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Pediatric patients with congenital heart disease (CHD) often undergo low dose ionizing radiation (LDIR) from cardiac catheterization (CC) for the diagnosis and/or treatment of their disease. Although radiation doses from a single CC are usually low, less is known about the long-term radiation associated cancer risks. We aimed to assess the risk of lympho-hematopoietic malignancies in pediatric CHD patients diagnosed or treated with CC. A French cohort of 17,104 children free of cancer who had undergone a first CC from 01/01/2000 to 31/12/2013, before the age of 16 was set up. The follow-up started at the date of the first recorded CC until the exit date, i.e., the date of death, the date of first cancer diagnosis, the date of the 18th birthday, or the 31/12/2015, whichever occurred first. Poisson regression was used to estimate the LDIR associated cancer risk. The median follow-up was 5.9 years, with 110,335 person-years. There were 22,227 CC procedures, yielding an individual active bone marrow (ABM) mean cumulative dose of 3.0 milligray (mGy). Thirty-eight incident lympho-hematopoietic malignancies were observed. When adjusting for attained age, gender and predisposing factors to cancer status, no increased risk was observed for lympho-hematopoietic malignancies RR /mGy = 1.00 (95% CI: 0.88; 1.10). In summary, the risk of lympho-hematopoietic malignancies and lymphoma was not associated to LDIR in pediatric patients with CHD who undergo CC. Further epidemiological studies with greater statistical power are needed to improve the assessment of the dose-risk relationship., (© 2023. Springer Nature B.V.)

Background: Although patients with systemic right ventricle (SRV) represent a significant proportion of patients with congenital heart disease (CHD) implanted with cardiac resynchronization therapy (CRT), there are limited and conflicting data in this specific patient group., Objective: We aimed to analyze outcomes of patients with SRV implanted with a CRT device., Methods: Data were analyzed from an observational, retrospective, multicenter cohort study including all patients with CHD implanted with a CRT device from 6 French centers from 2004 to 2020. Response to CRT was defined as an increase in systemic ventricular ejection fraction of ≥10% and/or an improvement in New York Heart Association functional class by at least 1 grade., Results: A total of 85 patients with CHD were enrolled (mean age 39.8 ± 20.0 years; 55 [64.7%] males; 25 defibrillators [29.4%]), including 31 patients with SRV (36.5%) (mean age 43.9 ± 19.8 years; 16 [51.6%] males). The mean change in QRS duration after implantation was similar as compared with patients with systemic left ventricle (-46 ± 26 ms vs -35 ± 32 ms; P = .16). During a mean follow-up of 5.1 ± 3.5 years, late complications included 2 lead dysfunctions (6.5%), 3 CRT-related infections (9.7%), and 1 inappropriate implantable cardioverter-defibrillator shock (3.2%). The proportion of CRT responders at 6, 12, and 24 months were 82.6%, 80.0%, and 77.8% in patients with SRV vs 66.7%, 64.3%, and 69.6% in patients with systemic left ventricle (P = NS)., Conclusion: In this multicenter cohort, one-third of patients with CHD implanted with a CRT device had SRV. CRT in patients with SRV was associated with a high rate of responders, comparable to that of patients with systemic left ventricle., (Copyright © 2021 Heart Rhythm Society. Published by Elsevier Inc. All rights reserved.)

Background: Progress in the management of complex congenital heart disease (CHD) led to an improvement in survival rates of adults with a Fontan-like circulation. The objective of this study was to assess the subjective health status and quality of life of this population., Methods and Results: Patients aged more than 18 years at the time of the study, who underwent a Fontan-like procedure. Subjective health status was assessed by the SF-36 questionnaire and a linear analog scale was used to score patients' self-perception of their quality of life; cardiac and demographic parameters were collected., Results: Among 65 eligible patients, 60 (23 females; mean ± SD age: 25.7 ± 7.2 years) answered the SF-36 questionnaire and 46 of these were interviewed to evaluate their perceived quality of life. Among them, 20 (33.3%) were working full-time and 21 (35%) experienced arrhythmias. The physical SF-36 scores were lower in patients than in the general population (p ≤ 0.05). The New York Hear Association (NYHA) class and occupation were correlated with SF-36 scores of physical activity (respectively, p = 0.0001 and p = 0.025). SF-36 scores of psychological status were associated with the number of drugs and occupation (respectively, p = 0.0001 and p = 0.02). The mean ± SD quality of life score measured using a linear analog scale was 7.02 ± 1.6 and was linked to education and occupation (p ≤ 0.05) but not with cardiac parameters., Conclusion: Adult Fontan patients perceive an impaired physical health but report a good overall quality of life. Education and occupation impacts significantly on Fontan patients' quality of life.

