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[Display omitted] • Hypertrophic cardiomyopathy (HCM) is rare in children, and sudden cardiac death (SCD) is difficult to predict. • HCM Risk-Kids and PRIMaCY score have been developed to predict 5-year risk of arrhythmic events. • We tested these scores in a real-life French paediatric HCM cohort. • Positive predictive values were poor, hence additional factors should be considered. Hypertrophic cardiomyopathy (HCM) is rare in children, and sudden cardiac death (SCD) is difficult to predict. Two prognostic scores – HCM Risk-Kids and Precision Medicine for Cardiomyopathy (PRIMaCY) – were developed to assess the risk of SCD in the next 5 years in children with HCM. To test the ability of these scores to predict SCD in children with HCM. Also, to identify factors associated with a severe cardiac rhythmic event (SCRE) (ventricular fibrillation, sustained ventricular tachycardia, heart transplant for rhythmic reasons or SCD). Retrospective, multicentre, observational study at 10 medical centres in the Nord-Pas-de-Calais region, France. This study included 72 paediatric patients with HCM during 2009–2019 who were followed for a median (interquartile range [IQR]) of 8.5 (5.0–16.2) years. Eleven patients (15.3%) presented with SCRE. HCM Risk-Kids was high, with a median (IQR) score of 6.2% (2.1–12.8%; significant threshold ≥ 6.0%) and the PRIMaCY median (IQR) score was 7.1% (2.6–15.0%; significant threshold ≥ 8.3%). The positive predictive value was only 27.1% (95% confidence interval [CI] 21.5–32.5%) for HCM Risk-Kids (with a threshold of ≥ 6.0%) and 33.2% (95% CI 27.1–38.9%) for the PRIMaCY score (with a threshold of ≥ 8.3%). The negative predictive values were 95.4% (95% CI 92.3–97.7%) and 93.0% (95% CI 89.8–96.2%), respectively. Three of 28 patients with an implantable cardioverter defibrillator (ICD) experienced complications (including inappropriate shocks). HCM Risk-Kids and the PRIMaCY score have low positive predictive values to predict SCD in paediatric patients. If used alone, they could increase the rate of ICD implantation and thus ICD complications. Therefore, the scores should be used in combination with other data (genetic and magnetic resonance imaging results). [ABSTRACT FROM AUTHOR]

