Abstract
BACKGROUND: Pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension are chronic diseases with a severe symptom burden. Common symptoms are dyspnoea at light activity and general fatigue that limits daily activities. Respiratory modulation by device-guided breathing decreased symptoms in patients with heart failure. The aim of this pilot study was to investigate if respiratory modulation could improve symptoms of dyspnoea in patients with pulmonary arterial hypertension or chronic thromboembolic pulmonary hypertension. METHOD: Adult patients with pulmonary arterial hypertension or chronic thromboembolic pulmonary hypertension with symptoms of dyspnoea at rest or light activity performed home-based respiratory modulation by device-guided breathing 20 min a day for 3 months. Patients were on stable disease-specific treatment ⩾3 months and willing to undergo all study procedures. Dyspnoea score, World Health Organization class, physical status, N-terminal pro b-type natriuretic peptide, quality of life, respiratory rate and 6-min walk distance were assessed before and after 3 months with respiratory modulation. RESULTS: Nine patients with pulmonary arterial hypertension and five with chronic thromboembolic pulmonary hypertension completed the study protocol. Mean age was 71 ± 14 years, and 11 were women. After 3 months of respiratory modulation, dyspnoea score (-0.6, p = 0.014), respiratory rate at rest (-3 breaths/min, p = 0.013), World Health Organization class (-0.3, p = 0.040), quality of life (EuroQol Visual Analogue Scale +5 points, not significant) and decreased N-terminal pro b-type natriuretic peptide (-163 ng/L, p = 0.043) had improved. The fatigue and respiratory rate after the 6-min walk decreased while the 6-min walk distance remained unchanged. CONCLUSION: Patients with pulmonary arterial hypertension or chronic thromboembolic pulmonary hypertension that used device-guided breathing for 3 months improved symptoms of dyspnoea and lowered the respiratory rate at rest and after exercise.