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Background: Congenital heart defects are common and occur in approximately 0.9% of births. In France, the registries cover approximately 20% of the population but not the entirety of France; therefore, we aimed to update the incidence data for congenital heart defects in France from 2013 to 2022 using the medico-administrative database PMSI-MCO (French Medical Information System Program in Medicine, Surgery, and Obstetrics). We aimed to compare the frequency of risk factors in a population with congenital heart defects and a reference population. Methods: From 2013 to 2022, we included children aged < 3 years diagnosed with congenital heart defects according to the International Classification of Diseases, 10th Revision, in the PMSI-MCO database. We compared them with a population without congenital defects on several medical data items (e.g., parity, gemellarity, and mortality rate). Bivariate and multivariate analyses compared children with congenital heart defects and children without congenital malformation. Results: We identified 83,879 children with congenital heart defects in France from 2013 to 2022 in the PMSI-MCO database and 7,739,840 children without such defects, including 7,218,952 without any congenital defects. We observed more deaths (7.49% vs. 0.68%, d = 0.59) and more twinning (8.67% vs. 1.23%, d = 0.35) among children with congenital heart defects. Multivariate analysis revealed an increased risk of congenital heart defects in male individuals (OR [odds ratio] 1.056, 95% CI [confidence interval] [1.039–1.076]) and cases of medically assisted reproduction (OR 1.115, 95% CI [1.045–1.189]) and a reduced risk in the case of multiparity (OR 0.921, 95% CI [0.905–0.938]). Conclusions: According to the PMSI-MCO database, the incidence of congenital heart defects in France from 2013 to 2022 is 1% of births. Congenital heart defects are more frequent in cases of prematurity, twinning, primiparity, male sex, and maternal age > 40 years. [ABSTRACT FROM AUTHOR]

Multisystem inflammatory syndrome in children (MIS-C or PIMS-TS) is a severe disease. N-terminal pro-B-type natriuretic peptide (NT-proBNP) is used for positive and differential diagnosis, diagnosis of complications and severity, and cardiogenic shock prediction. However, contrasting cut-offs have been suggested. The aims of the present study were to compare NT-proBNP values depending on the time of measurement and to describe the NT-proBNP course during the MIS-C episode. The data from a single-centre cohort observational study on the impact of time to diagnosis, defined as the time from first symptom to diagnosis of MIS-C, were used for the purpose of this study, with an extended period of inclusion from May 2020 to April 2023. The timing and level of all NT-proBNP samples available for each patient were retrospectively collected. Thirty-seven children (18 (49%) females, median age 8.8 years, 14 (38%) with shock) were included. Until diagnosis, NT-proBNP increased with time and was significantly higher at 6 days from first symptoms than at 3 days (median (interquartile range) 32,933 (7773–61,592) versus 1994 (1291–4190) pg/mL, respectively, p = 0.031). From diagnosis, NT-proBNP decreased by at least 50% after 3.0 (2.1–5.3) days (n = 12) when NT-proBNP at diagnosis was low ≤ 11,000 pg/mL versus 1.8 (0.7–3.4) days (n = 16) when NT-proBNP at diagnosis was high (p = 0.040), and after 3.6 (2.4–5.9) days (n = 7) when fever persisted after 48 h versus 1.8 (0.8–3.0) days (n = 21) when fever resolved before 48 h (p = 0.004). Conclusions: During the MIS-C episode, NT-proBNP increased over time until diagnosis and treatment. It dropped faster thereafter in children with high NT-proBNP at diagnosis > 11,000 pg/mL and slower in case of persistent fever. What is Known: • NT-proBNP is useful in MIS-C for positive and differential diagnosis, diagnosis of complications and severity, and cardiogenic shock prediction. • Contrasting cut-offs for differential diagnosis and severity assessment have been suggested. What is New: • Before diagnosis, NT-proBNP increases with time and is significantly higher at 6 days from first symptoms than at 3 days suggesting different cut-offs depending on the timing of measurement. • From diagnosis and treatment initiation, the 50% NT-proBNP drop occurs earlier in children with high NT-proBNP at diagnosis > 11,000 pg/mL and later in children with persistent fever. [ABSTRACT FROM AUTHOR]