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Over the past twenty years, progress of cardiac surgery has been such that actual life expectancy of a patient with congenital heart disease (CHD) approaches that of the average for the general population. The current challenge is no longer to decrease cardiac surgery's mortality rate, but rather to manage morbidity and a child's overall development regarding nutrition and neurodevelopment. Malnutrition is a common cause of morbidity in infants with CHD, especially before the age of one. It can easily be explained by an increased metabolic demand linked to cardiac failure, hypoxemia or pulmonary hypertension. Secondly, food intake is frequently not optimal in this population and the infant's growth is insufficiently examined by paediatric cardiologists. Another factor is that much needed therapeutics such as diuretic drugs for example, often lead to weight loss. Ultimately, malnutrition in infants with CHD increases surgical risk, morbidity (infections, ...), hospital stay, and alters long-term cognitive development. This study reports prevalence and associated factors of malnutrition on short and long-term follow-up of a large cohort of patients with various CHD, and who underwent cardiac surgery before the age of one. We conducted a retrospective and multicentre study that included infants from the North of France with CHD who underwent cardiac surgery before the age of one, between 2013 and 2017. To determine malnutrition, anthropometric measures (weight and height) were collected on the day of the surgery and during follow-up until 3 years after surgery to calculate Z-score for weight, height and weight/height. To test the statistical significance of the association between nutritional status and risk variables, univariate analysis was done applying Chi-square test. All variables significant at 0.1 in univariate analysis were included in the multivariate logistic regression analysis. The study was approved by French national ethic committee Commission national de l'informatique et des libertés (CNIL) and by the GFHGNP ethic committee (Groupe francophone d'hépatologie–gastro-entérologie et nutrition pédiatrique). The author declares that he has no competing of interest. In total, 331 patients with CHD who underwent their first cardiac surgery (palliation or repair) before the age of one were included. Table 1 lists the cardiac diagnosis of patients included in the study. Demographic and clinical characteristics are summarised in Table 2. Caesarean section was performed in 23.5% of cases. Only 38% infants were breastfed after maternity stay. An important part of our population was treated with diuretic drugs before surgery for congestive heart failure (38.5%). 69 (21%) infants needed a gastric tube before surgery. Feeding difficulties (including need for dietary enrichment and/or presence of a gastric tube) represented 35% of our population at the day of surgery. Only 39 (12%) were specifically assessed by speech therapist for feeding problems. Concerning surgery, 246 (74.5%) infants had undergone a biventricular repair, 51 (15.5%) infants had undergone a palliative intervention before a biventricular repair and 33 (10%) had a palliative intervention before a univentricular repair. 84 (26%) were cyanotic after their first surgery, 104 (32%) needed at least a second intervention. 49 (15%) infants had postoperative complications and 84 (26%) infants were treated by diuretics for congestive heart failure one month after surgery. 19 (5.9%) infants needed intravenous (IV) nutrition for more than 7 days. There were 19 (6%) deaths. Regarding nutritional status, at the time of the surgery, 14% of the infants presented moderate to severe growth failure (with a Z score weight for age < −2SD) and 26% presented minor growth failure (with a Z scoreweight for age between −2 and −1 SD). Thus, 40% of our cohort presented minor to severe growth failure the day of the surgery. Regarding the ratio weight for height, 17% presented moderate to severe malnutrition (with a Z score weight for height < −2SD) and 30% minor malnutrition (with a Z scoreweight for height between −2 and −1 SD). Because we had a severe lack of data for height, percentages were calculated on 319 patients concerning growth failure and on 211 patients concerning malnutrition at the time of surgery. We thus, concentrated on growth failure in follow-up. At one month after surgery, 45% infants still presented feeding difficulties. Overall, 20 (6%) had a gastrostomy for feeding difficulties. During follow-up, between six months and 12 months after surgery, moderate to severe growth failure persisted in 16% of the study population and minor growth failure in 24%. In other terms, 40% of our cohort presented a persistent minor to severe growth failure. Figs. 1 and 2 summarize evolution of growth failure and malnutrition respectively during follow-up from 15 days to 3 years after surgery. Nineteen patients died during follow-up. A significant proportion of patients had impaired somatic growth or malnutrition at the end of the follow-up. Several associated factors of persistent growth failure 6 to 12 months after surgery were identified in univariate analysis: prenatal diagnosis, presence of genetic syndrome, birth weight ≤ 3 kg, complex CHD (CHD with 2 or more significative lesions or double outlet right ventricule or single ventricule physiology), surgery after 30 days from birth, and diuretic therapy before surgery and/or still necessary one month after surgery. The results of multivariate logistic regression analysis are summarized in Table 3. CHD types were divided in 4 groups regarding anatomy and complexity, using TGA, Fallot and coarctation of Aorta as referent CHD with low impact on infant growth away from the day of surgery. The presence of a feeding tube at the day of surgery was not associated with growth failure 6 to 12 months after. Growth failure in infants with CHD who undergo cardiac surgery before the age of one persists between 6 and 12 months after surgery. This should be taken into account as it can have serious consequences on children's health and development. Highlighting predictive factors will allow medical staff to identify vulnerable infants at birth and during the period of surgery and therefore increase nutritional care to combat malnutrition and growth failure. [ABSTRACT FROM AUTHOR]

Abstract Objectives The aim of this study was to describe and analyze data from patients treated in France with the Edwards SAPIEN transcatheter heart valve (Edwards Lifesciences LLC, Irvine, California) in the pulmonary position. Background The Edwards SAPIEN valve has recently been introduced for percutaneous pulmonary valve implantation (PPVI). Methods From April 2011 to May 2017, 71 patients undergoing PPVI were consecutively included. Results The median age at PPVI was 26.8 years (range 12.8 to 70.1 years). Primary underlying diagnoses were conotruncal malformations (common arterial trunk, tetralogy of Fallot and variants; n = 45), Ross procedure (n = 18), and other diagnoses (n = 8). PPVI indication was pure stenosis in 33.8% of patients, pure regurgitation in 28.1%, and mixed lesions in 38.1%. PPVI was successfully implemented in 68 patients (95.8%). Pre-stenting of the right ventricular outflow tract was performed in 70 patients (98.6%). Early major complications occurred in 4 subjects (5.6%), including 1 death, 1 coronary compression, and 2 pulmonary valve embolizations. Three of the 4 major complications occurred in the first 15 operated patients. No significant regurgitation was recorded after the procedure. Transpulmonary gradient was significantly reduced from 34.5 to 10.5 mm Hg (p < 0.0001). No patient died during a 1-month follow-up period. At 1-year follow-up, the death rate was 2.9%, and 3 patients had undergone surgical reintervention (44%). Conclusions Early results with the Edwards SAPIEN valve in the pulmonary position demonstrate an ongoing high rate of procedural success. Graphical abstract [ABSTRACT FROM AUTHOR]

