Efficacy of a Transition Program in Adolescents and Young Adults With Congenital Heart Disease: The TRANSITION-CHD Randomized Controlled Trial.

PURPOSE: Currently, nearly 90% of patients with congenital heart disease (CHD) reach adulthood in relatively good health. Structured transition programs have emerged to support adolescents and young adults in transitioning to adult care structures, improve their autonomy, and limit healthcare ruptures. The TRANSITION-CHD randomized controlled trial aimed to assess the impact of a transition program on health-related quality of life (HRQoL) in adolescents and young adults with CHD. METHODS: From January 2017 to February 2020, 200 subjects with a CHD, aged 13-25 years, were enrolled in a prospective, controlled, multicenter study and randomized in two balanced groups (transition program vs. standard of care). The primary outcome was the change in PedsQL self-reported HRQoL score between baseline and 12-month follow-up, using an intention-to-treat analysis. The secondary outcomes were the change in disease knowledge, physical health (cardiopulmonary fitness, physical activity), and mental health (anxiety, depression). RESULTS: The change in HRQoL differed significantly between the transition group and the control group (mean difference = 3.03, 95% confidence interval (CI) = [0.08; 5.98]; p = .044; effect size = 0.30), in favor of the intervention group. A significant increase was also observed in the self-reported psychosocial HRQoL (mean difference = 3.33, 95% CI = [0.01; 6.64]; p = .049; effect size = 0.29), in the proxy-reported physical HRQoL (mean difference = 9.18, 95% CI = [1.86; 16.51]; p = .015; effect size = 0.53), and in disease knowledge (mean difference = 3.13, 95% CI = [1.54; 4.72]; p < .001; effect size = 0.64). DISCUSSION: The TRANSITION-CHD program improved HRQoL and disease knowledge in adolescents and young adults with CHD, supporting the generalization and systematization of similar preventive interventions in pediatric and congenital cardiology.

Comparative analysis of left ventricle function and deformation imaging in short and long axis plane in cardiac magnetic resonance imaging.

Background: Advancements in cardiac imaging have revolutionized our understanding of ventricular contraction. While ejection fraction (EF) is still the gold standard parameter to assess left ventricle (LV) function, strain imaging (SI) has provided valuable insights into ventricular mechanics. The lack of an integrative method including SI parameters in a single, validated formula may limit its use. Our aim was to compare different methods for evaluating global circumferential strain (GCS) and their relationship with global longitudinal strain (GLS) and EF in CMR and how the different evaluations fit in the theoretical relationship between EF and global strain. Methods: Retrospective monocenter study. Inclusion of every patient who underwent a CMR during a 15 months period with various clinical indication (congenital heart defect, myocarditis, cardiomyopathy). A minimum of three LV long-axis planes and a stack of short-axis slices covering the LV using classical steady-state free precession cine sequences. A single assessment of GLS on long axis (LAX) slices and a double assessment of GCS and EF with both short axis (SAX) and LAX slices were made by a single experienced CMR investigator. Results: GCS-SAX and GCS-LAX were correlated (r = 0.77, P < 0.001) without being interchangeable with a high reproducibility for GCS, GLS and EF. EF calculated from LAX images showed an overestimation compared to EF derived from SAX images of 7%. The correlation between calculated EF and theoretical EF derived from SI was high (r = 0.88 with EF-SAX, 0.95 with EF-LAX). Data conclusion: This study highlights the need to integrate strain imaging techniques into clinical by incorporating strain parameters into EF calculations, because it gives a deeper understanding of cardiac mechanics.

