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A Randomized Controlled Multicenter Trial of Exercise Training in Pulmonary Hypertension in European Countries

Not Applicable
Completed
Conditions
Pulmonary Hypertension
Interventions
Other: Exercise training
Registration Number
NCT03345212
Lead Sponsor
Heidelberg University
Brief Summary

Chronic pulmonary hypertension (PH) is associated with impaired exercise capacity, quality of life and right ventricular function. The disease is characterized by an increase of pulmonary vascular resistance and pulmonary arterial pressure, leading to right heart insufficiency.

Despite optimized combination-medical therapy most patients remain symptomatic, have reduced exercise capacity, quality of life and reduced survival rates, with an annual mortality rate of approximately 5 -15 % or even higher.

Previous training studies have suggested that exercise training as add-on to medical treatment is highly effective improving exercise capacity, quality of life and symptoms.

The current guidelines recommend exercise training only in specialized centres including both PH and rehabilitation specialists who are experienced in exercise training of severely compromised patients.

A specialized PH-training program has been performed in Heidelberg since 2003 including \>1200 patients with various forms of chronic PH. The exercise training program is performed in a special setting with an in-hospital start of the rehabilitation program. It is characterized by a low-dose closely supervised exercise training in small groups with additional psychological support and mental training.

This training program for patients with PH will be implemented in European centers to add exercise training to the existing PH therapies. The effect of the training on physical exercise capacity will be assessed by 6-minute walking distance (6-MWD). Further clinical parameters will be assessed to evaluate the effect on exercise capacity, quality of life and symptoms.

The aim of this study is to guide European PH-centers to become specialized centers for training in PH.

126 patients will be included, who either receive exercise training or continue their daily sedentary life style (1:1 randomization) for 15 weeks.

As inpatient settings are not available in all healthcare systems the training program will be adapted from the specific training program for PH patients developed in Heidelberg to a procedure, which is feasible in the local participating centres. Another objective of this study is to assess if the particular adopted training program specified for each participating centre and country is still safe and effective.

Detailed Description

Pulmonary hypertension (PH) is defined as a mean pulmonary arterial pressure ≥25 mmHg. PH is often diagnosed at an advanced stage (WHO functional class III-IV) with a massive increase of the mean pulmonary arterial pressure. A crucial parameter determining the symptoms and prognosis of the patients is the cardiac reserve. This parameter is defined by the pulmonary vascular resistance and the right ventricular adaptation. Severe PH is characterized by a decreased cardiac output at rest, an increased afterload and consecutive cor pulmonale.

Within the last years there has been a huge progress in the scientific fields of genetics, pathogenesis, pathophysiology and therapy of PH. This has also been documented in the PH world conferences. New disease-targeted medication has been developed such as endothelin receptor antagonists (bosentan, ambrisentan, sitaxentan, macitentan), prostacyclin derivates (inhaled and intravenous iloprost, epoprostenol, treprostinil), phosphodiesterase-5-inhibitors (sildenafil, tadalafil) and the soluble guanylate cyclase inhibitor riociguat. Despite these advances in treatment, the disease may not be treated causally or even be cured. In most cases however, disease progression may be slowed down. The use of PH-targeted treatment and supporting therapies such as anticoagulation and diuretics improve the symptoms and impede the progression of the disease. Nevertheless, the prognosis of the patients remains impaired. The first randomized controlled study investigating the effect of exercise training in PH showed a significant improvement of exercise capacity and quality of life. Further uncontrolled trials using a low-dose exercise and respiratory therapy in different etiologies of PH showed an improvement in exercise capacity, quality of life, muscle function and further prognostic parameters. A recent randomized controlled study could support these findings. Studies also showed an improvement in muscle capillarization of the quadriceps muscle.

The training program consists of interval ergometer training, respiratory therapy, muscle training and mental gait training. The interval ergometer training allows performing aerobic exercise training with a low cardio-circulatory stress. In patients with left heart insufficiency, this training has been successfully implemented. Respiratory therapy has been established in the rehabilitation of patients with lung disease within the last years. The different techniques aim to improve ventilation, strengthen the respiratory muscles, mobilize the thorax and enhance secretolysis. The training program also contains mental (gait) training. This training was adapted from mental imagery techniques used by sport psychologists in professional athletes. Mental imagery techniques have shown to improve physical and cognitive functions.