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Copyright of Archives of Cardiovascular Diseases is the property of Elsevier B.V. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Even though several devices have been developed to facilitate occlusion of cardio vascular structures in adults, only a few types of plugs have been approved in selected pediatric indications. However, apart from patent ductus arteriosus (PDA) closure, little data has been published in children, particularly with regard to the heterogeneity of the procedures and outcomes. To describe the efficacy, safety, and characteristics of the utilization of the Amplatzer Vascular Plug (AVP) II and IV for cardiovascular percutaneous occlusions in children under 10 years. Retrospective multicenter (2006–2020, 6 academic centers) observational review of pediatric transcatheter occlusions using AVP II or IV. A total of 125 consecutive children (60 < 1 year, 145 lesions) underwent 136 successive procedures using 175 different devices (112 AVP IV, 63 AVP II) with a success rate of 98.5%. The mean device diameter was 7.7 ± 3.2 mm (4–20 mm). The median age and weight at implantation were 1.0 year (0.01–9.98 years) and 9.1 kg (1–69 kg), respectively. Procedures involved 55 PDA, 28 arterial bronchial or aorto-pulmonary collaterals, 18 sequestrations or Scimitar syndrome, 16 arteriovenous or veinovenous fistulae, 6 coronary fistulae, 6 vertical veins, 6 conduits, 5 ventricular septal defect, and 7 miscellaneous occlusions. The occlusion rate at Day1 was 96.3% and 100%, 6 months after, without hemolysis. Major adverse events (MAE) occurred in 5.1% mainly the need for surgery, without any related death. MAE was related to a lower weight (P = 0.04), a younger age (P = 0.02) and large PDA closure using AVP II (P < 0.01). AVP II and IV are safe and effective for the percutaneous treatment of numerous cardiovascular lesions in children under ten. In our multicenter experience, such devices represent a real "off-label" pediatric alternative in well-selected patients. [ABSTRACT FROM AUTHOR]

This study sought to investigate patient intermediate-term outcomes after transcatheter pulmonary valve replacement (TPVR) with Edwards SAPIEN valve. The Edwards SAPIEN valve, initially designed for percutaneous aortic valve replacement, has been approved for TPVR in patients with dysfunctional right ventricular outflow tracts (RVOT), but only short-term follow-up has been reported. From 2011 to 2016, 62 patients undergoing successful TPVR using the SAPIEN XT valve were consecutively included into the study. Primary efficacy and safety endpoints were defined as freedom from valve-reintervention and freedom from infective endocarditis at last follow-up, respectively. The primary efficacy outcome was met for 87,1% patients after a mean follow-up of 55 ± 21 months, corresponding to a freedom of reintervention at 5 years of 89% (95% CI 74.8–95.6%). Reinterventions were exclusively due to recurrent obstruction, no significant valvular insufficiency was observed. One case of infective endocarditis was reported, corresponding to a rate of 0.35% per patient-year (95% CI 0.01–2.00%). At 5 years freedom from infective endocarditis was 98.4% (95% CI 89.1–99.8%) (Fig. 1). Six patients died or were transplanted due to advanced cardiac failure, without relationship with TPVR. In univariate analysis, reintervention was associated with young age, a smaller tube-graft, a higher pulmonary valve gradient after the procedure and a ratio of largest implanted stent diameter to invasive balloon conduit diameter over 1.35. This study confirms the mid-term safety and efficacy of the Edwards SAPIEN XT valve in patients with dysfunctional RVOT, and identifies a patient profile associated with an uncertain benefit-risk balance. [ABSTRACT FROM AUTHOR]

