Exercise training in pulmonary arterial hypertension associated with connective tissue diseases

Ekkehard Grünig,Frank Reichenberger,Felicitas Maier,Hans-Jürgen Seyfarth,Norbert Blank,Felix Prange,Henning Tiede,Nicola Ehlken,Christian Nagel,Christoph Fiehn,Christine Fischer,Michael Halank,Simone Wagner,Frank Stöckl,Mona Lichtblau,Gerd Staehler

doi:10.1186/ar3883

Abstract

IntroductionThe objective of this prospective study was to assess short- and long-term efficacy of exercise training (ET) as add-on to medical therapy in patients with connective tissue disease-associated pulmonary arterial hypertension (CTD-APAH).MethodsPatients with invasively confirmed CTD-APAH received ET in-hospital for 3 weeks and continued at home for 12 weeks. Efficacy parameters have been evaluated at baseline and after 15 weeks by blinded-observers. Survival rate has been evaluated in a follow-up period of 2.9 ± 1.9 years.ResultsTwenty-one consecutive patients were included and assessed at baseline, and after 3 weeks, 14 after 15 weeks. Patients significantly improved the mean distance walked in 6 minutes compared to baseline by 67 ± 52 meters after 3 weeks (p < 0.001) and by 71 ± 35 meters after 15 weeks (p = 0.003), scores of quality of life (p < 0.05), heart rate at rest, peak oxygen consumption, oxygen saturation and maximal workload. Systolic pulmonary artery pressure and diastolic systemic blood pressure improved significantly after 3 weeks of ET. The 1- and 2-year overall-survival rates were 100%, the 3-year survival 73%. In one patient lung transplantation was performed 6 months after ET.ConclusionET as add-on to medical therapy is highly effective in patients with CTD-APAH to improve work capacity, quality of life and further prognostic relevant parameters and possibly improves the 1-, 2- and 3-year survival rate. Further randomized controlled studies are needed to confirm these results.Trial registrationClinicalTrials.gov: NCT00491309.

Highlights

The objective of this prospective study was to assess short- and long-term efficacy of exercise training (ET) as add-on to medical therapy in patients with connective tissue disease-associated pulmonary arterial hypertension (CTD-Associated Pulmonary arterial hypertension (PAH) (APAH))
Therapy with ambrisentan resulted in significant improvement in the 6-minute walking distance (6MWD) among patients with idiopathic PAH (IPAH) but not with CTD-Associated PAH (APAH) [12]
The diagnosis of CTD-APAH was established at the participating pulmonary hypertension (PH) centers according to current guidelines [1,15]

Summary

Introduction

The objective of this prospective study was to assess short- and long-term efficacy of exercise training (ET) as add-on to medical therapy in patients with connective tissue disease-associated pulmonary arterial hypertension (CTD-APAH). Patients with CTD-APAH showed a significantly reduced time to hospitalization, had a higher mean age at diagnosis and higher incidence of comorbidities such as renal insufficiency and Raynaud’s phenomenon [7,10]. Therapy with ambrisentan resulted in significant improvement in the 6MWD among patients with IPAH but not with CTD-APAH [12]. Patients with CTD-APAH in particular, may have a high need for additional therapeutic tools to address their exercise capacity, quality of life (QoL) and survival

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