Herein, we report the screening of a large panel of genes in a series of 80 fetuses with congenital heart defects (CHDs) and/or heterotaxy and no cytogenetic anomalies. There were 49 males (61%/39%), with a family history in 28 cases (35%) and no parental consanguinity in 77 cases (96%). All fetuses had complex CHD except one who had heterotaxy and midline anomalies while 52 cases (65%) had heterotaxy in addition to CHD. Altogether, 29 cases (36%) had extracardiac and extra-heterotaxy anomalies. A pathogenic variant was found in 10/80 (12.5%) cases with a higher percentage in the heterotaxy group (8/52 cases, 15%) compared with the non-heterotaxy group (2/28 cases, 7%), and in 3 cases with extracardiac and extra-heterotaxy anomalies (3/29, 10%). The inheritance was recessive in six genes (DNAI1, GDF1, MMP21, MYH6, NEK8, and ZIC3) and dominant in two genes (SHH and TAB2). A homozygous pathogenic variant was found in three cases including only one case with known consanguinity. In conclusion, after removing fetuses with cytogenetic anomalies, next-generation sequencing discovered a causal variant in 12.5% of fetal cases with CHD and/or heterotaxy. Genetic counseling for future pregnancies was greatly improved. Surprisingly, unexpected consanguinity accounts for 20% of cases with identified pathogenic variants., (© 2020 Wiley Periodicals LLC.)

The population of patients with congenital heart disease (CHD) is continuously increasing, and a significant proportion of these patients will experience arrhythmias because of the underlying congenital heart defect itself or as a consequence of interventional or surgical treatment. Arrhythmias are a leading cause of mortality, morbidity and impaired quality of life in adults with CHD. Arrhythmias may also occur in children with or without CHD. In light of the unique issues, challenges and considerations involved in managing arrhythmias in this growing, ageing and heterogeneous patient population and in children, it appears both timely and essential to critically appraise and synthesize optimal treatment strategies. The introduction of catheter ablation techniques has greatly improved the treatment of cardiac arrhythmias. However, catheter ablation in adults or children with CHD and in children without CHD is more technically demanding, potentially causing various complications, and thus requires a high level of expertise to maximize success rates and minimize complication rates. As French recommendations regarding required technical competence and equipment are lacking in this situation, the Working Group of Pacing and Electrophysiology of the French Society of Cardiology and the Affiliate Group of Paediatric and Adult Congenital Cardiology have decided to produce a common position paper compiled from expert opinions from cardiac electrophysiology and paediatric cardiology. The paper details the features of an interventional cardiac electrophysiology centre that are required for ablation procedures in adults with CHD and in children, the importance of being able to diagnose, monitor and manage complications associated with ablations in these patients and the supplemental hospital-based resources required, such as anaesthesia, surgical back-up, intensive care, haemodynamic assistance and imaging. Lastly, the need for quality evaluations and French registries of ablations in these populations is discussed. The purpose of this consensus statement is therefore to define optimal conditions for the delivery of invasive care regarding ablation of arrhythmias in adults with CHD and in children, and to provide expert and - when possible - evidence-based recommendations on best practice for catheter-based ablation procedures in these specific populations., (Copyright © 2020 Elsevier Masson SAS. All rights reserved.)