Due to the beneficial results, exercise training and rehabilitation has received a 1A recommendation at the PH world symposium in Nice in 2013. This decision was mainly based on three randomized controlled trials that investigated a limited number of patients. To unequivocally demonstrate safety and positive effects of exercise training in different settings large multicenter RCTs are essential. An exercise program has not yet been implemented in most European countries, partly due to limited access to rehabilitation programs and institutions.

The aim of this large, multicenter, prospective, randomized controlled trial is to investigate the effect of exercise training and rehabilitation on physical exercise capacity across different European countries. Physical exercise capacity will be measured by exercise induced change of 6-minute walking distance (6-MWD) compared to baseline and the control group without training. As inpatient settings are not available in all healthcare systems the training program will be adapted from the specific training program for PH patients developed in Heidelberg in a system, which is feasible for the local participating centres. Another objective of this study is to assess if the adopted training program specified for each participating centre and country is still safe and effective.

Recruitment & Eligibility

Status
COMPLETED
Sex
All
Target Recruitment
129
Inclusion Criteria
  • Female and male patients of any ethnic origin ≥ 18 years

  • WHO functional class II-IV

  • PH diagnosed by right heart catheter showing:

    • Baseline mean pulmonary arterial pressure (mPAP) ≥ 25 mmHg
    • Baseline pulmonary vascular resistance (PVR) ≥ 240 dyn x s x cm-5
    • Baseline pulmonary capillary wedge pressure (PCWP) ≤ 15 mm Hg
  • Patients receiving optimized conventional PH therapy including intensified treatment with diuretics and who have been stable for 2 months before entering the study

  • Except for diuretics, medical treatment should not be expected to change during the entire 15-week study period

  • Negative pregnancy test (β-HCG) at the start of the trial and appropriate contraception throughout the study for women with child-bearing potential

  • Able to understand and willing to sign the Informed Consent Form

Exclusion Criteria
  • PH of any cause other than permitted in the entry criteria, e.g. concomitantly to portal hypertension, complex congenital heart disease, reversed shunt, HIV infection, suspected pulmonary veno-occlusive disease based on pulmonary edema during a previous vasoreactivity test or on abnormal findings compatible with that diagnosis (septal lines or pulmonary edema at high resolution computer tomography), congenital or acquired valvular defects with clinically relevant myocardial function disorders not related to pulmonary hypertension or unclear diagnosis
  • Pregnancy
  • Patients with signs of right heart decompensation
  • Walking disability
  • Acute infection
  • Pyrexia
  • Any change in disease-targeted therapy within the last 2 months
  • Any subject who is scheduled to receive an investigational drug during the course of this study
  • Severe lung disease: FEV1/FVC <0.5 and total lung capacity < 70% of the normal value
  • Active liver disease, porphyria or elevations of serum transaminases >3 x ULN (upper limit of normal) or bilirubin > 1.5 x ULN
  • Hemoglobin concentration of less than 75 % of the lower limit of normal
  • Systolic blood pressure < 85 mmHg
  • Active myocarditis, instable angina pectoris, exercise induced ventricular arrhythmias, decompensated heart failure, hypertrophic obstructive cardiomyopathy, highly impaired left ventricular function
  • History or suspicion of inability to cooperate adequately. will be excluded from the study.

Additional exclusion criteria for MRI (optional)

  • Acute psychosis or other states of mind, which seem to impair patient's ability to comprehend instructions
  • Patients with metal cardiac valves or other metal implants, incorporated ferromagnetic materials or MRI-incompatible active medicinal products
  • Claustrophobia

Study & Design

Study Type
INTERVENTIONAL
Study Design
PARALLEL
Arm && Interventions
GroupInterventionDescription
Training groupExercise trainingStandard rehabilitation therapy includes dietary measures, massages and relaxation techniques. Additionally, patients perform exercise and respiratory therapy and mental gait training. Patients will be informed about group allocation.
Primary Outcome Measures
NameTimeMethod
6 MWD15 weeks

Change in 6-MWD between baseline and 15 weeks in the training vs. the control Group; meters

Secondary Outcome Measures
NameTimeMethod
Change in oxygen saturation of the blood (SaO2)15 weeks

Blood gas analysis

Change in additional oxygen supplementation (yes/no and quantity)15 weeks

Blood gas analysis

Change in oxygen partial pressure15 weeks

Blood gas analysis

Change in oxygen saturation15 weeks

Safety Parameter; L/min

Assessment of clinical laboratory Investigation alerts (values out of range)15 weeks

