Arterial switch operation for transposition of the great arteries: A single-centre 32-year experience.

OBJECTIVE: Arterial switch operation (ASO) is nowadays the standard of care for neonates with D-transposition of the great arteries (D-TGA). We evaluated our early and late results with ASO for D-TGA. MATERIALS AND METHODS: We collected data on 267 patients with D-TGA, either with intact ventricular septum (D-TGA/IVS; n = 182, 68%) and with other associated congenital heart malformations (complex D-TGA; n = 85, 32%) that underwent ASO between January 1987 and July 2018. RESULTS: Median age at ASO was 8 days (interquartile range [IQR], 6-12 days). Fifteen patients (5.6%) died in hospital (6/182 with D-TGA/IVS, 3.3% and 9/85 with complex D-TGA, 11%; P = 0.02). Median follow-up time was 10.2 years (IQR, 3.7-18 years). There were 2 (0.8%) late sudden deaths. Overall survival at 10 and 20 years was 94% and 93%, respectively. Thirty-five patients (14%) required either reoperations or reinterventions, mainly for right ventricular outflow tract obstruction (n = 28, 11%). Freedom from reoperation/reintervention at 10 and 20 years was 87% and 78%, respectively. All patients were in NYHA I at latest clinical examination. Six over 173 patients (3.4%) who underwent a postoperative evaluation of their coronaries presented acquired anomalies. Forty-four patients (17%) who performed a cardiopulmonary exercise testing (CPET) have a predicted VO 2 comparable to normal peers. CONCLUSIONS: The results of ASO for D-TGA are excellent, with a fairly low mortality and reoperation/reintervention rate. Functional capacity evaluated with CPET is comparable to normal peers. Continuous follow-up for detecting asymptomatic acquired coronary artery disease is mandatory. A reassessment of competitive sport eligibility criteria for specific D-TGA patients should be considered.

Serial cardiopulmonary exercise testing in young patients after one-and-half ventricle repair and Fontan procedure: a comparative study.

AIM: The Fontan procedure is a palliative surgical treatment for different congenital heart diseases with a univentricular heart, but it has been associated with decreased exercise capacity, cardiovascular morbidity, and premature mortality. The one-and-half ventricle repair (1.5VR) was introduced as an alternative to the Fontan procedure, specifically for selected patients with borderline hypoplastic right ventricle (HRV), aiming for a more physiological circulation. Despite these efforts, the benefit of 1.5VR over Fontan circulation comparison on clinical and functional outcomes remains unclear. The aim of this study was to investigate and compare young patients with HRV after 1.5VR with those with functional single right or left ventricles (FSRV or FSLV) after Fontan palliation over a 10-year follow-up period. METHODS AND RESULTS: In this retrospective observational study, serial cardiopulmonary exercise tests (CPETs) performed in patients with 1.5VR and Fontan circulation between September 2002 and March 2024 have been analysed. Only patients with at least 10 years of follow-up were considered. A total of 43 patients were included (age at baseline 8.6 ± 2.6 years): 21 with FSLV, 12 with FSRV, and 10 with 1.5VR. No differences in cardiorespiratory fitness and efficiency were shown at the first CPET assessment among the three groups. At 10-year follow-up, 1.5VR had higher cardiorespiratory fitness and efficiency compared to FSLV and FSRV patients. CONCLUSION: These findings suggest that the 1.5VR may provide superior long-term functional outcomes than the Fontan procedure in patients with borderline HRV. Further studies are needed to evaluate the impact on hard clinical endpoints.

Exercise Capacity and Cardiorespiratory Fitness in Children with Congenital Heart Diseases: A Proposal for an Adapted NYHA Classification.

