Exercise training in patients with corrected tetralogy of Fallot: A protocol for systematic review and meta-analysis.

BACKGROUND: The arrival of transcatheter mitral valve therapies has provided feasible and safe alternatives to medical and surgical treatments for mitral regurgitation. The aim of this study is to estimate the relative efficacy and safety of exercise training in patients with corrected tetralogy of Fallot through meta-analysis. METHODS: : A systematic search will be performed using PubMed, EMBASE, the Cochrane Library, Web of Science, CBM, CNKI, WanFang Data, and VIP to include random controlled trials or nonrandom controlled trials comparing the efficacy and safety of exercise training in corrected tetralogy of Fallot patients. The risk of bias for the included nonrandom controlled studies will be evaluated according to Risk of Bias in Nonrandomized Studies of Interventions. We will use the Cochrane Collaboration's tool (version 2 of the Cochrane risk of bias tool for randomized trials) to assess risk of bias of included random controlled trials. Revman 5.4 and STATA 15.0 will be used to complete the meta-analysis and generate forest plots. Grading of recommendations assessment, development, and evaluation will be used to assess the quality of evidence. RESULTS: : The results of this systematic review and meta-analysis will be submitted to a peer-reviewed journal for publication. CONCLUSION: : This study will provide broad evidence of efficacy and safety of exercise training in patients with corrected tetralogy of Fallot and provide suggestions for clinical practice and future research. PROTOCOL REGISTRATION NUMBER: INPLASY202150006.

Analysis of the recovery phase after maximal exercise in children with repaired tetralogy of Fallot and the relationship with ventricular function.

BACKGROUND: Few studies demonstrate delayed recovery after exercise in children and adults with heart disease. We assess the recovery patterns of gas exchange parameters and heart rate (HR) in children with repaired Tetralogy of Fallot (rToF) compared to healthy peers and investigate the correlation with ventricular function and QRS duration. METHODS: 45 children after rToF and 45 controls performed a maximal incremental cardiopulmonary exercise test. In the subsequent recovery period, patterns of VO2, VCO2 and HR were analysed. Half-life time (T1/2) of the exponential decay and drop per minute (Recmin) were compared between groups. In the rToF group, correlations were examined between the recovery parameters and QRS-duration and ventricular function, described by fractional shortening (FS) and tricuspid annular plane systolic excursion (TAPSE) measured at baseline prior to exercise. RESULTS: Recovery of VO2 and VCO2 was delayed in rToF patients, half-life time values were higher compared to controls (T1/2VO2 52.51 ±11.29 s vs. 44.31 ± 10.47 s; p = 0.001 and T1/2VCO2 68.28 ± 13.84 s vs. 59.41 ± 12.06 s; p = 0.002) and percentage drop from maximal value was slower at each minute of recovery (p<0.05). Correlations were found with FS (T1/2VO2: r = -0.517; p<0.001; Rec1minVO2: r = -0.636, p<0.001; Rec1minVCO2: r = -0.373, p = 0.012) and TAPSE (T1/2VO2: r = -0.505; p<0.001; Rec1minVO2: r = -0.566, p<0.001; T1/2VCO2: r = -0.466; p = 0.001; Rec1minVCO2: r = -0.507, p<0.001), not with QRS-duration. No difference was found in HR recovery between patients and controls. CONCLUSIONS: Children after rToF show a delayed gas exchange recovery after exercise. This delay correlates to ventricular function, demonstrating its importance in recovery after physical activity.

Biventricular myocardial adaptation in patients with repaired tetralogy of Fallot: Mechanistic insights from magnetic resonance imaging tissue phase mapping.

