Exercise training in pulmonary arterial hypertension associated with connective tissue diseases.
BACKGROUND
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).


METHODS
Patients 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.


RESULTS
Twenty-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.


CONCLUSIONS
ET 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.


BACKGROUND
ClinicalTrials.gov: NCT00491309.
Centre for Pulmonary Hypertension at Thoraxclinic Heidelberg, Thoraxclinic at the University Hospital Heidelberg, Amalienstrasse 5, Heidelberg, 69126, Germany. ekkehard.gruenig@thoraxklinik-heidelberg.de
Ekkehard
E
Grünig
Felicitas
F
Maier
Nicola
N
Ehlken
Christine
C
Fischer
Mona
M
Lichtblau
Norbert
N
Blank
Christoph
C
Fiehn
Frank
F
Stöckl
Felix
F
Prange
Gerd
G
Staehler
Frank
F
Reichenberger
Henning
H
Tiede
Michael
M
Halank
Hans-Jürgen
HJ
Seyfarth
Simone
S
Wagner
Christian
C
Nagel
D003240:Connective Tissue Diseases / Q000150:complications / Q000401:mortality / Q000534:rehabilitation*; D005081:Exercise Therapy / Q000379:methods*; D065627:Familial Primary Pulmonary Hypertension; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000209:etiology / Q000401:mortality / Q000534:rehabilitation*; D053208:Kaplan-Meier Estimate; D008297:Male; D008875:Middle Aged; D012129:Respiratory Function Tests; D016037:Single-Blind Method