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. Patients with an SV physiology were retrospectively screened from 2011 and 2021 in a single-centre observational study. We evaluated (1) the correlation between baseline CMR and CPET parameters, (2) the association between baseline CMR results and change in peak oxygen uptake (peak VO2), and (3) the cut-off values of end-diastolic and end-systolic volume index in patients with an impaired cardiopulmonary fitness (low peak VO2 and/or high VE/VCO2 slope). 32 patients were included in the study. End-systolic volume index (r = 0.37, p = 0.03), end-diastolic volume index (r = 0.45, p = 0.01), and cardiac index (r = 0.46, p = 0.01) correlated with the VE/VCO2 slope. End-systolic ventricular volume (r = - 0.39, p = 0.01), end-diastolic ventricular volume (r = - 0.38, p = 0.01), and cardiac output (r = - 0.45, p < 0.01) inversely correlated with the peak VO2. In multivariate analysis, the cardiac index obtained from baseline CMR was inversely associated with the change in peak VO2 (p < 0.01). An end-diastolic volume index > 101 ml/m2 and an end-systolic volume index > 47 ml/m2 discriminated patients with impaired cardiopulmonary fitness. CMR parameters correlate with cardiopulmonary fitness in patients with SV and can therefore be useful for follow-up and therapeutic management of these patients.

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.

Use of T1 mapping in cardiac MRI for the follow-up of Fabry disease in a pediatric population.

Background: Fabry disease (FD) is a rare X-linked lysosomal disorder caused by pathogenic variants in the alpha-galactosidase-A gene (GLA). Life threatening complications in adulthood include chronic kidney failure, strokes and the cardiac involvement which is the leading cause of mortality. Usually, it presents with hypertrophic cardiomyopathy, together with arrhythmia and conduction abnormalities. An early indicator is decreased T1 value on cardiac magnetic resonance (CMR). Enzyme replacement therapy (ERT) is effective on some extra-cardiac symptoms but its effect on cardiac lesions depends on the level of initial myocardial lesions. CMR is routinely used to monitor cardiac involvement in FD due to its capacity for tissular characterization. However, there is a lack of data on the pediatric population to understand how to integrate CMR into early therapeutic decisions. Method: Monocentric longitudinal study carried out at Montpellier University Hospital from 2016 to 2022. All pediatric patients with FD were evaluated over time with clinical, biological, and cardiac imaging (CMR, echocardiography). Results: Out of the six patients included, (3 males), five were treated with ERT during the study. Low T1 values were observed in 4 patients. The normalization of T1 values was observed after 4 years of ERT in 3 patients. Conclusion: Due to the lack of strong clinical and biological markers of FD in pediatric patients, initiation and follow-up of ERT efficacy remain challenging. CMR with T1-mapping, a noninvasive method, could play a role in the evaluation of early cardiac impairment in young patients at diagnosis and during follow-up with or without ERT.

Multimodal imaging for cardiac follow-up and management of giant coronary aneurysm related to Kawasaki disease in childhood: a case report.

Background: Kawasaki disease (KD) is a rare paediatric condition that can lead to giant coronary aneurysms. Follow-up of such complex coronary lesions remains a challenge, and their management is difficult to standardize. Case summary: Our present case concerns a 17-year-old boy who suffered a giant aneurysm of the left coronary artery, complicated by an asymptomatic stenosis. During regular follow-up, his annual cardiopulmonary exercise test revealed signs of ischaemia (ST depression and premature ventricular complexes). After several further stress tests with inconsistent results, he underwent invasive coronary angiography that revealed significant stenosis with a positive fractional flow reserve (FFR). Discussion: We discuss the challenges of diagnosing and managing coronary artery stenosis in paediatric patients with KD, particularly in cases with calcified and thrombosed lesions. A multimodal approach is crucial, including non-invasive imaging, and coronary angiography with optical coherence tomography and FFR. The evaluation of the lesion and its follow-up is an important factor in anticipating the best therapeutic choice for each patient.

Cardiorespiratory fitness, muscle fitness, and physical activity in children with long QT syndrome: A prospective controlled study.