BACKGROUND: The myocardial adaptive mechanism in patients with repaired tetralogy of Fallot (rTOF) is less understood. We aimed to investigate biventricular myocardial adaptive remodeling in rTOF patients. METHODS: We recruited 32 rTOF patients and 38 age- and sex-matched normal controls. The pulmonary stenosis of rTOF patients was measured using catheterized pressure gradient between right ventricle (RV) and pulmonary artery (PGRVPA). rTOF patients with PGRVPA < 15 mmHg and ≥15 mmHg were classified as low pulmonary stenosis (rTOFlow, n = 19) and high pulmonary stenosis (rTOFhigh, n = 13) subgroups, respectively. Magnetic resonance imaging tissue phase mapping was employed to evaluate the voxelwise biventricular myocardial motion in longitudinal (Vz), radial (Vr), and circumferential (Vφ) directions. RESULTS: The rTOFlow subgroup presented higher pulmonary regurgitation fraction than rTOFhigh subgroup (p < 0.001). Compared with the normal group, only rTOFlow subgroup presented a decreased RV ejection fraction (RVEF) (p < 0.05). The rTOFlow subgroup showed decreased systolic and diastolic Vz in RV and LV, whereas rTOFhigh subgroup showed such change only in RV. In rTOFlow subgroup, RVEF significantly correlated with RV systolic Vr (r = 0.56, p < 0.05), whereas LVEF correlated with LV systolic Vz (r = 0.51, p = 0.02). Prolonged QRS correlated with RV systolic Vr (r = -0.58, p < 0.01) and LV diastolic Vr (r = 0.81, p < 0.001). No such correlations occurred in rTOFhigh subgroup. CONCLUSIONS: The avoidance of unfavorable functional interaction in RV and LV in rTOFhigh subgroup suggested that adequate pulmonary stenosis (PGRVPA ≥ 15 mmHg in this sereis) has a protective effect against pulmonary regurgitation.

Surgical Management of Fallot's Tetralogy With Pulmonary Atresia and Major Aortopulmonary Collateral Arteries: Multistage Versus One-Stage Repair.

A strict and rational approach to Fallot's tetralogy with pulmonary atresia and major aortopulmonary collateral arteries allows to achieve optimal results. Rehabilitative and unifocalization strategies do not constitute separate philosophies; instead the surgical strategy should be tailored to each individual patient. Based on our previous experience, the ability to achieve definitive intracardiac repair is the real determinant of both improved survival and adequate systolic right ventricular performance on mid-term follow-up.

Cardiac rehabilitation in an adolescent with DiGeorge Syndrome.

BACKGROUND: DiGeorge Syndrome is a rare disease that has variable clinical symptoms resulting from 22q11 deletions, included cardiac abnormality, abnormal face and thymic aplasia, and cognitive impairment. There was a no reports regarding the efficiency of cardiac rehabilitation (CR) in patients with DiGeorge Syndrome with tetralogy of Fallot. CASE REPORT: A 15-year-old girl with DGS visited our CR center. The patient carried out the exercise training 3 times a week for 6 weeks, using a treadmill with electrocardiogram monitoring. Exercise tolerance testing and Quality of life assessment were performed before and after 6 weeks of training. Improvement of aerobic capacity was not represented, but by her own estimation, her performance ability of daily activities was better than before. CLINICAL REHABILITATION IMPACT: Even though adolescents with congenital heart disease often limit themselves, or are restricted by others, from physical activity, CR should be recommended as a comprehensive health promotion strategy.

The impact of exercise on ventricular arrhythmias in adults with tetralogy of Fallot.

BACKGROUND: Sudden death of presumed arrhythmic etiology is the leading cause of mortality in adults with tetralogy of Fallot. OBJECTIVES: To assess the impact of exercise on ventricular arrhythmias in adults with tetralogy of Fallot. METHODS: Adults with repaired tetralogy of Fallot were randomized in a 2:1 ratio to an open-label trial of standard care versus 12weeks of supervised combined aerobic/resistance training with continuous Holter monitoring. Proportion of premature ventricular complexes (PVC) and runs of non-sustained ventricular tachycardia (NSVT) were assessed by mixed and Poisson regression models with generalized estimating equations for repeated measures. RESULTS: A total of 152 Holters were performed in 17 patients, median age 35 (interquartile range [IQR] 28, 42) years, 65% male, 13 of whom were randomized to exercise training. Baseline characteristics were similar between groups. Exercise training resulted in significant increases in peak oxygen uptake (11±19%, p=0.028), metabolic equivalents (11±18%, p=0.027), and exercise duration (8±10%, p=0.009) compared to no changes in controls. Frequent (≥30 per hour) PVCs were present in 46% of patients, couplets in 62%, and 3 to 7 beat runs of NSVT in 31%. The median proportion of PVCs was 1.93‰ (IQR 0.41, 5.89) at baseline and 1.45‰ (IQR 0.08, 2.76) during the initial exercise session (p=0.722), and remained stable over time (ß coefficient=-0.031, p=0.408). Runs of NSVT decreased significantly over time (ß coefficient=-0.032, p=0.018). CONCLUSION: In adults with repaired tetralogy of Fallot, exercise training is safe, improves exercise capacity, and appears to confer a beneficial effect on ventricular arrhythmias.