Copyright of Archives of Cardiovascular Diseases is the property of Elsevier B.V. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Copyright of Archives of Cardiovascular Diseases is the property of Elsevier B.V. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Copyright of Archives of Cardiovascular Diseases Supplements is the property of Elsevier B.V. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Copyright of Archives of Cardiovascular Diseases is the property of Elsevier B.V. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Objective: Off-pump valve replacement using self-expandable stents is an emerging technique for pulmonary valve disease. However, significant limitations are the lack of easily available valve substitute to be inserted within the stent and, in the setting of repaired tetralogy of Fallot, the existence of huge pulmonary trunk. We report the first experimental results of a transventricular approach using a decellularized porcine xenograft mounted in a self-expandable stent. Methods: Pulmonary valve replacement was realized in 15 lambs by direct access of the infundibulum through a left thoracotomy, combined with pulmonary artery banding. Animals were followed by transthoracic echocardiography and, after control hemodynamic study, were electively killed either at day 7, month 1, or month 4 after implantation. Results: Implantation succeeded in all lambs. Two animals died after implantation (1 pneumothorax and 1 endocarditis). Doppler echocardiographic follow-up did not show any significant transvalvular gradient and showed only mild pulmonary regurgitation. The hemodynamic control before termination revealed a systolic pulmonary valve gradient of 18.5 ± 12.4 mm Hg at 1 week (n = 4), 13.5 ± 10.6 mm Hg at 1 month (n = 4), and 4.3 ± 4.9 mm Hg at 4 months (n = 5). Gross examination demonstrated the presence of connective tissue between the valved stent and pulmonary wall, which increased with time. Conclusion: Fifteen lambs underwent successful deployment of a self-expandable valved stent in the pulmonary position using a transventricular approach. This technique combined with pulmonary artery banding could be a therapeutic option for pulmonary insufficiency after repair of tetralogy of Fallot with a transannular patch. [Copyright &y& Elsevier]

Epidemiologic of infective endocarditis (IE) is changing and paediatric population is heterogeneous. We compare clinical characteristics according cardiac status among children. A retrospective multi centric study was conducted over a decade. Clinical presentation, treatment, outcome, microorganism, underlying congenital heart disease data were recorded. We identified 64 cases of definite IE between January 2008 to December 2018 median age was 8.0 [0.8–13.2] and 26 were girls, S aureus was the most common pathogen, n = 22 (34%), presented in Table 1. CHD represented 45 cases of IE and 38 patients had prior cardiac surgery, they were significantly younger and community acquired IE was more common. New cardiac murmur 11 (24%) vs. 11 (58%), P < 0.02, acute ischemic stroke 1 (2%) vs. 4 (21%), P < 0.02, and heart failure 11 (22%) vs. 10 (54%), P < 0.03 were significantly higher in non-CHD group. Major MDC was met more often in patients without CHD than in patients with CHD (84% versus 46%, P < 0.006). There was no difference between IE related to children with or without CHD considering the side of the localisation. Valvular replacement was more frequent in this population [13 (28%) vs. 1 (5%) P < 0.05]. Overall morbidity rate was not significantly different but neurologic complications were significantly more prevalent in patients without CHD 7 (15) vs. 11 (58). Children without CHD presented significantly more neurologic impairment 1 (2%) vs. 4 (21%), P < 0.05. CHD patients seem older, require more valve replacement and present community acquired infection when patients without CHD presented more neurological outcome and more hospital acquired infection. [ABSTRACT FROM AUTHOR]

The COCCINELLE study is a nationwide retrospective French cohort set up to evaluate the risk of radiation associated cancer in patients who undergone cardiac catheterization (CC) procedures for diagnosis or treatment of congenital heart disease during childhood [1]. Children who undergone CC procedures from 01/01/2000 to 31/12/2013 before the age of 16 in one of the 15 pediatric cardiology departments which perform pediatric CC in mainland France were included. The follow-up started at the date of the first recorded CC procedure 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. The cohort was linked to the National Childhood Cancer Registry to identify patients diagnosed with cancer and with the French National Directory for the Identification of Natural Persons to retrieve the patients' vital status. An external comparison was conducted using standardized incidence ratios (SIR). Breslow and Day's approximation was used to estimate 95% confidence intervals (CI) for the SIRs [2]. A total of 17,104 children were included in the cohort and followed for 110,335 person-years, with 22,227 CC procedures collected. Among the patients, 81.6% received only one procedure. Fifty-nine cancer cases were observed in the cohort. SIRs were increased for all-cancer (SIR = 3.8, 95% CI 2.9, 4.9), leukemia (SIR = 3.3, 95% CI 2.0, 5.4), lymphoma (SIR = 14.9, 95% CI 9.9, 22.5) and solid cancers excluding central nervous system tumors (SIR = 3.3, 95% CI 2.0, 5.5) compared with the general population. Increased risks of cancer were observed. Potential explanations include shared genetic or environmental factors, immunosuppression drugs, and exposure to medical ionizing radiation procedures. The dose-response analysis between ionizing radiation doses received during CCs and cancer occurrence will allow to address this question. [ABSTRACT FROM AUTHOR]