Safety parameter

Assessment of adverse Events15 weeks

Safety Parameter; unrelated and related to procedure

Assessment of serious adverse events15 weeks

Safety parameter

frequency of hospitalizations15 weeks

Safety parameter

length of hospitalizations15 weeks

Safety parameter

Change in blood pressure15 weeks

Safety parameter

Change in Borg scale 6-MWD training vs. control group15 weeks

Borg scale 6-MWD

Change in Quality of life in training vs. control group15 weeks

Quality of life (SF-36)

Change in WHO functional class in training vs. control group15 weeks

WHO functional class

Change in tricuspid annular plane systolic excursion15 weeks

Echocardiographic parameter training vs. control Group; mm

Change in tissue Doppler imaging15 weeks

Echocardiographic Parameter training vs. control group

Change in forced vital capacity (FVC)15 weeks

Lung function

Change in left ventricular pump function15 weeks

Echocardiographic Parameter training vs. control Group; qualitative

Change in right ventricular pump function15 weeks

Echocardiographic Parameter training vs. control Group; qualitative

Change in thickness of interventricular septum15 weeks

Echocardiographic Parameter training vs. control Group; mm

Change insize of inferior vena cava15 weeks

Echocardiographic Parameter training vs. control Group; mm

Change in systolic pulmonary arterial pressure15 weeks

Echocardiographic Parameter training vs. control Group; mmHg

Change in left ventricular eccentricity index15 weeks

Echocardiographic Parameter training vs. control group

Change in Tei index15 weeks

Echocardiographic Parameter training vs. control group

Change in right ventricular area15 weeks

Echocardiographic Parameter training vs. control group

Change in right atrial area15 weeks

Echocardiographic Parameter training vs. control Group; square cm

Change in workload15 weeks

Cardiopulmonary exercise testing (spiroergometry) training vs. control Group; Watts

Change in heart rate15 weeks

Cardiopulmonary exercise testing (spiroergometry) training vs. control Group; bpm

Change in ventilation15 weeks

Cardiopulmonary exercise testing (spiroergometry) training vs. control Group; L/min

Change in carbon dioxide output15 weeks

Cardiopulmonary exercise testing (spiroergometry) training vs. control Group

Change in spiroergometry parameters in training vs. control group15 weeks

Cardiopulmonary exercise testing (spiroergometry): VO2 at anaerobic threshold determined by V-slope method

Change in VCO2 at anaerobic threshold15 weeks

Cardiopulmonary exercise testing (spiroergometry): determined by V-slope method

Change in oxygen uptake15 weeks

Cardiopulmonary exercise testing (spiroergometry); L/min/kg

Change in diffusion-limited carbon monoxide (DLCO)15 weeks

Lung function; Diffusion capacity

Change in alveolar volume (VA)15 weeks

Lung function

Change in residual volume (RV)15 weeks

Lung function

Change in total lung volume (TLC)15 weeks

Lung function

Change in forced expiratory flow15 weeks

Lung function

Change in peak expiratory flow rate15 weeks

Lung function

Change in forced expiratory volume in one second (FEV1)15 weeks

Lung function; total and in percentage

Change in NTproBNP15 weeks

Laboratory marker for the impairment of the right heart

Change in interleukins15 weeks

Laboratory marker for the impairment of the right heart

Change in inflammatory markers15 weeks

Laboratory marker for the impairment of the right heart

Change in carbon dioxide partial pressure15 weeks

Blood gas Analysis

Change in resting heart rate15 weeks

Safety parameter

frequency of pathological findings in long-term ECG15 weeks

Safety parameter

Qualitative Review of electrocardiogram (ECG)15 weeks

Safety Parameter; pathological findings

Assessment of survival1 year

Training and control Group; transplant-free and Overall survival

Change of the right ventricular size15 weeks

Optional: Changes in MRI parameters

Change of the right ventricular pump function15 weeks

Optional: Changes in MRI parameters

Change of the left ventricular pump function15 weeks

Optional: Changes in MRI parameters

Change in microRNA expression15 weeks

Optional: Epigenetic changes

Change in DNA-methylation15 weeks

Optional: Epigenetic changes

Assessment of relationship of DNA mutations and disease progression15 weeks

Optional: Investigation of DNA mutations relationship to disease progression

Change of the left ventricular size15 weeks

Optional: Changes in MRI parameters

Assessment of relationship of DNA mutations and training effects15 weeks

Optional: Investigation of DNA mutations

Trial Locations

Locations (1)

Centre for pulmonary hypertension of the Thoraxclinic at the University Hospital Heidelberg

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Heidelberg, Germany

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