Does exercise training improve cardiopulmonary fitness and daily physical activity in children and young adults with corrected tetralogy of Fallot or Fontan circulation? A randomized controlled trial.

BACKGROUND: Many patients with congenital heart disease do not meet current public health guidelines to participate in moderate-to-vigorous physical activity for ≥60 minutes per day. They are less fit than their healthy peers. We hypothesized that exercise training would increase cardiopulmonary fitness and daily physical activity in these patients. We therefore assessed effects of an exercise training program on cardiopulmonary fitness and daily physical activity in patients with corrected tetralogy of Fallot (ToF) or Fontan circulation. METHODS: In a multicenter prospective controlled trial, patients with ToF or Fontan circulation (age 10-25 years) were randomized, 56 patients to the exercise group and 37 to the control group. The exercise group participated in a 12-week standardized aerobic exercise training program. The control group continued lifestyle as usual. Cardiopulmonary exercise testing and activity measurements were performed before and after 12 weeks. RESULTS: Peak oxygen uptake increased in the exercise group by 5.0% (1.7 ± 4.2 mL/kg per minute; P = .011) but not in the control group (0.9 ± 5.2 mL/kg per minute; P = not significant). Workload increased significantly in the exercise group compared with the control group (6.9 ± 11.8 vs 0.8 ± 13.9 W; P = .047). Subgroup analysis showed a significant increase in pre-to-post peak oxygen uptake in the exercise group of ToF patients but not in the exercise group of Fontan patients. Percentage of measured time spent in moderate-to-vigorous activity at baseline was 13.6% ± 8.6%, which did not significantly change after training. CONCLUSIONS: Aerobic exercise training improved cardiopulmonary fitness in patients with ToF but not in patients with Fontan circulation. Exercise training did not change daily physical activity.

The effect of exercise training on cardiac remodelling in children and young adults with corrected tetralogy of Fallot or Fontan circulation: a randomized controlled trial.

BACKGROUND: Exercise can improve physical fitness in children and adults with congenital heart disease. We hypothesized that exercise training would not lead to adverse cardiac remodelling in this population. METHODS AND RESULTS: This multi-centre randomized controlled trial included children and young adults (10 to 25 years) with either corrected tetralogy of Fallot or Fontan circulation. The exercise-group was enrolled in a 12 week standardized aerobic dynamic exercise training program. The control-group continued their life-style and received care as usual. Both groups underwent cardiopulmonary exercise testing, cardiac magnetic resonance imaging (MRI), echocardiography and neurohormonal assessment, within 2 weeks before and 2 weeks after the intervention period. Fifty-six patients were randomized to the exercise-group and 37 to the control-group. We assessed changes between the pre- and the post-intervention period for the exercise group compared to the changes in the control-group. Peak load increased significantly in the exercise-group compared to the control-group (exercise-group 6.9 ± 11.8 W; control-group 0.8 ± 13.9 W; p=0.047). There were no adverse events linked to the study. Ventricular systolic parameters, cardiac dimensions and neurohormonal markers during follow-up did not change in patients allocated to the exercise-group and control-group. Although there were some isolated minor changes in inflow parameters, there was no consistent pattern of changes, indicating a lack of true change in the diastolic function. CONCLUSION: We demonstrated that no clinically relevant adverse cardiac remodelling occurred after 12 weeks of exercise training in patients with either corrected tetralogy of Fallot or Fontan circulation. CLINICAL TRIAL REGISTRATION: www.trialregister.nl, identification NTR2731.

Parental mental health moderates the efficacy of exercise training on health-related quality of life in adolescents with congenital heart disease.

To evaluate the moderating influence of parental variables on changes in health-related quality of life (HRQoL) in adolescents with Tetralogy of Fallot (ToF) or a Fontan circulation after participation in standardized exercise training. A multicenter randomized controlled trail in which 56 patients, aged 10-15, were randomly allocated (stratified by age, gender, and congenital heart disease) to a 12-week period with either: (a) 3 times per week standardized exercise training or (b) care-as-usual (randomization ratio 2:1). Adolescents and their parents filled in online questionnaires at baseline and at 12-week follow-up. In this randomized controlled trail, primary analyses involved influence of parental mental health and parental social support for exercise on changes in the TNO/AZL Child Quality of Life Questionnaire Child Form at follow-up. Secondary analyses concerned comparing levels of parental characteristics with normative data. Compared with controls, adolescents in the exercise group reported a decrease in social functioning when their parents had more anxiety/insomnia or severe depression themselves. Adolescents also reported a decrease in social functioning when their parents showed poorer overall mental health themselves. Parents reported comparable or even better mental health compared with normative data. The effect of a standardized exercise program on HRQoL changes in adolescents with ToF or a Fontan circulation is moderated by parental mental health, more specifically by parental anxiety/insomnia and severe depression. The trial registration number of this article is NTR2731 ( www.trialregister.nl ).