The Coaguchek INRange® is a self-testing meter to measure the INR, that is reimbursed in France since August 2017 for patients with mechanical valve. We evaluated the use of this anticoagulation method in a French population of GUCH (Grown-Up patients with Congenital Heart Disease) with mechanical valve. Since September 2018, 52 patients of 37 ± 11 years old were asked to attend a training course of 2 hours on anticoagulation and Coaguchek INRange® use, provided by specialized nurses, before getting home with the device. Patients had to attend a 3 months' medical re-evaluation appointment. 29 (56%) patients had an aortic mechanical valve, 12 (23%) a mitral one, 8 (15%) a double aortic and mitral one, 2 (4%) a double aortic and pulmonary one and 1 (2%) a tricuspid one. 28 patients (54%) had a mechanical valve for ≥ 10 years (group 1). In group 1, patients were older (41 ± 10 years old vs. 34 ± 10, P = 0.01). Fluindione was the preferred oral anticoagulant (30 patients, 57%), and was more frequent in group 1 (18 patients, 64%). In group 1, 61% of patients usually managed themselves dosage adjustment whereas in the other group, 52% of patients referred to their doctor. Thirty-one patients (62%) had a higher target of INR than recommended in last European guidelines. Concerning follow-up, 26 patients (50%) did not attend the 3 months appointment and had to be rescheduled or contacted by phone. 8 patients (15%) did not use the device at 3 months: 5 for variation > 15% compared to laboratory plasma technique and 3 for not trusting the new device. INR at 3 months was obtained in only 40 (77%) patients, and was in the attended target in 62% of cases. GUCH patients with mechanical valves, especially when present for ≥ 10 years, seem to validate Coaguchek, even though evaluation remains hard since a great proportion of them did not attend follow-up appointment, stick to their old INR target and did not use the device as often as recommended. [ABSTRACT FROM AUTHOR]

We report the case of an infant born prematurely at 27 weeks gestational age with life-threatening pulmonary hypertension crisis as a result of left upper pulmonary vein stenosis. Surgical treatment consisted of a lobectomy, which is a safe and effective procedure. Evidence strongly suggests that the venous stenosis may have resulted from hypertonic drugs infused through an umbilical catheter facing the upper left venous–atrial junction. [Copyright &y& Elsevier]

Background Congenitally corrected transposition of the great arteries (ccTGA) is rare congenital heart disease (CHD) with an estimated prevalence of less than 1% among all CHDs. Long time prognosis is dependent on the systemic right ventricule function, presence of heart block and surgical correction. Methods In this retrospective study, all cases of ccTGA from 2 tertiary centers (one university hospital, one private pratice) were analyzed starting from 1980. Diagnosis circumstances, associated lesions, apparition of conduction blockage, surgery (anatomical versus arterial switch), systemic ventricular dysfunction, cardiac magnetic Imaging (CMR) and cardiopulmonary exercise testing (CET) were retrieved from patient records. Results Eighty-two patients were included with a mean follow up time of 23.3 years (Interquartile range (IQR) = 25.6 years). Survival rate was 99% CI95% [96–100] at 5 years, 97% CI95% [93–100] at 10 years and 90% CI95% [81–98] at 20 years. Patients had complete heart block after a mean time of 9.7 years (IQR 14.3 years) with a prevalence of 28% CI95% [8.4–44] at 30 years. Patients with systemic right ventricule had heart failure after a mean time of 52.7 years CI95% [42.6–100]. The ejection fraction of the systemic right ventricule based on CMR was correlated with VO2 uptake (ρ=0.49, P = 0.05) at CET. Conclusion Lifetime follow-up is necessary for the diagnosis of complications of ccTGA, specifically complete heart block and systemic right ventricle dysfonction. Right heart ejection fraction with CMR is correlated and could be used as a surrogate of peak VO2 uptake at CET. [ABSTRACT FROM AUTHOR]

