Pulmonary Determinants of Cardiorespiratory Fitness in Fontan Patients

Study Overview
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Brief Summary
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This study will examine and record the patient's medical information and medical records (patient files, computer records, etc.). Physical examination, echocardiographic, electrocardiographic, laboratory, radiological, etc. evaluations are made during the routine controls of the patients. Within the scope of this study, no attempt will be made on the patients, no additional tests will be performed, or treatment changes will not be made in the patients because they are included in this study. After these records, KPET will be performed by a specialist doctor and physiotherapist for the patients who are directed to the Faculty of Physical Therapy and Rehabilitation Cardiopulmonary Rehabilitation Unit. Pulmonary function tests and respiratory muscle strength measurements will be performed on patients who have adequate rest after CPET.

Official Title
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Pulmonary Determinants of Cardiorespiratory Fitness in Patients With Fontan Procedure

Conditions
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Fontan Procedure, Congenital Heart Disease

Intervention / Treatment
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* Other: Cardiopulmonary exercise test
* Other: Pulmonary function test
* Other: Respiratory muscle strength test


Participation Criteria
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Eligibility Criteria
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Fontan Group Inclusion Criteria: Be between 8-55 years old Having undergone a Fontan operation Clinical stability of the patients (preserved ventricular function) No change in ongoing drug therapy that adversely affects clinical stability At least 1 year after the operation Fontan Group Exclusion Criteria: Neurological and/or genetic musculoskeletal disease Having orthopedic and cognitive problems that prevent testing The patient's and/or family's unwillingness to participate in the study Control Group Inclusion Criteria: Not have cardiovascular, neurological, and/or genetic musculoskeletal disease Not having orthopedic and cognitive problems that prevent testing The patient's and/or family's willingness to participate in the study

Ages Eligible for Study
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Minimum Age: 8 Years
Maximum Age: 55 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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Accepts Healthy Volunteers

Study Plan
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How is the study designed?
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Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Fontan group<br>Cardiopulmonary exercise test Pulmonary function test respiratory muscle strength test | Other: Cardiopulmonary exercise test<br>* CPET, the gold standard, will be used in the assessment of cardiorespiratory fitness. The cardiopulmonary exercise test system (Cosmed Quark CPET, Rome, Italy) is a safe method in which the patient can be monitored via ECG during the execution of the individual on a treadmill, and simultaneously lactate measurement, maximal O2 consumption, and CO2 production are evaluated. The Bruce protocol is the most commonly used method for KPET applied with a treadmill. However, since the Bruce protocol prefers high workload preferences, the Modified Bruce/half Bruce protocol with intermediate increments will be used. In addition, dyspnea, leg fatigue, and general fatigue at rest, during the test, and during the recovery phases after the test will be recorded with a sphygmomanometer using the Modified Borg Scale.<br>Other: Pulmonary function test<br>* Pulmonary function testing will be performed with a spirometer (Cosmed Quark CPET, Rome, Italy) connected to the CPET system using standard procedures. Forced vital capacity (FVC), forced expiratory volume in 1 second (FEV1), FEV1/FVC, peak expiratory volume measured at 25% and 75% of forced expiratory time (FEF 25-75%), and peak expiratory volume (PEF) parameters will be saved.<br>Other: Respiratory muscle strength test<br>* Intraoral maximum inspiratory pressure (MIP) and maximum expiratory pressure (MEP) will be used as indicators of respiratory muscle strength. A portable intraoral pressure gauge (MicroRPM, Micromedical, Kent, UK) and nose clip will be used during MIP and MEP assessments. MIP and MEP measurements will be obtained using standard procedures. 3 measurements will be made where the difference between each measurement is not more than 10% and the maximum value of MIP and MEP will be recorded.<br>|
| Control Group<br>Cardiopulmonary exercise test Pulmonary function test respiratory muscle strength test | Other: Cardiopulmonary exercise test<br>* CPET, the gold standard, will be used in the assessment of cardiorespiratory fitness. The cardiopulmonary exercise test system (Cosmed Quark CPET, Rome, Italy) is a safe method in which the patient can be monitored via ECG during the execution of the individual on a treadmill, and simultaneously lactate measurement, maximal O2 consumption, and CO2 production are evaluated. The Bruce protocol is the most commonly used method for KPET applied with a treadmill. However, since the Bruce protocol prefers high workload preferences, the Modified Bruce/half Bruce protocol with intermediate increments will be used. In addition, dyspnea, leg fatigue, and general fatigue at rest, during the test, and during the recovery phases after the test will be recorded with a sphygmomanometer using the Modified Borg Scale.<br>Other: Pulmonary function test<br>* Pulmonary function testing will be performed with a spirometer (Cosmed Quark CPET, Rome, Italy) connected to the CPET system using standard procedures. Forced vital capacity (FVC), forced expiratory volume in 1 second (FEV1), FEV1/FVC, peak expiratory volume measured at 25% and 75% of forced expiratory time (FEF 25-75%), and peak expiratory volume (PEF) parameters will be saved.<br>Other: Respiratory muscle strength test<br>* Intraoral maximum inspiratory pressure (MIP) and maximum expiratory pressure (MEP) will be used as indicators of respiratory muscle strength. A portable intraoral pressure gauge (MicroRPM, Micromedical, Kent, UK) and nose clip will be used during MIP and MEP assessments. MIP and MEP measurements will be obtained using standard procedures. 3 measurements will be made where the difference between each measurement is not more than 10% and the maximum value of MIP and MEP will be recorded.<br>|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Maximal oxygen consumption |  | 30-45 minutes | 
| Forced vital capacity (FVC) |  | 15-20 minutes | 
| Forced expiratory volume in 1 second (FEV1) |  | 15-20 minutes | 
| FEV1/FVC |  | 15-20 minutes | 
| Maximal inspiratory pressure |  | 15-20 minutes | 
| Maximal expiratory pressure |  | 15-20 minutes | 
| Ratio curve of minute ventilation to carbon dioxide production (VE/VCO2) slope |  | 30-45 minutes | 
| Ratio of minute ventilation to carbon dioxide production (VE/VCO2) |  | 30-45 minutes | 
| Oxygen uptake efficiency slope (OUES) |  | 30-45 minutes | 
| Minute ventilation (VE) |  | 30-45 minutes | 
| End tidal oxygen pressure (PetO2) |  | 30-45 minutes | 
| End tidal carbon dioxide pressure (PetCO2) |  | 30-45 minutes |