Background: Transcatheter closure of a patent foramen ovale (PFO) is performed in cryptogenic stroke and other conditions. Information is lacking for some devices. Methods: We aimed to evaluate the Figulla Flex II PFO Occluder (FFP) and Figulla Flex UNI Occluder (FFU) through a retrospective multi-center registry. Results: 527 patients were included. Mean age was 48.9 (±13.8) years. The procedure was under transthoracic, transesophageal or intracardiac echocardiography in 185 (35.1%), 193 (36.6%) and 149 (28.3%) cases, respectively, and under general anesthesia in 191 patients (36.2%). The FFP and FFU were used in 408 (77.4%) and 119 (22.6%) cases, respectively. The success rate was 99.1%. Median follow-up was 1.1 (0.5–2.5) years. A new atrial fibrillation/flutter within six months occurred in 14 (2.7%) cases, with no difference between devices. One device embolization in the pulmonary artery was identified two years post-procedure. Residual shunts occurred in 18 (6.9%) cases at 1 year, with TIA in three (16.6%) patients. Out of 437 patients with stroke/TIA, 260 (59%) were followed more than one year after closure. Median follow-up was 2.1 (1.17–3.1) years, with four recurrent strokes/TIA. Conclusions: The FFP and FFU devices are safe and effective for PFO closure, with very few atrial fibrillation/flutter and neurologic events, except in cases with a residual shunt. [ABSTRACT FROM AUTHOR]

Background Pulmonary valve replacement in patients with congenital heart diseases and heart failure is challenging. Case summary Here, we describe a case of a patient who had surgical fallot repair with chronic heart failure. Investigations found severe biventricular dysfunction and enlargement due to chronic pulmonary regurgitation. The right ventricle outflow tract was tortuous and large with a diameter of 35 mm. Percutaneous pulmonary valve implantation (PPVI) was done after a challenging pre-stenting. A 32 mm Myval valve over-sized to 35 mm was used for PPVI, which yielded a good result. Discussion A 32 mm Myval valve is effective at extending the possibilities of PPVI in a large and tortuous right ventricle outflow tract not accessible for the other valves. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Long-term growth failure can have negative impact on health (by increasing morbidity and mortality) and on neurodevelopmental outcomes. Its prevalence among children with congenital heart disease (CHD) is not well described. The aim of our study was to evaluate the prevalence of growth failure in a population of infants with CHD away from cardiac surgery and identify associated factors. We conducted a retrospective and multicentric study that included infants from the North of France who underwent cardiac surgery before the age of one, between January 2013 and December 2017. 331 infants were included among which 48% had a prenatal diagnosis, 15% had a genetic syndrome, and 15% were premature infants. Mean birth weight was 3 ± 0.6 kg. At surgery, 35% presented feeding difficulties (need for enriched formula and/or feeding tube) and 14% had growth failure (defined by Z-score weight for age < -2SD). 6–12 months after surgery, 16% still presented growth failure. Several associated factors were identified: prenatal diagnosis, genetic syndrome association, birth weight ≤ 3 kg, complex CHD (≥ 2 significative lesions, or double outlet right ventricle or single ventricle physiology), surgery after 30 days, and need for diuretic drug before surgery and/or still needed 1 month after surgery. Growth failure persists between 6 and 12 months after surgery in 16% of infants with CHD. More studies are needed to link growth failure and neurodevelopment, which is the new challenge for this aging population. [ABSTRACT FROM AUTHOR]

Between December 2014 and March 2021, 144 patients with aortic (Ao) or mitral (Mi) paravalvular leaks (PVLs) were enrolled at 21 sites in 10 countries. Safety data were available for 137 patients, who were included in the safety analysis fraction (SAF), 93 patients with Mi PVLs and 44 patients with Ao PVLs. The full analysis set (FAS) comprised 112 patients with available stratum (aortic/mitral leak) as well as baseline (BL), 180-day or later assessments (2 years). Procedural success (implantation of the device with a proper closure of the PVL, defined as reduction in paravalvular regurgitation of ≥one grade as assessed by echocardiography post implantation) was achieved in 91.3% of FAS patients with Mi PVLs and in 90.0% of those with Ao PVLs. The proportion of patients suffering from significant or severe heart failure (HF), classified as New York Heart Association (NYHA) class III/IV, decreased from 80% at baseline to 14.1% at 2-year follow-up (FAS). The proportion of FAS patients needing hemolysis-related blood transfusion decreased from 35.5% to 3.8% and from 8.1% to 0% in Mi patients and Ao patients, respectively. In total, 35 serious adverse events (SAEs) were reported in 27 patients (19.7%) of the SAF population. The SAEs considered possibly or probably related to the device included device embolization (three patients), residual leak (two patients) and vascular complication (one patient). During follow-up, 12/137 (8.8%) patients died, but none of the deaths was considered to be device-related. Patients implanted with the Occlutech Paravalvular Leak Device (PLD) showed long-lasting improvements in clinical parameters, including NYHA class and a reduced dependency on hemolysis-related blood transfusions. [ABSTRACT FROM AUTHOR]