OBJECTIVE: To propose and evaluate an adapted NYHA classification for children with congenital heart disease (CHD) as a feasible clinical tool for classifying patients' fitness, cardiorespiratory efficiency and functional limitations during their ordinary daily activities, which are also characterized by vigorous and competitive physical exercise among peers. METHODS: This cross-sectional investigation analyzed 332 patients (13.1 ± 3.01 y/o) who underwent surgical repair of CHD and performed Cardiopulmonary Exercise Testing (CPET). Patients were divided into NYHA class I, IIA and IIB by specific questioning regarding functional limitation and performance compared to peers and at strenuous intensity. Class IIA was characterized by slight exercise limitation only for strenuous/competitive activities, whereas IIB for already ordinary physical activities. These NYHA classes were compared with maximal CPET on treadmill. RESULTS: Patients' exercise capacity (exercise time, METs), aerobic capacity (VO2peak) and chronotropic response were found progressively impaired when NYHA class I was compared with IIA and IIB. Indeed, ventilatory-perfusion mismatch (PETCO2, VE/VCO2) significantly worsened from NYHA class I to IIA, while no difference was found between IIA and IIB. CONCLUSION: This adapted NYHA-CHD classification could allow regular functional evaluations and accurate assessments by clinicians, leading to facilitated clinical management and timely medical interventions.

[Physical activity in patients with repaired and unrepaired congenital heart diseases. Task Force for exercise prescription in patients with congenital heart disease of the Italian Society of Pediatric Cardiology and Congenital Heart Disease]. / L'attività fisica nei soggetti con cardiopatia congenita in storia naturale ed operata. Task Force sull'attività fisica nel cardiopatico congenito della Società Italiana di Cardiologia Pediatrica e delle Cardiopatie Congenite.

Regular physical activity is essential for physical health and mental wellbeing in children and teenagers. However, patients with congenital heart disease are often restricted from being physically active due to parental overprotection and lack of physical activity promotion or exercise prescription from their physicians. A comprehensive medical evaluation is crucial for the development of personalized exercise programs for these patients. The aim of this review is to provide physicians with a practical guide on how to promote physical activity and prescribe exercise for patients with congenital heart disease with or without surgical correction.

Do Kawasaki disease patients without coronary artery abnormalities need a long-term follow-up? A myocardial single-photon emission computed tomography pilot study.

OBJECTIVE: To determine the frequency and risk factors for long-term myocardial perfusion scintigraphy abnormalities in patients with Kawasaki disease (KD). METHODS: A cohort of patients with KD at least 3 years after disease onset and with persistent coronary artery aneurysms (CAA) (group 1) or without CAA (group 2) underwent stress-rest myocardial single-photon emission computed tomography (SPECT). Clinical and laboratory parameters at disease onset were considered to assess their predictive value for the development of myocardial perfusion abnormalities. RESULTS: Forty patients, 20 in group 1 and 20 in group 2, entered the study. The two groups turned out to be comparable for demographic, clinical and laboratory characteristics. Five patients (12.5%), two in group 1 and three in group 2, had abnormal myocardial perfusion assessed by SPECT. Neither the presence of CAA nor the overall cardiac involvement at the disease onset significantly increased the risk for these abnormalities. CONCLUSION: Cardiac SPECT abnormalities are not unusual in KD and can be found in patients with or without CAA. If confirmed in a larger cohort of patients, these preliminary data indicate that careful long-term cardiac follow-up should be considered, regardless of the presence of CAA.

Psychometric Properties of the Physical Activity Questionnaire for Older Children in Italy: Testing the Validity among a General and Clinical Pediatric Population.

The purpose of this research was to assess an Italian version of the Physical Activity Questionnaire for Older Children (PAQ-C-It). Three separate studies were conducted, whereby testing general psychometric properties, construct validity, concurrent validity and the factor structure of the PAQ-C-It among general and clinical pediatric population. Study 1 (n = 1170) examined the psychometric properties, internal consistency, factor structure (exploratory factor analysis, EFA) and construct validity with enjoyment perception during physical activity. Study 2 (n = 59) reported on reliability, construct validity with enjoyment and BMI, and on cross-sectional concurrent validity with objectively measured MVPA (tri-axial accelerometry) over the span of seven consecutive days. Study 3 (n = 58) examined the PAQ-C-It reliability, construct validity with BMI and VO2max as the objective measurement among a population of children with congenital heart defects (CHD). In study 2 and 3, the factor structure of the PAQ-C-It was then re-examined with an EFA. The PAQ-C-It showed acceptable to good reliability (alpha .70 to .83). Results on construct validity showed moderate but significant association with enjoyment perception (r = .30 and .36), with BMI (r = -.30 and -.79 for CHD simple form), and with the VO2max (r = .55 for CHD simple form). Significant concurrent validity with the objectively measured MVPA was reported (rho = .30, p < .05). Findings of the EFA suggested a two-factor structure for the PAQ-C-It, with items 2, 3, and 4 contributing little to the total score. This study supports the PAQ-C-It as an appropriate instrument to assess the MVPA levels of Italian children, including children with simple forms of CHD. Support is given to the possible instrument effectiveness on a large international perspective in order to level out data gathering across the globe.

