Predictive factors of surgical repair technique in infants with coarctation of the aorta.

Objectives: Coarctation of the aorta (CoA) is the most common undiagnosed congenital heart disease during prenatal screening and its overall prognosis relies on the quality of the surgical repair. This study aimed to identify the prenatal and postnatal factors associated with the type of surgical technique repair in infant CoA.

Methods: Multicentre, retrospective, observational study in 680 infants (68% males) aged <1 year who underwent CoA surgical repair in 4 CHD surgical centres in France over 11 years.

Health-Reported Quality of Life in Patients with A Systemic Right Ventricle: The QUALISYSTEMIC Study.

Background: The systemic right ventricle (SRV) represents one of the most complex congenital heart diseases (CHD), involving two main anatomical presentations: D-looped transposition of the great arteries and congenitally corrected transposition of the great arteries. Recent therapeutic advances have attempted to improve prognosis in adults with an SRV, but the impact on health-related quality of life (HRQoL) has not been clearly established.

Methods: Adult patients aged >18 years with an SRV were prospectively enrolled in a multicentre cross-sectional study with recruitment across 13 CHD centres.

Perceived health, psychological distress and quality of life in 8415 adults with congenital heart disease from 32 countries.

Background: The global prevalence of congenital heart disease (CHD) is increasing. Research on patient-reported outcomes (PROs) predominantly originates from high-income countries, resulting in an incomplete understanding of the true global burden of CHD from the patient perspective. Therefore, we described perceived health, psychological distress and quality of life (QoL) in a large sample of adults with CHD from the globe and explored the relationship between PROs and the income level of the countries.

Which classification best predicts functional status in children with CHD?

Background: Medical advances in the management of CHD have shifted the focus from childhood mortality to life-long morbidity; therefore, clinical research in paediatric cardiology tends to replace mortality outcomes with functional and patient-reported outcomes. Despite these advances, the stratification of disease severity using a simple and reproducible CHD classification has not been established. The aim was to determine which classification best predicts functional status in children with CHD, in terms of cardiopulmonary fitness.

Clinical value of calibrated abdominal compression plus transthoracic echocardiography to predict fluid responsiveness in critically ill infants: a diagnostic accuracy study.

Background: Predicting fluid responsiveness is challenging in infants. It is however crucial to avoid unnecessary volume expansion, which can lead to fluid overload. We tested the hypothesis that the stroke volume changes induced by a calibrated abdominal compression (ΔSV-AC) could predict fluid responsiveness in infants without cardiac disease.

Guidelines for anaesthesia of adults with congenital heart disease in non-cardiac surgery.

Objective: To provide guidelines for the anesthetic management of adults with congenital heart disease for non-cardiac surgery.

Design: A consensus committee of 16 experts was convened. A formal conflict-of-interest (COI) policy was developed at the beginning of the process and enforced throughout.

Optimal brain perfusion pressure derived from the continuous monitoring of cerebral autoregulation status during neonatal heart surgery under cardiopulmonary bypass in relation to brain injury: An observational study.

Background: Understanding cerebral blood flow regulation and later optimizing brain perfusion is part of neuroprotection during cardiopulmonary bypass (CPB) in neonates.

Methods: A total of 38 neonates undergoing CPB were monitored using near-infrared spectrometry and mean arterial pressure (MAP). Cerebral autoregulation (CAR) was assessed through the continuous measurement of the Cerebral Oxygenation Index (COx), and CAR-derived metrics were determined by plotting averaged COx values by MAP: Optimal MAP (MAPopt), lower limit of CAR (LLA), upper limit of CAR (ULA).

Cardiopulmonary Fitness and Physical Activity Among Children and Adolescents With Inherited Cardiac Disease.

Importance: Historical restrictions on children with inherited cardiac arrhythmia or cardiomyopathy have been implemented to mitigate the potential risk of sudden death, but these limitations can be detrimental to overall health and cardiopulmonary fitness.

Objectives: To evaluate cardiopulmonary fitness and physical activity among children with inherited cardiac disease and identify the factors associated with maximum oxygen uptake (V̇o2max) in this population.

Design, Setting, And Participants: This cross-sectional, multicenter, prospective controlled study was conducted in 7 tertiary care expert centers for inherited cardiac disease in France from February 1, 2021, to June 20, 2023, with a 2-week follow-up.

Cardiac rehabilitation in children and adolescents with long QT syndrome: the RYTHMO'FIT pilot study.

Background: To assess the feasibility, acceptability, safety, and short-term benefits of a tailored cardiac rehabilitation program for children and adolescents with long QT syndrome (LQTS).

Methods: Eight participants, aged between 6 and 18, with a positive LQTS genotype and impaired cardiorespiratory fitness, were enrolled in a 12-week centre-based cardiac rehabilitation program. The program included supervised exercise training group sessions (aerobic, resistance, and outdoor activities) and patient education workshops.

Implications of right aortic arch prenatal diagnosis: the multicentric nationwide ARCADE cohort.

Objectives: This study aims to describe the various presentations of the prenatally diagnosed isolated right aortic arch (RAA), that is, without associated congenital heart defect and to evaluate the impact of prenatal diagnosis of isolated RAA in terms of postnatal outcome.

Method: In this multicentric retrospective study, from 2010 to 2019, all live births with a prenatal ultrasound diagnosis of isolated RAA were included, with a 1-year postnatal follow-up. The concordance between the different diagnostic steps (prenatal ultrasound, postnatal ultrasound and postnatal CT scan) was evaluated using Gwet's AC1 coefficient.