The aim of this study was to evaluate the frequency of neurodevelopmental disorders (NDD) in children with significant congenital heart disease (CHD) and to determine associated factors to NDD and frequency of follow-up in developmental therapies. Two hundred and ten children with significant CHD aged from 6 to 66 months were enrolled over a period of six months. The Ages & Stages Questionnaire Third Edition in French (ASQ-3) was used to assess neurodevelopmental domains. NDD were defined if cut-off scores were ≤ − 1SD. – 1SD corresponded to "Monitor" range: children with minor or emerging disorders; − 2SD corresponded to "Refer" range: children exhibiting neurodevelopmental delays. Forty children were in "Monitor" range and 86 in "Refer" range. NDD rate was 60.0% (n = 126, 95% CI, 53.4 to 66.6%). There was no difference regarding CHD severity (p = 0.99). Only the presence of non-cardiac disease (OR = 2.14; 95% CI, 1.11 to 4.20) was associated with NDD. Forty-six children with NDD had no developmental follow-up (among them 21 were in "Refer" range (10%)) despite this being available. Conclusion: Children with significant CHD are at risk for NDD regardless of CHD severity. Systematic and early monitoring in a specific care program is required. Barriers that prevent access of care must be identified. Trial registration: Neurodevelopmental Disorders in Children With Congenital Heart Disease. NeuroDis-CHD. NCT03360370. https://clinicaltrials.gov/ct2/show/NCT03360370 What is Known: • Children with CHD are at risk for neurodevelopmental disorders and behavioural problems impacting their social adaptation, academic achievements and quality of personal and family life even in adulthood. What is New: • Children with CHD are at risk for neurodevelopmental disorders regardless of the complexity of the CHD. • Even with the availability of appropriate developmental services, children with CHD are not correctly followed, highlighting the need of a specific program of care for a better outcome. Local barriers that prevent access of care of those children must be identified. [ABSTRACT FROM AUTHOR]

Background: The incidence of diastolic heart failure has increased over time. The evaluation of left-ventricular diastolic function is complex, ongoing, and remains poorly performed in pediatric intensive-care patients. This study aimed to review the literature and to provide an update on the evaluation of left-ventricular diastolic function in adults and children in intensive care. Data sources: We searched data from PubMed/Medline. Thirty-two studies were included. Four pragmatic questions were identified: (1) What is the physiopathology of diastolic dysfunction? (2) Which tools are required to evaluate diastolic function? (3) What are the echocardiographic criteria needed to evaluate diastolic function? (4) When should diastolic function be evaluated in pediatric intensive care? Results: Early diastole allows characterization of relaxation, whereas compliance assessments and filling pressures are evaluated during late diastole. The evolution of diastolic function differs between adults and children. Unlike in adults, decreased compliance occurs at the same time as delayed relaxation in children. Diastolic function can be evaluated by Doppler echocardiography. The echocardiographic criteria for ventricular relaxation include the E wave, E/A wave ratio, and isovolumic relaxation time. Ventricular compliance can be assessed by the E/e' wave ratio, atrial volume, and Ap wave duration during pulmonary vein flow. In adult intensive-care patients, the E/e' ratio can be used as an index of tolerance for volume expansion in septic patients and to adjust the inotropic support. Conclusion: Clinical studies would allow some of these parameters to be validated for use in children in intensive care. [ABSTRACT FROM AUTHOR]

