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.

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 ).

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.

[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.

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.

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.

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.

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.

[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)

[Tetralogy of Fallot. 20 years' experience in 144 cases]. / Tétralogie de Fallot. Vingt ans d'expérience portant sur 144 cas.

Between 1968 and December 1987, 144 patients with tetralogy of Fallot were examined at La Pitié Hospital, Paris. 76 were female and 68 male, with a mean age of 8.1 years at the first visit. The patients were regularly followed up by the same physician relying on radiography of the chest, electrocardiography and, since 1982, two-dimensional echocardiography. These visits were coupled with an interview with the welfare officer attached to our department for information on the patient's way of life as well as his socio-professional and familial problems. 129 patients of mean age 14.8 years underwent corrective surgery preceded in 81 cases by palliative surgery. The overall immediate mortality rate was 12.4% (16 cases), falling from 19.5% between 1968 and 1977 to 3% during the last 10 years. The mean follow-up period was 10.7 years, with 51 patients being followed up for more than 10 years and 18 for more than 20 years. Late mortality now stands at 5.3% (7 patients, 6 of whom died of a cardiac cause). Residual lesions consisted in significant (27%) pulmonary insufficiency in 35 patients, residual interventricular septal defect in 16 patients (12.4%) and pulmonary obstruction in 11 patients (8%). 18 patients presented with dysrhythmias, including 7 cases of ventricular arrhythmia; 5 two-bundle blocks and 5 complete atrioventricular blocks were also observed. 11 patients required reoperation with a 27.2% mortality rate (3 cases).(ABSTRACT TRUNCATED AT 250 WORDS)

[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.

[Long-term (10 to 23 years) clinical evaluation of quality of life in patients following open heart repair of tetralogy of Fallot].

The quality of life of 78 patients out of 79 (99% follow-up rate) was evaluated 10 to 23 years following intracardiac repair of tetralogy of Fallot. Mean age at operation was 6 years of age. Their present ages ranged from 12 to 35, averaging 21 years old. Eighty three percent of patients are assessed to be in good quality of life so far in terms of physical development, life style, CTR values, Ekg findings and so on. Pregnancy and delivery in 5 patients were uneventful. On the other hand, 17% showed various degree of morbidity including significant residua necessitating reoperation in 3 patients, complications such as complete A-V Block requiring pacemaker implantation in 7 and sequela like postoperative life threatening cardiomyopathy in 3. There were 2 deaths due to this sequela comprising 2.6% of mortality rate. Although excellent results are reported in recent years, further efforts will be necessary to minimize residuum, complication and sequela after surgery especially for this particular disease with broad morphological spectrum.

[Long-term follow-up after repair of tetralogy of Fallot].

One hundred sixty-six patients with tetralogy of Fallot including 15 extreme type-patients repaired since 1971 were analyzed with respect to their physical activities, school or social life, hemodynamics, ECG, reoperation and late mortality. Follow-up period ranged from 1 to 19 years with an average of 9.1. There were 9 late deaths including 4 sudden deaths. Actuarial survival rates at 10 and 19 years were 95.2% and 90.5%, respectively. Eighty-five patients (72.0%) of 118 replied were in NYHA functional class I, 31 (26.3%) in class II and 2 (1.7%) in class III. Thirteen patients (7.8%) demonstrated right ventricular/left ventricular systolic pressure ratio over 0.80 because of inadequate relief of the pulmonary stenosis or obstruction, hypoplasia of the pulmonary artery or external conduit stenosis. Although the left ventricular ejection fraction was maintained at normal range (0.65 +/- 0.09), that of the right ventricule was below normal level (0.52 +/- 0.09). Two patients developed complete heart block postoperatively and underwent pacemaker implantations. Complete right bundle branch block was observed in 85 patients including 6 with left axis deviation. It is concluded that early correction even in the early infancy, adequate enlargement of the right ventricular outflow tract and minimal right ventriculotomy should be applied for correction of tetralogy of Fallot.

[Postoperative long-term quality of life at present after total correction of tetralogy of Fallot].

We performed a questionnaire study in 47 patients who underwent corrective surgery for tetralogy of Fallot (TF) more than 10 years previously. To evaluate quality of life, we used well-being score advocated by Kaplan. 12 patients whose well-being score was 1.0 were in entirely good health without any complaints. Well-being score of patients averaged 0.773 with the range from 0.58 to 1.0. The patient with high RV/LV pressure ratio or large CTR tended to have a low well-being score. Grade of Lown classification did not correlate to well-being score.

[Long-term result of total correction of tetralogy of Fallot].

Long-term result of total correction of tetralogy of Fallot was investigated in 51 patients who underwent operation at least 10 years previously. Ninety percent of the patients who responded to the questionnaires expressed his daily life as satisfactory and 4% of the patients had no exercise limitation even in strenuous sports like swimming and running. It was disclosed that the exercise capacity in long-term period was thought to be prescribed by the developmental state of the pulmonary vascular bed at the time of operation which was expressed by the preoperative hemoglobin level and the PA/Ao diameter ratio and no correlation was found with the RV/LV pressure ratio recorded immediately after correction. Three patients died after discharge, 2 years, 2 years and 6 months and 21 years after operations respectively and the cause of death was supposed to be arrhythmia. In case of postoperative complete right bundle block associated with an extreme right axis deviation or left axis deviation, prophylactic pacemaker implantation was recommended.

[Adaptive reactions of the erythrocytes in the evaluation of physical work capacity in patients with tetralogy of Fallot]. / Adaptatsionnye reaktsii eritrotsitov v otsenke fizicheskoi rabotosposobnosti bol'nykh s tetradoi Fallo

One of the leading factors affecting the functional state of the red blood system is arterial hypoxemia. Under such conditions signs of polycythemia are known to appear. At the same time, changes in the quality characteristics of the blood develop in patients with congenital heart diseases (tetralogy of Fallot including). Therefore a study of the physical rehabilitation as a total functional index may describe the adaptation of the body to hypoxemia, the variants and stages of its development. For this purpose the red blood parameters were examined (the content of hemoglobin, the erythrocyte count and their diameter) and the tolerance of physical exercises was tested by ergometry. The data were analyzed with due regard of the age of the patients and the reduction of their capacity for work, as compared to the due level. Stage 1 reduction of the capacity for work was found in patients with high red blood parameters. In groups of patients with Stage 2, 3 and especially 4 reduction of working capacity the red blood indices were significantly reduced. A distinct correlation was revealed between the erythrocyte count and their diameter, on the one hand, and the capacity for work, on the other.