exercise limitation in subjects with PAH are known and thandful of reports. There is evidence of impaired signaling between the periphery and central hemodynamic performance. Exercise training has recently been proven to be effective and safe to enhance physical performance in patients with PAH already treated with specific drug therapy. The rationale of training in PAH has been specifically proven also in experimental animal models of PAH. Moreover, repeated positive results in randomized controlled trials and observational studies in humans gave convincing ...