Respiratory and Physical Therapy in Patients With Associated Pulmonary Arterial Hypertension (APAH) With Congenital Heart Defects

Influence of Respiratory and Exercise Therapy on Oxygen Uptake, Quality of Life in Patients With Severe Associated Pulmonary Arterial Hypertension (APAH) as Part of a Congenital Heart Defect With / Without Eisenmenger's Syndrome

The purpose of this study is to investigate the influence of physical training on exercise capacity, quality of life, functional class, oxygen consumption and right ventricular function in patients with severe associated pulmonary arterial hypertension (APAH) as part of a congenital heart defect with / without Eisenmenger's Syndrome

Study Overview

• Status: Recruiting
• Conditions: Pulmonary Arterial Hypertension; Eisenmenger Syndrome
• Intervention / Treatment: Other: respiratory and exercise therapy

• Study Type: Interventional
• Enrollment (Anticipated): 50
• Phase: Not Applicable

Contacts and Locations

• Study Contact: Name: Ekkehard Gruenig, MD; Phone Number: +49 6221 396 8053; Email: ekkehard.gruenig@med.uni-heidelberg.de

Study Locations

• Germany
  • Heidelberg, Germany, 69126
    • Recruiting
    • : Center for pulmonary Hypertension, Thoraxclinic Heidelberg
    • Principal Investigator: Ekkehard Gruenig, MD

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 18 years to 80 years (Adult, Older Adult)
• Accepts Healthy Volunteers: No
• Genders Eligible for Study: All

Description

Inclusion Criteria:

• signed consent form
• men and women> 18 years <80 years
• APAH with congenital heart defects with / without Eisenmenger syndrome (WHO functional class II-IV), invasively diagnosed by right heart and left heart catheterization: mean pulmonary arterial pressure (mPAP) ≥ 25 mmHg, with targeted PAH medication for at least two months stable before study inclusion (exception: compensated WHO class II without vasodilating drug therapy)

Exclusion Criteria:

• Pregnancy or lactation
• Change in medication during the last 2 months
• severe walking disturbance
• uncertain diagnoses
• No previous invasively confirmation of PH
• acute diseases, infections, fever
• Serious lung disease with FEV1 <50% or TLC <70% of target
• Further exclusion criteria are the following diseases: active myocarditis, unstable angina pectoris, exercise-induced ventricular arrhythmias, recurrent syncope within 4 weeks before study entry

Study Plan

How is the study designed?

Design Details

• Primary Purpose: Supportive Care
• Allocation: Randomized
• Interventional Model: Parallel Assignment
• Masking: None (Open Label)

Number of Arms

2

Arms and Interventions

Participant Group / Arm	Intervention / Treatment
Active Comparator: Respiratory and exercise therapy; Randomized, prospective, controlled, blinded study of three-week inpatient rehabilitation and subsequent continuing of the training at home for 12 weeks. The control group received conventional rehabilitation without a specific training program. After 15 weeks training is also offered to patients in the control group.	Other: respiratory and exercise therapy; Conventional therapy with specific respiratory and physical therapy plus mental walking training
No Intervention: Control group without exercise training; patients of the control group continue their sedentary lifestyle without given advice for exercise training. The time before start of rehabilitation (three months) serves as control group. Afterwards patients take part in the training program as well.

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Time Frame
Changes in the maximum 6-minute walk distance (6MGT)	up to 15 weeks
Changes in quality of life	up to 15 weeks

Secondary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Changes in hemodynamics	Changes in maximum oxygen uptake; Changes in exercise capacity: 6-minute walk distance, Recumbent Bike (Watts), respiratory economy (EQO2, EQCO2); Improved condition(NYHA class, Borg scale); Changes in Magnetic resonance tomography and echocardiographic parameters of right and left ventricle: size and pump function.; Change of laboratory parameters, which are markers of right heart failure as NTproBNP, interleukins	up to 15 weeks

Collaborators and Investigators

• Sponsor: Heidelberg University
• Investigators: Study Chair: Ekkehard Gruenig, MD, Center for pulmonary Hypertension, Thoraxclinic Heidelberg

Study record dates

Study Major Dates

• Study Start: January 1, 2012
• Primary Completion (Anticipated): December 1, 2025
• Study Completion (Anticipated): December 1, 2025

Study Registration Dates

• First Submitted: July 13, 2011
• First Submitted That Met QC Criteria: July 18, 2011
• First Posted (Estimate): July 19, 2011

Study Record Updates

• Last Update Posted (Actual): July 14, 2022
• Last Update Submitted That Met QC Criteria: July 12, 2022
• Last Verified: July 1, 2022

More Information

Terms related to this study

• Additional Relevant MeSH Terms: Pathologic Processes; Heart Diseases; Cardiovascular Diseases; Vascular Diseases; Respiratory Tract Diseases; Lung Diseases; Disease; Congenital Abnormalities; Hypertension, Pulmonary; Cardiovascular Abnormalities; Hypertension; Syndrome; Pulmonary Arterial Hypertension; Familial Primary Pulmonary Hypertension; Heart Defects, Congenital; Eisenmenger Complex
• Other Study ID Numbers: 2011-07-12