Background: Pulmonary arterial hypertension (PAH) is a rare disease characterised by a
severe impairment of functional status and quality of life (QoL). Use of rehabilitative programmes
may help to improve outcomes. The aim of this pre/post test case series was to evaluate the impact
of a training program, including sessions of aerobic and resistance exercise, inspiratory muscle
reinforcement, slow breathing, relaxation, and psychological support, on functional outcomes.
Methods: Fifteen patients affected by PAH, in World Health Organization (WHO) Functional Class
(FC) II or III and in stable clinical condition, were included in a 4-week cardiorespiratory training
programme conducted in outpatient service. Patients were tested during a routine control visit (T0),
one month later at the beginning of the training programme (T1), and at study end (T2). Between T0
and T1, patients continued their normal activities and therapies. At each step, patients underwent
respiratory and functional evaluation by spirometry, 6-minute walk test (6-MWT), maximal
cardiopulmonary exercise testing (CPET), echocardiography, and levels of brain natriuretic peptide
(BNP). QoL was also assessed at T1 and T2 using the Hospital Anxiety and Depression Scale and
the EuroQoL-5D questionnaire. The primary endpoint was the effect of training on peak oxygen
consumption (peak V̇O2).
Results: There were no significant differences in BNP levels, or in any of the respiratory or
echocardiographic parameters measured, between T0 and T1. Between T1 and T2, significant
improvements were recorded in QoL (HADS-Anxiety mean change 3.5 ± 3.3 and HADS-Depression
mean change 1.6 ± 2.0, all p < 0.01). Significant improvements were also observed in functional
capacity with distance walked at 6-MWT increasing from 455 ± 115 to 487 ± 120 (+8%, p < 0.01),
workload (WR) of CPET increased of 22% (from 73 ± 22 to 87 ± 21 watt, p < 0.001), peak V̇O2
increasing from 17.3 ± 4.2 to 19.9 ± 4.5 mL/kg/min (p < 0.001) and pulse O2 increasing from 7.8 ±
1.8 to 8.8 ± 2.4 mL/beat (p < 0.01). No adverse events or deterioration in clinical status were
observed during the training sessions.
Conclusion: Cardiorespiratory training in a outpatient service is a suitable option for patients with
PAH in WHO FC II/III thanks to improved exercise capacity and QoL, which may allow them to
achieve better outcomes.