Aerobic exercise influences quality of life of children and youngsters with congenital heart disease: a randomized controlled trial.

PURPOSE: To evaluate effects of an exercise program on health-related quality of life (HRQoL) in children and adolescents with tetralogy of Fallot (ToF) or a Fontan circulation. METHODS: Stratified, randomized, controlled intervention study conducted in five participating centers of pediatric cardiology in The Netherlands. In total, 93 patients, aged 10-25 years, with surgical repair for tetralogy of Fallot or with a Fontan circulation for single-ventricle physiology were included. They were randomly allocated with a ratio of 2:1 to: (1) a 12-week period with an exercise program for 3 times per week or (2) to a control group. Randomization was stratified by age, gender, and cardiac diagnosis. At baseline and follow-up after 12 weeks, all participants completed Web-based age-appropriate HRQoL questionnaires. Primary analyses involved change in HRQoL during follow-up. Secondary analyses concerned influence of cardiac diagnosis and comparison with normative data. RESULTS: Forty-eight (86%) and 32 (86%) patients in the exercise-group and control-group respectively completed all questionnaires at baseline and follow-up. Compared with the control-group, children, aged 10-15 years, in the exercise-group improved significantly on self-reported cognitive functioning, p < .05, r = .30, and parent-reported social functioning, p < .05, r = .30. Youngsters aged 16-25 years did not change their HRQoL. Cardiac diagnosis had no influence on pre/post changes. Children and youngsters in this study reported comparable or better HRQoL than norm groups. CONCLUSIONS: Participation in an exercise program improved HRQoL of children with ToF or a Fontan circulation, especially in those with low baseline QoL.

Timing of pulmonary valve replacement after tetralogy of Fallot repair.

Tetralogy of Fallot (TOF) is the most common form of cyanotic congenital heart disease. If left untreated, it carries a 33% mortality in the first year of life and a 50% mortality in the first 3 years of life. Since the introduction of the first open-heart repair by Lillehei and Varco in 1954, surgical management of TOF has evolved to be the primary repair during infancy in the majority of patients. Surgical management of TOF results in anatomic and functional abnormalities in the majority of patients, such as chronic pulmonary valve regurgitation and right ventricular (RV) dysfunction. Long-standing chronic pulmonary valve regurgitation can result in RV dilatation and failure, increasing tricuspid regurgitation, impaired exercise performance and supraventricular or ventricular arrhythmias. A timely reoperation may prevent these consequences, with a complete RV-function recovery. This article provides insight into the questions of when to perform a pulmonary valve implantation and in whom.

Pulmonary atresia with ventricular septal defect and multifocal pulmonary blood supply: does an intensive interventional approach improve the outcome?

INTRODUCTION: Pulmonary atresia with ventricular septal defect (VSD) continues to be associated with significant morbidity and mortality, with significant institutional variation in therapeutic strategies. This study reports a single center experience utilizing an intensive transcatheter approach to promote pulmonary vascular growth. METHODS: A retrospective analysis of 20 patients undergoing surgical and transcatheter treatment for pulmonary atresia with VSD between 2002 and 2010. RESULTS: The median age at initial surgical palliation was 6.3 months (8 days to 2.5 years). Eleven patients (group 1) underwent initial surgical palliation without VSD closure and nine patients (group 2) underwent an initial complete repair with fenestrated or complete VSD closure. Group 1 had a smaller Nakata index (54 mm2/m2 vs. 134 mm2/m2 , P = .04) and a smaller absolute native pulmonary artery diameter (2.7 mm vs. 4.5 mm, P = .01) than group 2. Intraoperative angiography was performed in 10 cases to evaluate if early transcatheter intervention was warranted. The median follow-up during the study period was 2.3 years (1.6 months to 8.3 years). Of the 16 patients who survived the initial early postoperative period, 15 patients (94%) went on to receive surgical (n = 11) and/or interventional (n = 25) catheterization procedures. There was improvement in the mean Nakata index from the initial presurgical evaluation to the most recent catheterization data (38.4 mm2/m2 vs. 169.7 mm2/m2, P ≤ .05). To date, two of 11 (18%) patients in group 1 ultimately underwent surgical VSD closure. Overall mortality was six of 20 (30%) with four deaths in group 1 and two deaths in group 2. There were no procedural deaths. CONCLUSIONS: Combining surgical unifocalization procedures with subsequent early and intensive catheter-based pulmonary artery rehabilitation may improve vascular growth, ultimately rendering many patients suitable for fenestrated VSD closure. Risk stratification, including intraoperative exit angiography, is essential to determine the need for early transcatheter interventions.