Background: In children with congenital long QT syndrome (LQTS), the risk of arrhythmic events during exercise commonly makes it difficult to balance exercise restrictions versus promotion of physical activity. Nevertheless, in children with LQTS, cardiorespiratory fitness, muscle fitness, and physical activity, have been scarcely explored. Materials and methods: In this prospective, controlled, cross-sectional study, 20 children with LQTS (12.7 ± 3.7 years old) and 20 healthy controls (11.9 ± 2.4 years old) were enrolled. All participants underwent a cardiopulmonary exercise test, a muscular architecture ultrasound assessment, (cross-sectional area on right rectus femoris and pennation angle), a handgrip muscular strength evaluation, and a standing long broad jump test. The level of physical activity was determined using with a waist-worn tri-axial accelerometer (Actigraph GT3X). Results: Peak oxygen uptake (VO2peak) and ventilatory anaerobic threshold (VAT) were lower in children with LQTS than in healthy controls (33.9 ± 6.2 mL/Kg/min vs. 40.1 ± 6.6 mL/Kg/min, P = 0.010; 23.8 ± 5.1 mL/Kg/min vs. 28.8 ± 5.5 mL/Kg/min, P = 0.007, respectively). Children with LQTS had lower standing long broad jump distance (119.5 ± 33.2 cm vs. 147.3 ± 36.1 cm, P = 0.02) and pennation angle (12.2 ± 2.4° vs. 14.3 ± 2.8°, P = 0.02). No differences in terms of moderate-to-vigorous physical activity were observed (36.9 ± 12.9 min/day vs. 41.5 ± 18.7 min/day, P = 0.66), but nearly all children were below the WHO guidelines. Conclusion: Despite similar physical activity level, cardiorespiratory fitness and muscle fitness in children with LQTS were lower than in healthy controls. The origin of this limitation seemed to be multifactorial, involving beta-blocker induced chronotropic limitation, physical and muscle deconditioning. Cardiovascular rehabilitation could be of interest in children with LQTS with significant physical limitation.

Health-related quality of life and physical activity in children with inherited cardiac arrhythmia or inherited cardiomyopathy: the prospective multicentre controlled QUALIMYORYTHM study rationale, design and methods.

BACKGROUND: Advances in paediatric cardiology have improved the prognosis of children with inherited cardiac disorders. However, health-related quality of life (QoL) and physical activity have been scarcely analysed in children with inherited cardiac arrhythmia or inherited cardiomyopathy. Moreover, current guidelines on the eligibility of young athletes with inherited cardiac disorders for sports participation mainly rely on expert opinions and remain controversial. METHODS: The QUALIMYORYTHM trial is a multicentre observational controlled study. The main objective is to compare the QoL of children aged 6 to 17 years old with inherited cardiac arrhythmia (long QT syndrome, Brugada syndrome, catecholaminergic polymorphic ventricular tachycardia, or arrhythmogenic right ventricular dysplasia), or inherited cardiomyopathy (hypertrophic, dilated, or restrictive cardiomyopathy), to that of age and gender-matched healthy subjects. The secondary objective is to assess their QoL according to the disease's clinical and genetic characteristics, the level of physical activity and motivation for sports, the exercise capacity, and the socio-demographic data. Participants will wear a fitness tracker (ActiGraph GT3X accelerometer) for 2 weeks. A total of 214 children are required to observe a significant difference of 7 ± 15 points in the PedsQL, with a power of 90% and an alpha risk of 5%. DISCUSSION: After focusing on the survival in children with inherited cardiac disorders, current research is expanding to patient-reported outcomes and secondary prevention. The QUALIMYORYTHM trial intends to improve the level of evidence for future guidelines on sports eligibility in this population. Trial registration ClinicalTrials.gov Identifier: NCT04712136, registered on January 15th, 2021 ( https://clinicaltrials.gov/ct2/show/NCT04712136 ).

Disseminated Aspergillosis Complicated With a Myocardial Abscess in an Immunodeficient Child.

Aspergillosis infection is a major complication of acquired immunosuppression, and is associated with poor prognosis in its invasive form. Cardiac dissemination of invasive aspergillosis is a rare and usually fatal consequence of systemic mycosis. We describe a pediatric case of myocardial aspergillosis abscess in a 12-year-old girl diagnosed with multimodality cardiac imaging approach. The patient underwent prolonged antimycotic treatment and bone marrow transplant, which improved her immunity, and, for the first time in the literature, resulted in a favorable outcome.

The Lugones Procedure for Surgical Repair of Scimitar Syndrome: Preserved Growth in a Young Infant.

Surgical repair of Scimitar syndrome is challenging, especially in small patients. Our images demonstrate that the pericardial tunnel technique is feasible even in low-weight patient and that it provides a good growth potential.