Introduction: Infective endocarditis (IE) is a life-threatening adverse event for patients with congenital heart disease (CHD). Its incidence has changed little over time despite progress in techniques for diagnosis and treatment, and guidelines for prophylaxis., Areas Covered: The review sought for key-words: 'congenital heart disease,' 'infective endocarditis,' 'microbial diagnosis,' 'imaging diagnosis,' 'surgical techniques,' 'prognosis,' 'prophylaxis.' Objectives were to investigate epidemiology, novel techniques for imaging and microbial diagnosis, therapeutic management and prognosis, and guidelines for prophylaxis in patients with CHD. The incidence of IE is increasing in adults with CHD. Morbidity caused by a broad clinical spectrum of cardiac and extracardiac episode-related complications is high. Surgical management is increasingly required in the early phase of the disease. Despite new techniques for diagnosis and microbiological therapy, mortality rate is still up to 10-20%., Expert Opinion: IE has increased in the growing cohort of adults with complex heart disease, living with residual cardiac lesions and prosthetic materials. Diagnosis is challenging for complex heart defects. Pet-scan technique can provide beneficial information to locate intracardiac lesions and embolic foci. Identification of the microbiological agents is improving. Innovative surgical techniques aim to avoid prosthetic material. Guidelines for prophylaxis currently emphasize oral and skin daily hygiene.

To assess the outcomes of neonates prenatally diagnosed with ventricular asymmetry and not operated on within the neonatal period and to determine the risk factors for left heart obstruction occurrence at follow-up. All neonates with prenatal asymmetry of the ventricles, diagnosed from August 1993 to July 2015, not operated on within the neonatal period, were retrospectively included in the study. Left heart echocardiographic measurements at birth and at last follow-up were collected and compared. Left heart anomaly included isthmus and/or aortic valve and/or mitral valve obstruction. There were a total of 34 newborns included in the study. The median follow-up was 2 years. There was no death. Eleven patients were operated on at a median age of three months; seven of them had an obstruction of the left heart (five coarctations of the aorta, one sub-aortic and aortic valve stenosis, and one mitral stenosis). Estimated freedom of left heart surgery was 80% at 6 months and 75% at 10 years. The main risk factor for progression to a left heart anomaly was a hypoplasia of the aortic isthmus (p = 0.0003), while the presence of a left superior vena cava was more frequent in these patients although the difference was not significant. Patients with an aortic isthmus z-score below - 2 at the closure of arterial duct are at risk of later coarctation and therefore follow-up should be extended to at least 3 months. Furthermore, the prenatal ventricular asymmetry does not only identify patients at risk of coarctation but also of other left heart anomalies. This last point should be a better approach with future parents to improve prenatal counseling on a more complex postnatal diagnostic than a simple isolated coarctation.

Infective endocarditis can negatively impact prognosis of congenital heart disease and has a 10-15% incidence of mortality. New guidelines recommend prophylaxis of infective endocarditis only for high-risk group (cyanotic congenital heart disease, valvular prosthesis, previous endocarditis) and for some specific invasive dental procedures. New guidelines are focused mainly on oral and cutaneous hygiene. Native shunts and valvular disease are no more targets for prophylaxis. More than half of the physicians do not follow the new guidelines. High-velocity shunts like ventricular septal defect should be included in the at-risk congenital disease for infective endocarditis where prophylaxis should be applied. The new guidelines for prophylaxis of infective endocarditis did not impact on the incidence of infective endocarditis., (Copyright © 2017. Published by Elsevier Masson SAS.)

Infective endocarditis (IE) is a life-threatening complication that may impair significantly the long-term prognosis of patients with cardiac disease. The profile of IE has changed over recent decades, with a decreasing prevalence of rheumatic fever and increasing survival of patients with congenital heart disease (CHDs). Given the high rates of morbidity and mortality, and based on previous experimental studies, antibiotic prevention of IE has long been recommended for at-risk groups. Serial revised guidelines for prophylaxis have been published over the years. The most recent recommendations differ dramatically from previous guidelines and provide new insights into the prophylaxis of IE. Emphasis is put on oral activities (particularly brushing teeth) as both buccal and skin hygiene may present the greatest threats for individuals at-risk of IE. Significant limitations in both at-risk patients and procedures result in a potential and substantial change in the practice of clinicians and raise concerns about the safety and reliability of these new recommendations for patients with CHD., (Copyright © 2012. Published by Elsevier Masson SAS.)

