ABSTRACT
Care of patients with pulmonary arterial hypertension (PAH) needs a multi-facetet concept and measures, including management of adverse reactions, right heart insufficiency as well as information on pregnancy, travels by air, psychosocial support, physical exercise training and prophylaxis by vaccination.Positive study results led to an higher recommendation of specialized exercise training in pulmonary hypertension. Also, the recommendation on iron substitution was amended according to the current evidence.In the current guidelines, special focus was given to the elaboration of recommendations regarding pregnancy, including patient information, contraception and patient management in case of pregnancy.This article aims to provide an overview on the recommendations of general measuremes, special circumstances and patient management according to the ESC/ERS guidelines. Amendments to the guideline recommendations are given as comments from the authors of this article.
Subject(s)
Heart Failure , Hypertension, Pulmonary , Pulmonary Arterial Hypertension , Pregnancy , Female , Humans , Hypertension, Pulmonary/diagnosis , Hypertension, Pulmonary/therapy , ExerciseABSTRACT
AIMS: The impact of exercise training on the right heart and pulmonary circulation has not yet been invasively assessed in patients with pulmonary hypertension (PH) and right heart failure. This prospective randomized controlled study investigates the effects of exercise training on peak VO2/kg, haemodynamics, and further clinically relevant parameters in PH patients. METHODS AND RESULTS: Eighty-seven patients with pulmonary arterial hypertension and inoperable chronic thrombo-embolic PH (54% female, 56 ± 15 years, 84% World Health Organization functional class III/IV, 53% combination therapy) on stable disease-targeted medication were randomly assigned to a control and training group. Medication remained unchanged during the study period. Non-invasive assessments and right heart catheterization at rest and during exercise were performed at baseline and after 15 weeks. Primary endpoint was the change in peak VO2/kg. Secondary endpoints included changes in haemodynamics. For missing data, multiple imputation and responder analyses were performed. The study results showed a significant improvement of peak VO2/kg in the training group (difference from baseline to 15 weeks: training +3.1 ± 2.7 mL/min/kg equals +24.3% vs. control -0.2 ± 2.3 mL/min/kg equals +0.9%, P < 0.001). Cardiac index (CI) at rest and during exercise, mean pulmonary arterial pressure, pulmonary vascular resistance, 6 min walking distance, quality of life, and exercise capacity significantly improved by exercise training. CONCLUSION: Low-dose exercise training at 4-7 days/week significantly improved peak VO2/kg, haemodynamics, and further clinically relevant parameters. The improvements of CI at rest and during exercise indicate that exercise training may improve the right ventricular function. Further, large multicentre trials are necessary to confirm these results.
Subject(s)
Exercise Therapy/methods , Hypertension, Pulmonary/rehabilitation , Thromboembolism/rehabilitation , Analysis of Variance , Biomarkers/metabolism , Cardiac Output/physiology , Chronic Disease , Exercise Test , Exercise Tolerance/physiology , Female , Heart Rate/physiology , Humans , Hypertension, Pulmonary/physiopathology , Male , Middle Aged , Natriuretic Peptide, Brain/metabolism , Oxygen Consumption/physiology , Peptide Fragments/metabolism , Prospective Studies , Pulmonary Wedge Pressure/physiology , Thromboembolism/physiopathology , Treatment Outcome , Vascular Resistance/physiology , Ventricular Dysfunction, Right/physiopathology , Ventricular Dysfunction, Right/rehabilitationABSTRACT
BACKGROUND: The objective of this prospective study was to assess the efficacy of exercise training as add-on to medical therapy in patients with congenital heart disease associated pulmonary arterial hypertension (CHD-APAH). METHODS: Patients with invasively confirmed CHD-APAH received in-hospital exercise training for 3 weeks and continued at home. Efficacy parameters were evaluated at baseline, after 3 and 15 weeks. Medical treatment remained unchanged. Worsening events and survival rate were assessed in a follow-up period of 21 ± 14 months. RESULTS: Twenty consecutive CHD-APAH patients (16 female, 4 male, mean pulmonary arterial pressure 60 ± 23 mm Hg) were included. Patients significantly improved the mean distance walked in 6 min compared to baseline by 63 ± 47 m after 3 weeks (p<0.001) and by 67 ± 59 m after 15 weeks (p=0.001). Quality of life-score (p=0.05), peak oxygen consumption (p=0.002) and maximal workload (p=0.003) improved significantly by exercise training after 15 weeks. The 1- and 2-year survival rates were 100%, the transplantation-free survival rate was 100% after 1 year and 93% after 2 years. CONCLUSION: Exercise training as add-on to medical therapy may be effective in patients with CHD-APAH and improved work capacity, quality of life and further prognostic relevant parameters. It was associated with an excellent long-term survival. Further randomized controlled studies are needed to confirm these results.