Effects of aerobic exercise training in children after the Fontan operation.

It was demonstrated that patients who have undergone the Fontan operation can safely undertake exercise training and that this results in an improvement in aerobic capacity. These findings suggest that aerobic training could be useful in the long-term management of these patients to optimize their cardiovascular fitness for more active lives.

Leptin reduction after endurance races differing in duration and energy expenditure.

Serum leptin concentrations are reduced in the presence of a negative energy balance. It has been demonstrated, however, that strenuous and prolonged exercise, which induces a marked negative energy balance, is not always followed by a reduction in serum leptin levels. We therefore analysed serum leptin concentrations before and after three endurance races, which differed in duration and energy expenditure (EE), with the aim of clarifying the relationship between the level of EE and the reduction in leptin levels. Forty-five males participated in one of three competitive endurance races, a half-marathon run [21.097 km, estimated EE 1,400 kcal (5,852 kJ)], a ski-alpinism race [about 45 km, estimated EE 5,000 kcal (20,900 kJ)], and an ultramarathon race [100 km, estimated EE 7,000 kcal (29,269 kJ)]. Blood samples for analysis of serum leptin, and plasma free fatty acids (FFA) were collected before and after the races. Pre-race leptin values were significantly correlated with both body mass index and body fat mass ( r=0.672 and r=0.699, respectively; P<0.0001). After exercise, serum leptin levels decreased significantly in the ultramarathon [from 4.15 (0.63) microg/l to 1.01 (0.15) microg/l; P<0.001] and in the ski-alpinism race [from 1.10 (0.28) microg/l to 0.62 (0.15) microg/l; P<0.01], but not in the half-marathon [from 1.38 (0.40) microg/l to 1.20 (0.36) microg/l]. Plasma FFA were found to have significantly increased in all three of the races, showing a negative correlation with the percent reduction in leptin ( r=0.369, P<0.02). Our data indicate that only a prolonged endurance exercise involving a high EE can induce a marked reduction in circulating serum leptin levels.

Decreased serum leptin levels during prolonged high altitude exposure.

Circulating leptin concentrations are shown to be influenced not only by hormones, but also by body weight and energy balance. High altitude (HA) exposure induces a daily negative energy balance and stress hormone activation. The aim of our study was to evaluate leptin concentration during both acute and prolonged HA exposure and its correlations with some metabolic and hormonal parameters. Twelve males were studied during a stay at HA (15-20 days at 5,050 m). Blood samples for serum leptin, plasma insulin and 24-h urinary epinephrine (E) and norepinephrine (NE) were collected at sea level (SL), at the arrival at HA (A) and after 12-16 days (C) of stay. Symptoms of Acute Mountain Sickness (AMS) were evaluated using the Lake Louise score and the results showed there was no relationship with leptin concentrations. During the stay, both body mass index and leptin levels significantly decreased in both groups [leptin from 1.88 (1.12) to 1.21 (1.04) ng/ml, P<0.008, in A; and to 1.06 (0.74) ng/ml, P<0.003, in C]. Acute HA exposure induced a clear-cut significant increase of NE ( P<0.001 in A, P<0.003 in C) while E and insulin levels were unchanged in both phases. Moreover, a significant correlation between leptin and NE absolute values, and leptin and insulin variations was found ( r 0.359, P<0.034 and r=0.560, P<0.007, respectively). Exposure to HA induces a decrease in fasting serum leptin concentrations in men. These changes are not linked to symptoms of AMS but to hormonal and energy balance variations, suggesting that leptin is involved in the endocrine and metabolic adaptations occurring during HA exposure.