Impact of a transition education program on health-related quality of life in pediatric patients with congenital heart disease: study design for a randomised controlled trial.

BACKGROUND: Recent advances in the field of congenital heart disease (CHD) led to an improved prognosis of the patients and in consequence the growth of a new population: the grown up with congenital heart disease. Until recently, more than 50% of these patients were lost to follow up because of the lack of specialized structures. The critical moment is the transition between paediatric and adult unit. Therapeutic education is crucial to solve this issue by helping patients to become independent and responsible. The TRANSITION-CHD randomized trial aims to assess the impact of a transition education program on health-related quality of life (HRQoL) of adolescents and young adults with CHD. METHODS: Multicentre, randomised, controlled, parallel arm study in CHD patients aged from 13 to 25 years old. Patients will be randomised into 2 groups (education program vs. no intervention). The primary outcome is the change in self-reported HRQoL between baseline and 12-month follow-up. A total of 100 patients in each group is required to observe a significant increase of the overall HRQoL score of 7 ± 13.5 points (on 100) with a power of 80% and an alpha risk of 5%. The secondary outcomes are: clinical outcomes, cardiopulmonary exercise test parameters (peak VO2, VAT, VE/VCO2 slope), level of knowledge of the disease using the Leuven knowledge questionnaire for CHD, physical and psychological status. DISCUSSION: As the current research is opening on patient related outcomes, and as the level of proof in therapeutic education is still low, we sought to assess the efficacy of a therapeutic education program on HRQoL of CHD patients with a randomized trial. TRIAL REGISTRATION: This study was approved by the National Ethics Committee (South-Mediterranean IV 2016-A01681-50) and was registered on Clinicaltrials.gov (NCT03005626).

Health-related quality of life in children with congenital heart disease aged 5 to 7 years: a multicentre controlled cross-sectional study.

BACKGROUND: In the context of tremendous progress in congenital cardiology, more attention has been given to patient-related outcomes, especially in assessing health-related quality of life (HRQoL) of patients with congenital heart diseases (CHD). However, most studies have mainly focused on teenagers or adults and currently, few HRQoL controlled data is available in young children. This study aimed to evaluate HRQoL of children with CHD aged 5 to 7 y.o., in comparison with contemporary peers recruited in school, as well as the factors associated with HRQoL in this population. METHODS: This multicentre controlled prospective cross-sectional study included 124 children with a CHD (mean age = 6.0 ± 0.8 y, 45% female) during their outpatient visit and 125 controls (mean age = 6.2 ± 0.8 y, 54% female) recruited at school. A generic paediatric HRQoL instrument was used (PedsQL 4.0). RESULTS: Self-reported HRQoL in children with CHD was similar to controls, overall (73.5 ± 1.2 vs. 72.8 ± 1.2, P = 0.7, respectively), and for each dimension. Parents-reported HRQoL was significantly lower in the CHD group than in controls. HRQoL was predicted by the disease severity and by repeated invasive cardiac procedures (surgery or catheterization). CONCLUSION: HRQoL in young children with CHD aged 5 to 7 years old was good and similar to controls. This study contributed to the growing body of knowledge on HRQoL in congenital cardiology and emphasized the need for child and family support in the most complex CHD. Trial registration This study was approved by the institutional review board of Montpellier University Hospital (2019_IRB-MTP_02-19) on 22 February 2019 and was registered on ClinicalTrials.gov (NCT03931096) on 30 April 2019, https://clinicaltrials.gov/ct2/show/NCT03931096 .

Health-related quality of life correlates with time in therapeutic range in children on anticoagulants with International Normalised Ratio self-monitoring.

