Early hybrid cardiac rehabilitation in congenital heart disease: the QUALIREHAB trial.

BACKGROUND AND AIMS: Cardiopulmonary fitness in congenital heart disease (CHD) decreases faster than in the general population resulting in impaired health-related quality of life (HRQoL). As the standard of care seems insufficient to encourage and maintain fitness, an early hybrid cardiac rehabilitation programme could improve HRQoL in CHD. METHODS: The QUALIREHAB multicentre, randomized, controlled trial evaluated and implemented a 12-week centre- and home-based hybrid cardiac rehabilitation programme, including multidisciplinary care and physical activity sessions. Adolescent and young adult CHD patients with impaired cardiopulmonary fitness were randomly assigned to either the intervention (i.e. cardiac rehabilitation) or the standard of care. The primary outcome was the change in HRQoL from baseline to 12-month follow-up in an intention-to-treat analysis. The secondary outcomes were the change in cardiovascular parameters, cardiopulmonary fitness, and mental health. RESULTS: The expected number of 142 patients was enroled in the study (mean age 17.4 ± 3.4 years, 52% female). Patients assigned to the intervention had a significant positive change in HRQoL total score [mean difference 3.8; 95% confidence interval (CI) 0.2; 7.3; P = .038; effect size 0.34], body mass index [mean difference -0.7 kg/m2 (95% CI -1.3; -0.1); P = .022; effect size 0.41], level of physical activity [mean difference 2.5 (95% CI 0.1; 5); P = .044; effect size 0.39], and disease knowledge [mean difference 2.7 (95% CI 0.8; 4.6); P = .007; effect size 0.51]. The per-protocol analysis confirmed these results with a higher magnitude of differences. Acceptability, safety, and short-time effect of the intervention were good to excellent. CONCLUSIONS: This early hybrid cardiac rehabilitation programme improved HRQoL, body mass index, physical activity, and disease knowledge, in youth with CHD, opening up the possibility for the QUALIREHAB programme to be rolled out to the adult population of CHD and non-congenital cardiac disease.

Impact of cardiac surgical timing on the neurodevelopmental outcomes of newborns with Complex congenital heart disease (CHD).

Background: More than half of infants with complex congenital heart disease (CHD) will have a neurodevelopmental disorder of multifactorial causes. The preoperative period represents a time-window during which neonates with complex CHD are in a state of hypoxia and hemodynamic instability, which fosters the emergence of brain injuries and, thus, affects early brain networks and neurodevelopmental outcomes. Currently, there is no consensus regarding the optimal age for cardiac surgery in terms of neurodevelopmental outcomes, and its definition is a real challenge. Our aim is to determine the relationship between cardiac surgical timing and long-term neurodevelopmental outcomes for various types of complex CHD. Methods: We hypothesize that earlier surgical timing could represent a neuroprotective strategy that reduces perioperative white matter injuries (WMIs) and postoperative morbidity, leading to improved neurodevelopmental outcomes in infants with complex CHD. Firstly, our prospective study will allow us to determine the correlation between age at the time of surgery (days of life) and neurodevelopmental outcomes at 24 months. We will then analyze the correlation between age at surgery and (i) the incidence of WMIs (through pre- and postoperative MRIs), (ii) postoperative morbidity, and (iii) the duration of the hospital stay. Implications and Dissemination: This research protocol was registered in the Clinical Trial Registry (National Clinical Trial: NCT04733378). This project aims to help launch the first Neurocardiac Investigation Clinic in Marseille - AP-HM - to propose an overall personalized monitoring and treatment program for patients operated on for complex CHD.

How should we determine normal echocardiographic right ventricle function reference values in pediatrics?

Several models have been used to establish pediatric Z-scores for echocardiographic right ventricle (RV) variables. This study aimed to determine the most appropriate mathematical model for RV function reference values in pediatrics. Prospective cross-sectional study among 314 healthy children ages 2 days to 18 years (46% female, 88 infants, 26 neonates, 226 children). RV parameters (S', E', A' waves, TEI index, TAPSE) were modelized with four explanatory variables: age, height, weight and body surface area. Four mathematical models were applied: a linear model, a polynomial model of degree 2, a linear regression model by spline with free knot, and a polynomial regression model of degree 2 by spline with a free knot. The choice of the best method used the adjusted coefficient of determination (aR2). The modelization of RV variables did not follow a linear model. A single explanatory variable could not determine all Z-scores, as specific and independent variables were required for each parameter. The quadratic spline model best adjusted the modelization of RV variables. The S' wave was best modelized by the spline model with the weight (knot at 6.86 kg). The E' wave was best modelized by the spline model with the age (knot at 0.29 years). The A' wave was best modelized by the model with the height (knot at 81 cm). The TAPSE was best modelized by the spline model with the weight (knot at 9.04 kg). The spline regression models best applies to echocardiographic RV reference values in pediatrics.

Infective endocarditis after device closure of atrial septal defects: Case report and review of the literature.

We report a case of late infective endocarditis in an 8-year-old boy 3 years after transcatheter closure of an atrial septal defect with the Amplatzer Atrial Septal Occluder Device. Echocardiography showed a very thick pannus lining the left atrial disc of the prosthesis, with some mobile elements. MRI showed several cerebral microemboli. The patient had dental caries and blood cultures found Staphylococcus aureus. Patient was treated with gentamicin and oxacillin before surgical removal of the prosthesis. Twenty-one cases of infective endocarditis after atrial defect device closure have been reported in the literature (13 ostium secundum ASD and 8 patent foramen ovale). Seven pediatric cases were reported. S. aureus was the most frequent bacterium. Antibiotics were administered in all cases and most patients (n = 18) underwent surgical removal of the device. Incomplete endothelialization of the prosthesis was a suggested mechanism. Two patients died after surgery. © 2016 Wiley Periodicals, Inc.

Long-Term Results of Balloon Valvuloplasty as Primary Treatment for Congenital Aortic Valve Stenosis: a 20-Year Review.

In the presence of new surgical techniques, the treatment of congenital valvular aortic stenosis is under debate. We reviewed the results and late outcomes of all 93 patients aged 1 day to 18 years, treated with balloon valvuloplasty (BAV) as first-line therapy for congenital aortic valve stenosis in our center from January 1991 to May 2012. Mean age at procedure time was 2.4 years; 37 patients underwent BAV at age ≤30 days (neonates), 29 patients at age ≥1 month and <1 year (infants), and 27 patients were older than 1 year (children). The invasive BAV peak-to-peak aortic valve gradient (mean 59 ± 22 mmHg) was immediately reduced (mean 24 ± 12 mmHg). The observed diminution of gradient was similar for each age group. Four patients had significant post-BAV AI. Mean follow-up after BAV was 11.4 ± 7 years. The last echo peak aortic gradient was 37 ± 18 mmHg and mean gradient was 23 ± 10 mmHg, and two patients had significant AI. Actuarial survival for the whole cohort was 88.2 and 72.9 % for the neonates. All infants, except one, and all children survived. Sixty-six percent of patients were free from surgery, and 58 % were free from any reintervention, with no difference according to age. Freedom from surgery after BAV at 5, 10, and 20 years, respectively, was 82, 72, and 66 %. Our study confirms that BAV as primary treatment for congenital AS is an efficient and low-risk procedure in infants and children. In neonates, the prognosis is more severe and clearly related to "borderline LV."