Quality of life of grown-up congenital heart disease patients after congenital cardiac surgery.

BACKGROUND: Due to better early and long-term outcome, the increasing population of grown-ups with congenital heart disease (GUCH) brings up unexpected quality of life (QoL) issues. The cardiac lesion by itself is not always the major problem for these patients, since issues pertaining to QoL and psychosocial aspects often predominate. This study analyses the QoL of GUCH patients after cardiac surgery and the possible impact of medical and psychosocial complications. PATIENTS AND METHODS: A questionnaire package containing the SF-36 health survey (health related QoL), the HADS test (anxiety/depression aspects) and an additional disease specific questionnaire was sent to 345 patients (mean 26+/-11 years) operated for isolated transposition of the great arteries (TGA), tetralogy of Fallot (TOF), and ventricular septal defect (VSD). The scores were compared with age- and gender-matched standard population data and in relation to the underlying congenital heart disease (CHD). RESULTS: In all SF-36 and HADS health dimensions the GUCH patients showed excellent scores (116+/-20), which are comparable to the standard population (100+/-15), regardless of the initial CHD (p=0.12). Eighty-two percent of the patients were found to be in NYHA class I and 83% patients declared that they do not consider their QoL to be limited by their malformation. Complications like reoperations (p=0.21) and arrhythmias (p=0.10) do not show significant impact on the QoL. The additional questionnaire revealed that 76% of adult patients have a fulltime job, 18% receive a full or partial disability pension, 21% reported problems with insurances, most of them regarding health insurances (67%), and 4.4% of adult patients declared to have renounced the idea of having children due to their cardiac malformation. CONCLUSION: QoL in GUCH patients following surgical repair of isolated TOF, TGA and VSD is excellent and comparable to standard population, this without significant difference between the diagnosis groups. However, these patients are exposed to a high rate of complications and special psychosocial problems, which are not assessed by standardized questionnaires, such as the SF-36 and HADS. These findings highlight the great importance for a multidisciplinary and specialized follow-up for an adequate management of these complex patients.

[17th part: Transvenous pacemaker implantation in corrected Fallot tetralogy with heart failure]. / 17. Folge: Transvenöse Schrittmacher-Implantation bei korrigierter Fallot-Tetralogie mit Herzinsuffizienz.

Implantation of transvenous pacemaker leads can be particularly--and unexpectedly--difficult in patients with corrected congenital heart disease. If the operation has been performed a long time ago, there are frequently no more reports about the exact intervention available. The present case illustrates the importance of the ECG and fluoroscopy during implantation in complex congenital heart disease.

A pilot study of exercise training in adult patients with repaired tetralogy of Fallot.

BACKGROUND: Positive effects of physical training in adults with acquired heart disease have been reported. The role of exercise training in adults with congenital heart disease, however, is less well defined. OBJECTIVES: To assess the level of physical activity in adult patients with repaired tetralogy of Fallot (TOF), as well as the safety and effect of exercise training in these patients. PATIENTS AND METHODS: A questionnaire of physical activity was filled upon entering the study by the participants. Eighteen adult patients with repaired TOF were then randomly assigned to participate in a three-month structured exercise program (exercise group, nine patients) or to continue their usual exercise routine (control group, nine patients). Each patient in the exercise group received an individualized exercise program to be performed three times a week, whereas the control group was told to continue living their life as usual. Cardiopulmonary testing was performed in all patients at baseline (before randomization) and at the end of the study. RESULTS: No death or incidents occurred during the study period. All patients but one were physically inactive at the onset of the study. There was a significant increase in peak oxygen consumption in the exercise group by the end of the study (22.1 mL/kg/min [6 metabolic equivalents (METS)] versus 24.3 mL/kg/min [7 METS], P=0.049), whereas it remained unchanged in the control group (21.8 mL/kg/min [6 METS] versus 22.1 mL/kg/min [6 METS], P=0.825). CONCLUSION: In clinically stable adult patients with repaired TOF, a moderate level of exercise training improves aerobic capacity. Regular exercise should be encouraged in these sedentary patients.