BACKGROUND: Managing oral anticoagulant therapy with vitamin K antagonists remains challenging in paediatric medicine. AIMS: This study aimed to assess the correlation between time in therapeutic range and quality of life in children participating in a non-selective International Normalised Ratio self-monitoring and vitamin K antagonist education programme. METHODS: Children aged from 2 to 18 years and receiving vitamin K antagonist therapy were eligible for this prospective multicentre study. Clinical and demographic data were collected. Health-related quality of life was assessed using the PedsQL™ 4.0 questionnaire. Correlations between quality of life scores and time in therapeutic range were measured. RESULTS: A total of 121 children were included in the study (mean age 9.6±4.9 years). Cardiac conditions were the predominant indication for vitamin K antagonists. The mean time in therapeutic range was 0.78±0.15 overall, and 0.76±0.24 over the 3-month period before quality of life assessment. The mean total quality of life score was 76.2±18 in self reports, 71.4±22 in mother reports and 73.5±19 in father reports. The time in therapeutic range correlated with the total quality of life scores in self reports (r=0.22; P=0.04), mother reports (r=0.23; P=0.02) and father reports (r=0.28; P=0.02). The time in therapeutic range predominantly correlated with school functioning in self reports (r=0.38; P=0.002) and mother reports (r=0.40; P<0.001), and with physical functioning in father reports (r=0.28; P=0.03). CONCLUSIONS: Time in therapeutic range correlated with quality of life in children participating in a non-selective International Normalised Ratio self-monitoring and vitamin K antagonist education programme. Regular assessment of quality of life in patient education programmes contributes towards understanding the concerns and needs of patients.

Use of Treprostinil in Pediatric Pulmonary Hypertension: Case Reports and Review of the Literature.

BACKGROUND: Add-on therapy with prostacyclin in pediatric refractory pulmonary hypertension (PH) poses a challenge, especially when considering continuous intravenous administration in younger children. A search for alternate routes of drug delivery has led to the clinical investigation of stable and long-acting prostacyclin analogues, such as subcutaneous treprostinil. We reported 2 pediatric cases of PH treated with subcutaneous treprostinil and reviewed the literature on treprostinil use in children. METHOD: The literature review used 3 electronic databases and a combination of terms (treprostinil, pediatric, PH, prostanoid, etc). We also searched for pediatric clinical trials on treprostinil registered on international clinical trial registries. RESULTS: The reported cases highlighted the multifactorial nature of PH in pediatrics: a female child with a giant omphalocele, and intracardiac and extracardiac shunts; and a male premature child with a congenital diaphragmatic hernia and long-term PH. The literature review identified 19 studies reporting treprostinil use in 421 children with various types of PH (groups 1 and 3). Subcutaneous treprostinil was the most administered formulation, at a mean dose of 40 ng/kg/min. Overall, 12 clinical trials on treprostinil for children with PH were registered on the clinical trial registries. Most authors concluded that subcutaneous treprostinil was effective, well tolerated, and represented an alternative to intravenous epoprostenol. CONCLUSIONS: Subcutaneous treprostinil may be a useful adjunct in the therapeutic algorithm for children with severe PH, refractory to oral drugs, and after a complete check-up for all PH etiologies.

Impact of Sophrology on cardiopulmonary fitness in teenagers and young adults with a congenital heart disease: The SOPHROCARE study rationale, design and methods.

BACKGROUND: Recent advances in the field of congenital heart disease (CHD) have significantly improved the overall prognosis. Now more attention is being given to health-related quality of life (HRQoL) and promotion of physical activity. Non-invasive relaxation therapy may be effective in cardiac patients concerned with exercise-induced dyspnoea. The SOPHROCARE randomised trial aims to assess the impact of Caycedian Sophrology on cardiopulmonary fitness in adolescents and young adults with CHD. METHODS: The SOPHROCARE trial is a nationwide, multicentre, randomised, controlled study in CHD patients aged from 13 to 25 years old. Patients will be randomised into 2 groups (8 Sophrology group sessions vs. no intervention). The primary outcome is the change in percent predicted maximum oxygen uptake (VO2max) between baseline and 12-month follow-up. A total of 94 patients in each group is required to observe a significant increase of 10% in VO2max with a power of 80% and an alpha risk of 5%. The secondary outcomes are: clinical outcomes, cardiopulmonary exercise test parameters (VE/VCO2 slope, ventilatory anaerobic threshold, oxygen pulse, respiratory response to hypercapnia), health-related quality of life score (PedsQL), physical and psychological status. CONCLUSION: After focusing on the survival in CHD, current research is opening on secondary prevention and patient-related outcomes. We sought to assess in the SOPHROCARE trial, if a Sophrology program, could improve exercise capacity and quality of life in youth with CHD. TRIAL REGISTRATION: Clinicaltrials.gov (NCT03999320).