Objectives: Arterial oxygen saturation (SaO(2)) monitoring using pulse oximeter (SpO(2)) is mandatory in the intensive care unit. The aim was to assess bias and precision of new (SpO(2)ng) and old (SpO(2)og) pulse oximeter technologies in the postoperative period following pediatric cardiac surgery in cyanotic children., Study Design: Prospective, monocentric., Patients and Methods: Ten patients (7 days to 53 months old) were studied in the postoperative period following palliative cardiac surgery. SaO(2), SpO(2)og, and SpO(2)ng were obtained every 4 hours. SaO(2) of arterial blood sample was obtained from an intra-arterial catheter located in the radial artery, on the same side as the oximeters. Bias and precision were assessed using Bland-Altman analysis., Results: We obtained 136 SaO(2) determinations. Mean SaO(2) was 76+/-15%. SpO(2)og was significantly different from SaO(2), while SpO(2)ng was not different from SaO(2). In 21 (15%) cases, SpO(2)og was not available whereas SpO(2)ng was available in 136 (100%) cases. In the remaining 115 cases, SpO(2)ng's precision was significantly better than SpO(2)og's precision., Discussion: SpO(2)ng is more accurate and more reliable than SpO(2)og for SaO(2) monitoring in the postoperative period following pediatric cardiac surgery in cyanotic children.

Objective: The purpose of this study was to review the early postoperative course of stage 1 Norwood with Blalock-Taussig shunt (BTS) or right ventricle-to-pulmonary artery conduit (RVPA) and to identify early predictors of failure., Material and Methods: A retrospective analysis was conducted in 33 consecutive neonates who underwent BTS (n=19) or RVPA (n=14) stage 1 Norwood procedure between 2000 and 2005. Pre-, peri-, and postoperative data included: hourly hemodynamics and blood gases, pulmonary to systemic flow ratio, duration of mechanical ventilatory and inotrope support, intensive care and hospital stay. Failure was defined as death or transplantation., Results: Thirteen patients failed the procedure (39.4%): 10 BTS (52.6%) and 3 RVPA (21.4%). Failure decreased from 61.1% in 2000-2002 to 13.3% in 2003-2005 and was associated with: low systolic, mean and diastolic blood pressure, urine output, pH, base excess, bicarbonates, and high pulmonary to systemic flow ratio within 24 h postoperatively. Arterial oxygen and CO2 pressure, and oxygen saturation did not differ with failure. RVPA had higher diastolic blood pressure and more stable hemodynamics despite similar pulmonary to systemic flow ratio. Duration of mechanical ventilation, inotrope support, intensive care stay were shorter in RVPA. Postoperative echographic ventricular dysfunction and tricuspid regurgitation grade were correlated with failure., Conclusions: Excessive pulmonary to systemic flow ratio and low blood pressure are associated with failure. High diastolic blood pressure more than low pulmonary to systemic flow ratio seems to account for more favorable outcomes in RVPA compared to BTS procedure.