Reliability of subjective estimates of exercise capacity after total repair of tetralogy of Fallot.

Sixty-nine patients (age 10 +/- 3.5 years, median 9.7 years) operated on for tetralogy of Fallot, underwent exercise testing 5.1 +/- 2.5 years after total correction. Aerobic capacity was assessed by the ventilatory threshold method. After the exercise test, parents completed a validated standardized questionnaire to evaluate the habitual level of physical activity of their child. They were then asked to place their child into one of three groups, describing their child's activity level, compared to normals (below average, average and above average). In the average and above-average groups, 73% and 65% of the patients were misclassified when compared to objective exercise testing. However, in the below-average group, no misclassifications were found. Subjective estimates of activity level, and consequently exercise tolerance, were poorly associated with objective measurements of exercise performance in these patient groups. Our results question the reliability of self-reported estimates of physical activity level and consequently exercise capacity in children after surgical correction of tetralogy of Fallot.

[Tetralogy of Fallot. Physical work capacity after its total correction]. / Zespól Fallota. Wydolnosc fizyczna po korekcji calkowitej.

Results of exercise tests in 21 children after total correction of Fallot tetralogy (mean 5 years after surgery) are reviewed. Maximal exercise capacity recalculated for body mass and for total body area was approximately 60% of normal values. Maximal heart rate was lower than among healthy children. There was no statistical correlation between exercise capacity nad age while operated, nor time after surgery.

[Evaluation of the quality of life in adulthood of 158 patients surgically-treated for tetralogy of Fallot]. / Evaluation de la qualité de vie d'adulte de 158 patients opérés de tétralogie de Fallot.

Open heart correction of tetralogy of Fallot is now carried out in infancy. This operation provides children with the best physical and mental conditions for them to complete their education. Until recently, follow-up had been too short to assess the results of repair during infancy in adults. The object of this study was to evaluate the quality of adult life in the first 158 survivors of this surgery, now aged 19 to 36 years, performed in our department since 1968. Ninety eight per cent of these patients are physically capable of leading a normal life; 24% obtained their "baccalaureate" and hold positions of high professional responsibility; 37% achieved basic education diplomas. Even those who left school without qualifications were usually able to find employment. The number of unemployed is less than that of the same age group in the general population. Thirty per cent are married or live with someone, and twenty five per cent have normal healthy children. The large majority have normal active lives. Thirty per cent play sport regularly as a leisure activity. Notwithstanding, all these patients were operated for severe symptoms. Two thirds had had previous Blalock shunts. Severe and persistent cyanosis is not a sign of anatomic severity of tetralogy of Fallot and does not seem to influence the socio-professional outcome. These results are those of what was considered to be high risk surgery at the time because of the inaccuracy of angiographic diagnosis, the inadequacy of myocardial protection and the chronicity of the anatomo-pathological lesions (average age of operated patients was 8 years).(ABSTRACT TRUNCATED AT 250 WORDS)

Rehabilitation of children after total correction of tetralogy of Fallot.

Nine children who had undergone total correction of tetralogy of Fallot were studied with the purpose of observing the physical changes that might be produced by a period of rehabilitation in hospital and comparing these results with those of a control group having the same pathology but which had not followed a protocol for physical activity. Precise criteria were used in the selection of the patients. Careful clinical examinations and exercise tests were conducted before and after the programme, in particular maximal stress testing on the bicycle ergometer and submaximal exercise on the treadmill. The sessions were held three times weekly in a gymnasium over three months, gradually increasing the duration of each session to a maximum of one hour. During the session, heart rate was monitored with a Sport-tester 3000 so as not to exceed 60%, then 70%, of the maximal heart rate recorded during the stress test. Testing at the end of the programme demonstrated an improvement in tolerance under maximal stress in 7 of the 9 patients. As for submaximal performance, 8 out of 9 children covered a greater distance using the same parameters as for initial testing. At the end of the programme, the children all showed increased independence and initiative and more self-confidence in establishing social relations. The programme has proven to be a comprehensive method for safely improving physical fitness in these patients and represents an important starting point for a better future.