Purpose: To report percutaneous closure of aortocaval fistulas with the Amplatzer Duct Occluder. Case Reports: An aortocaval fistula was diagnosed after surgical repair of an abdominal aortic aneurysm in a 73-year-old man. A 3-year-old girl was treated for a congenital aortocaval fistula in another case. An 8 x 6-mm Amplatzer Duct Occluder was introduced via a 6-F introducer in each case, successfully occluding the fistulous track. Both patients are well and without any echocardiographic evidence of a shunt at 6 months. Conclusions: In selected patients, transcatheter closure of aortocaval fistula with the Amplatzer Duct Occluder could be an alternative to open surgery. Further evaluation is necessary. [ABSTRACT FROM AUTHOR]

Magnetic resonance angiography is increasingly used as a non-invasive method in the evaluation of coarctation of the aorta. The aim of this study was to compare aortic dimensions calculated by MR angiography and those obtained by more conventional MR sequences and conventional angiography. Twenty-six consecutive patients with coarctation underwent three-dimensional MR angiography. Two independent observers retrospectively evaluated three aortic segments, site of coarctation, presence of aneurysm and existence of collateral circulation. Three aortic segments were also compared with those obtained on classical MR sequences and conventional angiography. The MR angiography was successfully performed in all showing 1 aneurysm and collateral circulation in 8 patients. Almost perfect intraobserver ( r(2)>0.91) and excellent interobserver ( r(2)>0.80) reliabilities were obtained for each aortic segment no matter which MR sequence was employed. Similarly, mainly excellent ( r(2)>0.80) concordance analysis was observed between MR angiography measurements and those calculated by either spin-echo/gradient-echo sequences or conventional angiography. This study demonstrates that MR angiography is a fast, accurate and reproducible method in the evaluation of coarctation of the aorta. It provides excellent anatomic information and reliably detects collateral vessels. Magnetic resonance angiography could probably replace the conventional angiography and will provide an additional diagnostic value in combination with turbo spin-echo sequence. [ABSTRACT FROM AUTHOR]

Aims The natural history of congenital or childhood non-immune, isolated atrioventricular (AV) block is poorly defined. Methods and results We retrospectively studied 141 children with isolated, non-immune AV block diagnosed in utero, or up to 15 years of age, at 13 French medical centres, between 1980 and 2009. Patients with structural heart disease or maternal antibodies were excluded. Atrioventricular block was asymptomatic in 119 (84.4%) and complete in 100 (70.9%) patients. There was progression to complete AV block in 29/41 (70.7%) patients with incomplete AV block over 2.8 ± 3.4 years (1–155 months), but all patients with incomplete AV block may not have been included in the study. Narrow QRS complex was present in 18 of 26 patients (69.2%) with congenital, and 106 of 115 (92.2%) with childhood AV block. Pacemakers were implanted in 112 children (79.4%), during the first year of life in 18 (16.1%) and before 10 years of age in 90 (80.4%). The mean interval between diagnosis of AV block and pacemaker implants was 2.6 ± 3.9 years (0–300 months). The pacing indication was prophylactic in 70 children (62.5%). During a mean follow-up of 11.6 ± 6.7 years (1–32 years), no patient died or developed dilated cardiomyopathy (DCM). The long-term follow-up was uncomplicated in 127 children (90.1%). Conclusion In this large multicentre study, the long-term outcome of congenital or childhood non-immune, isolated AV block was favourable, regardless of the patient's age at the time of diagnosis. No patient died or developed DCM, and pacemaker-related complications were few. [ABSTRACT FROM PUBLISHER]

Abstract: Objective: To report a specific pathophysiology of hemidiaphragmatic paralysis that may result in severe hypoxemia. Design: Case series. Setting: Intensive care unit in a cardiology hospital. Patients: The series included three patients with refractory hypoxemia in whom a diagnosis of right-to-left-shunt through a patent foramen ovale was made by contrast echocardiography. The three patients had a complete right hemidiaphragmatic paralysis. Intervention: Permanent percutaneous closure of the patent foramen ovale was successfully proceeded in all cases. Main result: These procedures resulted in complete normalization of arterial oxygen saturation. Conclusion: To our knowledge, only three previous reports have described the association of right-to-left shunt through a patent foramen ovale and hemidiaphragmatic paralysis. Such association may be underestimated. [Copyright &y& Elsevier]

Reports on comments of readers on diseases related to the lungs. Use of amiodarone as antiarrhythmic; Paradoxical vocal cord movement; Link of nasal obstruction to sleep apnea; Sleep-disordered breathing in middle-aged adults.