Objective: To assess the changing profile of infective endocarditis in patients with congenital heart disease., Methods: All cases diagnosed from 1966 to 2001 (revised Duke criteria) were retrospectively reviewed and categorised in periods I (< 1990) and II (>or= 1990)., Results: 153 episodes occurred, 81 in period I and 72 in period II. Mean age of affected patients was higher in period II. Non-operated ventricular septal defect, Rastelli correction and palliated cyanotic heart disease increased. Infective endocarditis in corrective surgery changed to patients with prosthetic material. Post-surgical cases decreased. Dental problems were the leading cause (period I 20% v II 33% of cases) with a large variety of pathological organisms (multiple species of Streptococcus). Cutaneous causative infections increased (5% to 17%) with different species of Staphylococcus. Negative blood cultures lessened (20% to 7%, p = 0.03). Streptococci were the most common causative organisms in both periods. Severe heart failure and cardiac complications lessened (20% to 4% and 31% to 18% during periods I and II, respectively). Early surgery was more frequent in period II (32% v 18.5%, p = 0.02). One- and 10-year survival was 91% v 97% in period I and 89% v 97% in period II, respectively (NS)., Conclusion: Current targets include complex cyanotic disease, congenital heart disease corrected with prosthetic material and small ventricular septal defect. Postoperative cases lessened; dental and cutaneous causes increased. Survival was unchanged. Prophylactic measures targeted at dental and cutaneous sources should be emphasised.

Echocardiography has revolutionized the diagnosis and follow-up of congenital heart disease over the last 20 years. Permanent technological innovation in the field of ultrasonic investigation and in the limitations inherent to this technique are illustrated in the assessment of congenital disease of the aorta, the subject of this review. The role of echocardiography associated with Doppler techniques in the investigation of congenital disease of the aorta varies with age: there is no rival technique in investigation of the foetus; in neonates, infants and young children, the role of ultrasound is preponderant because of the excellent echogenicity and the high incidence of congenital aortic disease occurring in a clinical context of cardiorespiratory distress. The limitations and insufficiencies of the techniques are greater in adolescents and adults in whom other non-invasive techniques are possible in acceptable practical conditions. The reality of progress in diagnosis is demonstrated by the possibility of therapeutic indications based only on the association of clinical and echocardiographic data without need for diagnostic catheterization and angiography. The limitations of ultrasonic techniques should however be recognized to avoid inappropriate usage.

Between December 1984 and September 1996, 43 cardiac transplantations were carried out in 40 patients aged 2 days to 21 years (one third under 10 years of age) for cardiomyopathy (21 cases), congenital heart disease (19 cases) or retransplantation (3 cases). The average waiting time for transplantation was 80 days: this delay increased by a factor of five in 2 years (from 1 month, before 1994, to 5 months at present). Twelve patients dies, including 6 before the 8th day. The 28 survivors were prescribed triple immunosuppressive therapy: the average follow-up was 4.4 years (range 3 months to 11 years). Monitoring rejection was carried out by non-invasive methods based on clinical, electrocardiographic and Doppler echocardiographic observations. Any suspicion of acute rejection led to endomyocardial biopsy for confirmation and therapeutic guidance. There was a total of 47 episodes of acute rejection (0.3 per patient), mainly in the first 3 months: acute rejection was less common in the younger children. Graft function was normal in 71% of cases. Five children have a pacemaker implanted during the first month. Despite continuous steroid therapy, 82% of patients had normal staturo-ponderal growth. The myocardial mass of the graft increased in parallel with the body surface area. Nephrotoxicity of ciclosparine was responsible for significant renal failure in 19% of patient and seemed more common in the young children. Psychological disturbances were commonest in adolescence and could result in poor treatment compliance (4 cases, with 1 death and 2 retransplantations). Despite satisfactory medium-term results, nephrotoxicity of ciclosporine, long-term graft function and psychological difficulties of adolescents remain unresolved so that transplantation is reserved for terminal cardiac disease resistant to all other forms of treatment.

Double outlet left ventricle is a very rare condition due to an abnormality of conotruncal morphogenesis. The authors report 7 new cases to the 119 already published, one with an anatomical variation not previously described. Three of the cases reported were of the most usual type similating tetralogy of Fallot. Two of these cases underwent complete correction with excellent results 13 months and 2 years after surgery. The third patient aged 6 months is well after initial palliative neonatal surgery. A case with an L-malposition pedicle with subpulmonary ventricular septal defect and pulmonary outflow tract obstruction died after early palliative surgery (Blalock-Taussig). A case with subaortic ventricular septal defect, pulmonary stenosis, and tricuspid atresia, underwent physiopathological correction (Fontan procedure) after a Waterston shunt and is well at 19 years of age. The other two cases presented more unusual anatomical forms aortic outflow obstruction: one had hypoplasia of the aortic arch with an isthmic coarctation requiring a Crafoord procedure in the neonatal period associating with banding followed by complete correction at 19 months of age. After 3 years, the patient is asymptomatic. The last case with atresia of the aortic valve and severe hypoplasia of the ascending aorta died after corrective surgery of first intent. Other cases have been described in the literature with different clinical presentations: absence of pulmonary or aortic obstruction; intact interventricular septum. The anatomical variability is due to the complex embryogenesis of the conotruncal region and explains the clinical diversity of this congenital cardiac malformation.