Acquired systemic-to-pulmonary shunts in a 6-month-old child: case report and review of the literature.

The incidence of paediatric venous thromboembolism has steadily increased in the past decade, by nearly 10% per year. Deep venous thrombosis may remain completely asymptomatic during the acute phase and symptoms may occur later, due to complications. We related the case of a 9-month-old child with increasing cyanosis. A computed tomography (CT) angiography showed a thrombosis of the superior vena cava (SVC) with the development of collateral flow from the systemic to the pulmonary veins. Transcatheter shunt occlusion after SVC recanalization was successfully performed. We discussed the characteristics of these cases and the consequence on our practice in term of treatment (anticoagulation, transcatheter, intervention) and screening.

Factors influencing the participation of adolescents and young adults with a congenital heart disease in a transition education program: A prospective multicentre controlled study.

OBJECTIVE: Transition education programs dedicated to adolescents and young adults with congenital heart disease (CHD) aim to facilitate transfer to adult cardiology and bring more autonomy to teenagers. This prospective controlled multicentre study analysed the factors influencing the participation in a transition education program. METHODS: CHD patients aged 13-25 y were offered to participate in the transition program. A multiple linear regression identified the explanatory factors for participation in the program. RESULTS: A total of 123 patients (mean age 19.6 ±â€¯3.4 y) were included in the study, with 57 participants and 66 non-participants. Both groups showed similar socio-demographic and quality of life characteristics, low level of physical activity with muscular deconditioning and high exposure to risk behaviours (71% patients with ≥1 risk factor). Patients with complex CHD (OR = 4.1, P = 0.03), poor disease knowledge (OR = 0.3, P = 0.02), risk behaviours (body piercing, OR = 5.53, P = 0.01; alcohol, OR = 3.12, P = 0.06), and aged <20 y (OR = 0.29, P = 0.03), were more likely to join the program. CONCLUSION: Many risk factors influencing the participation of adolescents and young adults with CHD in transition education programs are controllable. PRACTICE IMPLICATION: Further randomized studies are necessary to evaluate the impact of transition education program on quality of life, successful transfer to adult centre and, ultimately, prognosis.

Parental anxiety before invasive cardiac procedure in children with congenital heart disease: Contributing factors and consequences.

OBJECTIVE: Medical information provided to parents of a child with a congenital heart disease can induce major stress. Visual analog scales have been validated to assess anxiety in the adult population. The aim of this study was to analyze parental anxiety using a visual analog scale and to explore the influencing factors. DESIGN: This prospective cross-sectional study. SETTING: Tertiary care regional referral center for congenital heart disease of Marseille-La Timone university hospital. PATIENTS: Parents of children with a congenital heart disease, as defined by the ACC-CHD classification, referred for cardiac surgery or interventional cardiac catheterization, were offered to participate. INTERVENTION AND OUTCOME MEASURE: The parental level of anxiety was assessed using a visual analog scale (0-10) before intervention and after complete information given by the cardiologist, the surgeon or the anesthetists. RESULTS: Seventy-three children [7 days-13 years], represented by 49 fathers and 71 mothers, were included in the study. A total of 42 children required cardiac surgery and 31 children underwent interventional cardiac catheterization. The mean score of maternal anxiety was significantly higher than the paternal anxiety (8.2 vs 6.3, P < .01). A high level of maternal anxiety (visual analog scale > 8) was associated with paternal anxiety (P = .02), the child's comorbidity (P = .03), the distance between home and referral center (P = .04), and the level of risk adjustment for congenital heart surgery (P = .01). In multivariate analysis, maternal anxiety was associated with paternal anxiety (OR = 4.9; 95% confidence interval [1.1-19.2]), and the level of risk adjustment for congenital heart surgery (OR = 11.4; 95% confidence interval [1.2-116.2]). No significant association was found between parental anxiety and prenatal diagnosis. CONCLUSION: This study highlighted several factors associated with the parental anxiety. Identifying the parents at risk of high stress can be useful to set up psychological support during hospitalization.