Kawasaki Disease (KD) is a child vasculitis. The prognosis is associated with a higher risk of coronary artery aneurysm (CAA). Currently the main goal of treatment consists of preventing CAA. At first the treatment consists on immunoglobulin (IVIG). The last American 2016 guidelines recommends echographical coronary diameter express as z-score. The epidemiology of KD is not well known in France. The aim of this study was to describe the population of the children in a region of North of France and to look for risk factors of CAA. We included patients with KD who were admitted in hospital centers of the region from 2006 to 2016. We reviewed retrospectively the medical, biological and echographical records and their monitoring data. We compared patients in group with and without CAA at 4 weeks from the diagnosis. We included 240 children from 6 hospital centers. The median age was 28 months (14–50), 20% were less than 1 year old. The male-to-female ratio was 1.8. Diagnosis was done after 7 days of fever at mean. We found 87 children with initial z-score ≥ 2DS and 28 with CAA. Patients were treated with IVIG and 35 get more than one cure, 95% get aspirin for anti-inflammatory then 87% as antiplatelet therapy. Five received corticosteroids, 1 an anti-TNFα and 1 an anti-IL1. Risk scores of CAA from Kobayashi, Egami and Sano present low sensitivity and low specificity. Several risk factors were associated with CAA: age < 6 months (OR = 4, P = 0.05), IVIG resistance (OR = 3.6, P = 0.007), z-score ≥ 2DS at diagnosis (OR = 6.7, P = 0.09 10−4) and platelet count (Pq) ≥ 444 G/L (P = 0.04). Only the initial z-score ≥ 2 DS (P = 0.02) and Pq ≥ 444 G/l (P = 0.04) were significant in multivariate analysis. The Japanese risk scores were not significant in the French population, as previously shown in North American or English populations. The initial z-score ≥ 2 DS is a good risk factor of CAA so is the Pq ≥ 444 G/l after the day 7th of fever in our population. [ABSTRACT FROM AUTHOR]

Neurodevelopmental disorders (NDD) are the most common, and potentially the most damaging sequelae in children with congenital heart disease (CHD) [1]. All areas could be affected impacting social adaptation, academic achievements, and quality of personal and family life even in adulthood. In our region, there is currently no specific additional follow-up for children with CHD. The objective was to assess the frequency of NDD in children with « significant » CHD (mild and moderate operated CHD, severe CHD), associated factors and frequency of follow-up in developmental therapies. This is a prospective observational study on children with significant CHD aged from 6 to 66 months conducted over a period of 6 months. Ages & Stages Questionnaire in French, Third Edition (ASQ-3) was used to screen neurodevelopmental domains. NDD were defined as cut-off scoring ≤ 1 SD below the normative mean. – 1 SD corresponding to «Monitor» range: children with minor or emerging disorders; – 2 SD corresponding to «Refer» range: children exhibiting neurodevelopemental delays. Socio-medico-surgical data were collected to assess associated factors and follow-up in developmental care services. Among the 210 included children, the rate of NDD was 60,0% (n = 126, 95%CI(53.4 - 66.6)). 40 children were in «Monitor» range, and 86 in «Refer» range. There was no difference regarding the severity of CHD (P = 0.99). Only presence of comorbidities (OR = 2.1; 95%CI(1.1 - 4.2); P = 0.02) was a significant associated factor of NDD in multivariate backward logistic regression analysis. 46 children with NDD had no follow-up in developmental services (among them 21 were in «Refer» range) despite provision of pathway of care for children with NDD. Children with CHD are at risk for NDD regardless of the severity of the CHD. Systematic and early monitoring in a specific program of care is necessary for all children with CHD for a better long-term prognosis. [ABSTRACT FROM AUTHOR]

