Primary Objective(s): - To determine the relation between CPET and pulmonary and muscular performance in patients with Tetralogy of Fallot.- To study the effect of previous lateral thoracotomy on pulmonary function and exercise.Secondary Objective(s…
ID
Source
Brief title
Condition
- Congenital cardiac disorders
- Cardiac and vascular disorders congenital
Synonym
Research involving
Sponsors and support
Intervention
Outcome measures
Primary outcome
Main endpoint: Cardiopulmonary exercise testing (peak VO2).
Main study parameters:
1. Cardiac function (EF on MRI)
2. Pulmonary function (FVC)
3. Muscle strength (BOT-2)
Secondary outcome
1. CPET variables: peak workload, peak Ve, Ve/VCO2 slope, O2-pulse, heart rate
response to exercise (28).
2. Cardiac assessment: residual lesions on echo (valve insufficiency and/or
stenosis) graded according to Hamer and Pieper. Ventricular morphology and
volumes on MRI (29)
3. Pulmonary function: total lung capacity (TLC), force expiratory volume in 1
second (FEV1), maximal inspiratory/expiratory pressures (MIP/MEP), residual
volume (16,17).
4. Muscle strength: handgrip strength, isotonic muscle strength (hand-held
dynamometer of quadriceps, triceps and biceps).
5. Quality of life assessed with PedsQL.
6. Physical activity measured with accelerometer.
Background summary
Tetralogy of Fallot (ToF) is the most prevalent form of cyanotic congenital
heart disease (ConHD), affecting the right ventricle and pulmonary arteries.
Although mortality is low, morbidity (caused by inevitable residual lesions
after surgery) is increasing threatening the Quality of Life (QoL) of the
patient. Surgical strategies have evolved in time, aimed to reduce the effect
of prolonged right ventricular overload. However, it is yet unknown if these
changes, indeed reduce the risk of attrition. Studies in children and young
adults with ToF in the literature are scarce.
The golden standard to monitor the cardiac function in ToF patients is to
perform a cardiopulmonary exercise test (CPET), with primary outcome peak
oxygen uptake (peak VO2). The CPET is not only an indicator for cardiac
function, but also for pulmonary function and muscle strength. Previous studies
demonstrated a decreased peak VO2 in young patients with ToF. Whether this is
related to cardiac function is doubtful. Patients with ConHD have a higher
prevalence of restrictive lung patterns and respiratory and skeletal muscle
weakness. Also, patients with ConHD tend to be less physically active than
their peers. In our preliminary study, we studied 28 ToF patients and 11
healthy controls, are results were similar to findings in the literature.
Additionally, patients with a previous Blalock-Taussig shunt (BT-shunt) showed
a disadvantage compared to primary repaired ToF patients. Therefore we
hypothesize: 1. Reduced pulmonary function and reduced muscular strength,
rather than cardiac function, have a negative effect on CPET outcome (peak
VO2). 2. Previous lateral thoracotomy has a negative effect on the CPET outcome
(peak VO2) due to a poorer pulmonary function. 3. QoL correlates with clinical
status cardiac function, pulmonary function and muscle strength. 4. Physical
inactivity has a negative effect on CPET (peak VO2) results due to lower muscle
strength.
Study objective
Primary Objective(s):
- To determine the relation between CPET and pulmonary and muscular performance
in patients with Tetralogy of Fallot.
- To study the effect of previous lateral thoracotomy on pulmonary function and
exercise.
Secondary Objective(s):
- To determine pulmonary function and muscle strength in children and
adolescents after early ToF-repair.
- To assess the relationship between clinical parameters (heart, lung, muscle)
with QoL of children and adolescents after ToF-repair.
- To determine predictors of a decreased peak VO2.
- To study the physical activity in relation to exercise performance and muscle
strength.
Study design
Observational cross-sectional study (period end-2015 until mid-2019).
Study burden and risks
Benefits for patients and controls
The study leads to a detailed analysis of the physical performance of the
participant. This analysis will be shared with the participants and their
parents/legal representatives. Participants and parents/legal representatives
can receive a personal patient-tailored advice to improve their physical status
based upon these results.
The burden for patients and controls is a longer stay in the hospital of
additionally 1.5 respectively 2 hours. Also, during the pulmonary function test
an unknown obstructive lung pattern may be revealed. Additional examinations
and treatment will be started if necessary. The control group will undergo the
CPET instead of an ergometry. Ergometry is easier and without a face mask. The
burden for the muscle examinations, accelerometer and general QoL questionnaire
are low.
Risk assessment for both groups: All examinations to be carried out in this
study are part of routine follow-up care in other pediatric patient groups
(e.g. pulmonary function in asthma and cystic fibrosis, muscle strength tests
in muscle dystrophy). Pulmonary function testing and muscle strength assessment
are without risks. Complications that may occur during a CPET are chest pain
(0.69%), dizziness or syncope (0.29%), decreased blood pressure (0.35%) and
hazardous arrhythmias (0.46%). The overall incidence of complications during
pediatric exercise testing is 1,79% (Alpert et al). However children and young
adults who will be included in the study have no restrictions in their level of
physical exercise. In daily life they are subject to peak exercise levels,
without any type of monitoring.
Hanzeplein 1
Groningen 9700 RB
NL
Hanzeplein 1
Groningen 9700 RB
NL
Listed location countries
Age
Inclusion criteria
1) Children and adolescents corrected for Tetralogy of Fallot, 21 years old or younger (minimum age of 8) followed routinely in the University Medical Centre Groningen.
2) Children and adolescents with a corrected Coarctatio aortae, 21 years old or younger (minimum age of 8) followed routinely in the University Medical Centre Groningen.
3) Height > 120 cm in order to be able to fit on the bicycle.
4) Written informed consent.
Exclusion criteria
For all participants
- Patients with mental retardation.
- Patients who have contra-indications for exercise testing.
- Patients known with a muscular dystrophy (such as Becker or Duchenne).
- Patients with instable lung disease.
- Patients with previous surgery for scoliosis.
- Pregnant patients;For patients with coarctatio aortae
- Patients with more than one surgical intervention.
- Patients known to suffer from a hemodynamic significant re-coarctation (definition: pressure differences between ascending and descending aorta > 20 mmHg).
Design
Recruitment
Followed up by the following (possibly more current) registration
No registrations found.
Other (possibly less up-to-date) registrations in this register
No registrations found.
In other registers
| Register | ID |
|---|---|
| CCMO | NL53571.042.15 |