Our objective was to determine influence of age, body size, and gender on SAECG signals and to establish normal range for standard SAECG parameters in the pediatric population. Five hundred thirty healthy children and adolescents (290 males, 240 females) aged 6.0–17.3 years were studied with high resolution ECG. Patients were divided into five age groups, and each age group was divided in two subgroups according to gender. Parameters studied were filtered ORS duration, root mean square (RMS) voltage of the last 40 ms and of the last 30 ms of the filtered QRS complex, and duration of the terminal waveform of the filtered QRS < 40 μV of amplitude (LAS). After validation of the distribution of the various variables, means, standard deviations, and 95% confidence intervals were determined for each SAECG parameter at 25- to 250-Hz filtering and at 40- to 250-Hz filtering for the ten subgroups. There was a significant age and body surface area (BSA)-associated increase in filtered QRS duration in boys and girls, and a decrease in RMS voltage of the end of the ORS complex in girls. In addition, girls had a significantly shorter ORS and longer LAS durations than boys. These differences of signal-averaged signals between genders were not related to age or body size differences since analysis of covariance with age at evaluation or BSA as covariates showed the same trend. Three children (0.6%) had a SAECG out of normal range for age for the four parameters studied either at 25- or 40-Hz high pass filter. Due to the large sample studied, our results provide a basis for interpretation of SAECG in children. Normal ranges for the various variables were determined, and it appeared that adjustments for age, body size, and gender are mandatory when studying SAECG in children. [ABSTRACT FROM AUTHOR]

Background: Insufficient elastin synthesis leads to vascular complications and arterial hypertension in children with Williams-Beuren syndrome. Restoring sufficient quantity of elastin should then result in prevention or inhibition of vascular malformations and improvement in arterial blood pressure.Methods: The aim of this study was to assess the efficacy and safety of minoxidil on Intima Media Thickness (IMT) on the right common carotid artery after twelve-month treatment in patient with Williams-Beuren syndrome. We performed a randomized placebo controlled double blind trial. All participants were treated for 12 months and followed for 18 months. The principal outcome was assessed by an independent adjudication committee blinded to the allocated treatment groups.Results: The principal outcome was available for 9 patients in the placebo group and 8 patients in the minoxidil group. After 12-month treatment, the IMT in the minoxidil group increased by 0.03 mm (95% CI -0.002, 0.06) compared with 0.01 mm (95%CI - 0.02, 0.04 mm) in the placebo group (p = 0.4). Two serious adverse events unrelated to the treatment occurred, one in the minoxidil and 1 in the placebo group. After 18 months, the IMT increased by 0.07 mm (95% CI 0.04, 0.10 mm) in the minoxidil compared with 0.01 mm (95% CI -0.02, 0.04 mm) in the placebo group (p = 0.008).Conclusion: Our results suggest a slight increase after 12 and 18-month follow-up in IMT. More understanding of the biological changes induced by minoxidil should better explain its potential role on elastogenesis in Williams-Beuren syndrome.Trials Registration: US National Institutes of Health Clinical Trial Register (NCT00876200). Registered 3 April 2009 (retrospectively registered). [ABSTRACT FROM AUTHOR]

The incidence of ventricular septal defect (VSD) occurs in up to 4.5% of penetrating cardiac trauma. We report a patient with persistent VSD who underwent surgical repair with significant left-to-right shunt and signs of heart failure. We performed a successful transcatheter closure of the VSD with an Amplatzer septal occluder (AGA Medical Corporation; Golden Valley, MN). [ABSTRACT FROM AUTHOR]

The incidence of ventricular septal defect (VSD) occurs in up to 4.5% of penetrating cardiac trauma. We report a patient with persistent VSD who underwent surgical repair with significant left-to-right shunt and signs of heart failure. We performed a successful transcatheter closure of the VSD with an Amplatzer septal occluder (AGA Medical Corporation; Golden Valley, MN). [ABSTRACT FROM AUTHOR]