In emergency cases of babies with a single ventricle and an obstruction to aortic outflow, low pulmonary flow or, on the contrary, high pulmonary flow, only palliative surgical procedures can be proposed. The authors set out to determine the prognosis of a population with this type of lesion having undergone one of the three following procedures: systemic pulmonary shunt, pulmonary artery banding, repair of the aortic arch (usually associated with pulmonary banding). One hundred and nineteen (63%) of the 185 patients hospitalised between 1/01/1970 and 31/12/1991 in the paediatric cardiology unit of the Cardiac Hospital of Lyon with a diagnosis of single ventricle, underwent one of these three procedures as a treatment of first intention. The survival of the 22 patients who underwent pulmonary artery banding (90 +/- 6%, 85 +/- 8%, 85 +/- 8% at 1.5 and 10 years respectively) was significantly better than that of the patients undergoing systemico-pulmonary shunt (63 +/- 6%, 53 +/- 6% and 49 +/- 6% at 1.5 and 10 years respectively). On the other hand, repair of an obstacle of the aortic arch was a precarious procedure as the survival was only 23 +/- 11%, 16 +/- 11% and 16 +/- 11% and 1.5 and 10 years respectively). These results suggest, with the reserve inherent to the methodology of retrospective studies of small populations, that it is not illogical to continue to propose pulmonary artery banding for babies with single ventricle associated with high pulmonary flow. This procedure should only be envisaged after strict selection of candidates and providing there are facilities for intensive postoperative care.(ABSTRACT TRUNCATED AT 250 WORDS)

The authors analysed the anatomical and clinical prognostic factors of single or common ventricle. This retrospective study was based on a series of 185 patients hospitalised between 1/2/70 and 31/12/91 in the paediatric cardiological unit of the Cardiological Hospital of Lyon with this condition. A number of anatomical and clinical parameters were identified in this population. For each parameter, a given patient could only relate to single modality and a survival graph determined by Kaplan-Meier analysis was established for each modality. For each variable, the survival curve of the most frequently encountered modality served as a reference and the other modalities were compared with it using a logrank test. The different modalities potentially related to patient survival were then entered into a multivariable model using logistic regression. The results of this study of multivariable analysis using the Odds-Ratio (OR) independently suggest that four variables may influence negatively survival of patients with a single ventricle: pH < or = 7.3 on admission (OR = 3.55), a non-left ventricular morphology of the main ventricular chamber (OR = 3.11), the presence of an obstacle on the aortic outflow (OR = 5.58) and a total anomalous pulmonary venous drainage (OR = 26.88).

The poor results of palliative surgery in the hypoplastic left heart syndrome has led Bailey to propose neonatal cardiac transplantation for this condition. The authors report the case of a neonate who underwent transplantation 2 days after birth for hypoplastic left heart with mitro-aortic atresia, diagnosed in utero. The child was prescribed triple immunosuppressive therapy (steroids, azathioprine, ciclosporine) and is now 24 months old. An episode of acute rejection during the first month was treated with a short intensive course of intravenous steroids. At the fourth month, the child underwent aortic angioplasty for an isthmic stenosis. Growth is retarded (- 2SD) and he has mild renal failure but psychomotor development has been normal. The number of neonatal cardiac transplantations remains limited by parental refusal and the lack of donor organs. Despite encouraging medium term results, questions remain as to the long-term viability of the graft and the patient's renal function.