Health-related quality of life in children and adolescents with Marfan syndrome or related disorders: a controlled cross-sectional study.

Background: This cross-sectional controlled study aims to assess health-related quality of life (HRQoL) of children and adolescents with a molecular diagnosis of Marfan syndrome (MFS) or related disorders and to evaluate the factors associated with HRQoL in this population. Sixty-three children with MFS and 124 age- and sex-matched healthy children were recruited. HRQoL was assessed using the Pediatric Quality of Life Inventory (PedsQL™) generic questionnaire.

Cardiac magnetic resonance ventricular parameters correlate with cardiopulmonary fitness in patients with functional single ventricle.

Owing to advances in medical and surgical fields, patients with single ventricle (SV) have a greatly improved life expectancy. However, progressive functional deterioration is observed over time, with a decrease in cardiopulmonary fitness. This study aimed to identify, in patients with SV, the association between cardiac magnetic resonance imaging (CMR) parameters and change in cardiopulmonary fitness assessed by cardiopulmonary exercise test (CPET), and if certain thresholds could anticipate a decline in aerobic fitness.

Aerobic physical capacity and health-related quality of life in children with sickle cell disease.

Background: Aerobic fitness is a predictor of cardiovascular health which correlates with health-related quality of life in the general population. The aim is to evaluate the aerobic capacity by cardiopulmonary exercise test (CPET) in children with sickle cell disease in comparison with healthy matched controls.

Methods: Controlled cross-sectional study.

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.

Ryanodine receptor dysfunction causes senescence and fibrosis in Duchenne dilated cardiomyopathy.

Background: Duchenne muscular dystrophy (DMD) is an X-linked disorder characterized by progressive muscle weakness due to the absence of functional dystrophin. DMD patients also develop dilated cardiomyopathy (DCM). We have previously shown that DMD (mdx) mice and a canine DMD model (GRMD) exhibit abnormal intracellular calcium (Ca) cycling related to early-stage pathological remodelling of the ryanodine receptor intracellular calcium release channel (RyR2) on the sarcoplasmic reticulum (SR) contributing to age-dependent DCM.

Children With Cardiac Disease and Heat Exposure: Catastrophic Converging Consequences?

Unlabelled: The detrimental impact of extreme heat exposure on the health and well-being of children is widely acknowledged. The direct and indirect effects of climate change have led to an increased risk of certain cardiovascular events which may be particularly harmful to children who are born with, or develop, heart disease.

Purpose: To highlight the worrying paucity of investigative research aimed at differentiating how higher ambient temperatures further tax an already compromised cardiovascular system in children.

Generation of patient-specific induced pluripotent stem cell lines with Type 2 Long QT Syndrome and the KCNH2 c.379C > T pathogenic variant.

Type 2 Long QT Syndrome (LQT2) is a rare genetic heart rhythm disorder causing life-threatening arrhythmias. We derived induced pluripotent stem cell (iPSC) lines from two patients with LQT2, aged 18 and 6, both carrying a heterozygous missense mutation on the 3rd and 11th exons of KCNH2. The iPSC lines exhibited normal genomes, expressed pluripotent markers, and differentiated into trilineage embryonic layers.

Reference Values of Cardiopulmonary Exercise Test Parameters in the Contemporary Paediatric Population.

Background: The evaluation of health status by cardiopulmonary exercise test (CPET) has shown increasing interest in the paediatric population. Our group recently established reference Z-score values for paediatric cycle ergometer VO, applicable to normal and extreme weights, from a cohort of 1141 healthy children. There are currently no validated reference values for the other CPET parameters in the paediatric population.

Think out of the box: association of left congenital diaphragmatic hernia and abnormal origin of the right pulmonary artery : A train can hide another.

Background: We report the occurrence of a severe pulmonary hypertension (PH) in a neonate affected by a left congenital diaphragmatic hernia (CDH). PH in this patient was associated with an abnormal origin of the right pulmonary artery from the right brachiocephalic artery. This malformation, sometimes named hemitruncus arteriosus, has to the best of our knowledge never been reported in association with a CDH.

Use of new paediatric VO2max reference equations to evaluate aerobic fitness in overweight or obese children with congenital heart disease.

Aims: Overweight and obesity in children with congenital heart disease (CHD) represent an alarming cardiovascular risk. Promotion of physical activity and cardiac rehabilitation in this population requires assessing the level of aerobic fitness (VO2max) by a cardiopulmonary exercise test (CPET). Nevertheless, the interpretation of CPET in overweight/obese children with CHD remains challenging as VO2max is affected by both the cardiac condition and the body mass index (BMI).

Health-Related Quality of Life and Its Association With Outcomes in Adults With Congenital Heart Disease and Heart Failure: Insight From FRESH-ACHD Registry.

Background Quality of Life (QoL) is a prognostic factor in heart failure (HF) of patients with acquired cardiac disease. The aim of this study was to determine the predictive value of QoL on outcomes in adults with congenital heart disease (ACHD) and HF. Methods and Results Quality of life of 196 adults with congenital heart disease with clinical heart failure (HF) (mean age: 44.

Correlation between three-dimensional echocardiography and cardiopulmonary fitness in patients with univentricular heart: A cross-sectional multicentre prospective study.

Background: The prognosis of patients with a functional single ventricle has improved, with better cardiopulmonary fitness, health-related quality of life and survival. Conventional echocardiography remains the first-line technique in single ventricle follow-up. Three-dimensional (3D) echocardiography has shown recent value in congenital cardiology, but its ability to predict functional status in patients with a single ventricle remains unknown.