Traumatic induced VSD is a rare but serious disease because of the acute hemodynamic changes. We reported one center experience in the interventional catheterization closure of traumatic induced VSD during the last ten years. We had 3 patients with four VSD. Mean age was 60 (40-71) years. VSD was muscular secondary to external trauma in one patient, and to transapical transcatheter replacement of both of the aortic and mitral valves in the second. Last patient had membranous and muscular VSD post Ross-Konno intervention. All patients had acute congestive heart failure. All procedures were performed under general anesthesia and transesophageal echocardiography control. Arteriovenous loop was always used to introduce the delivery sheath to the left ventricle. VSDs diameter was evaluated by echography and ranged from 9 to 13mm. Device diameter was chosen 1 to 2mm over the echo-graphic measures. Multiple devices were used (Amplatzer® septal occluder, Amplatzer® muscular VSD occluder, Occlutech® Figulla septal occluder). Mean procedures time was 113 (100-145) min, and mean irradiation dose was 160 (103-203) Gycm2. Non significant residual shunt was observed in all patients, but the heart failure was resolved in all. Complications were registered in three procedures: transient hemolytic anemia, severe bradycardia, tricuspid cordage rupture and groin hematoma. Conclusion Traumatic VSD closure is required because of the acute hemo-dynamic changes. Trancatheter closure is effective. Complications are frequents because of the critical clinical status. [ABSTRACT FROM AUTHOR]

Introduction Fontan patients can develop veno-venous shunts with time, which are usually closed percutaneously when they reduce oxygen saturation. But the significance of these shunts regarding the type of congenital heart disease or surgical repair and their clinical relevance is not well known. Objective The purpose of this study was to evaluate the proportion of Fontan patients presenting veno-venous shunt and to evaluate the impact on aerobic capacity. Results Twenty-six Fontan patients (mean age 23 ± 9 years) who underwent cardiopulmonary exercise testing with blood gas measurement between January 2016 and January 2018 were included. Twenty patients had total cavopulmonary connection with extracardiac conduit surgery and 6 had atriopulmonary connection. Mean oxygen saturation at rest was 94.9 ± 2.1%. A resting shunt fraction, defined as PaO 2 + PaCO 2 < 120 mmHg was found in 100% of patients, with a mean of 96 ± 6 mmHg. In the group of patients presenting an important shunt fraction (defined as PaO 2 + PaCO 2 ≤ 96 mmHg), patients reached a lower maximal workload (102 ± 24 versus 121 ± 38 watts, P = 0.07 or 58 ± 10 versus 69 ± 15% of predicted value, P = 0.04) and had lower maximal oxygen consumption (22 ± 6 versus 27 ± 9 mL/kg/min, P = 0.06 or 54 ± 9% versus 69 ± 16% of predicted value, P < 0.01). There was a small correlation between the importance of shunt fraction and maximal oxygen consumption, r 2 = 0.22. Conclusion The importance of shunt fraction in Fontan patients seems correlated to aerobic capacity. Further studies are needed to confirm these results. Whether percutaneous closing of veno-venous shunts in Fontan patients can reduce this shunt fraction and could improve maximal oxygen consumption still remains to be proved. [ABSTRACT FROM AUTHOR]

Introduction Pulmonary hypertension (PH) affects 16–25% of premature infants with bronchopulmonary dysplasia (BPD), contributing significantly to perinatal morbidity and mortality. Inflammation is highly implicated in the pathogenesis of PH and BPD. Polyunsaturated fatty acids ω-3 (PUFA ω-3) can improve vascular remodeling, angiogenesis, and inflammation under pathophysiological conditions. However, the effects of PUFA ω-3 supplementation in BPD-associated PH are unknown. Objective Evaluate the effects of PUFA ω-3 on pulmonary vascular remodeling, angiogenesis, and inflammatory response in a hyperoxia-induced rat model of PH. Methods From embryonic day 15, pregnant Spague-Dawley rats were supplemented daily with PUFA ω-3, PUFA ω-6, or normal saline (0.2 ml/day). After birth, pups were pooled, assigned as 12 per litter, and randomly to either in air or continuous oxygen exposure (FiO 2= 85%) for 20 days, then sacrificed for pulmonary hemodynamic, morphometric, and immunohistological analysis. Results PUFA ω-3 supplementation improved survival, decreased right ventricular systolic pressure and RVH caused by hyperoxia, and significantly improved alveolarization, vascular remodeling, and vascular density. PUFA ω-3 supplementation produced a higher level of total ω-3 in lung tissue and breast milk, and was found reversing the reduced levels of VEGFA, VEGFR-2, ANGPT-1, TIE-2, eNOS, and no concentrations in lung tissue, and the increased ANGPT-2 levels in hyperoxia-exposed rats. The beneficial effects of PUFA ω-3 in improving lung injuries were also associated with an inhibition of leukocyte infiltration, and reduced expression of proinflammatory cytokines IL-1β, IL-6 and TNF-α. Conclusion These data indicated that maternal PUFA ω-3 supplementation strategies could effectively protect against infant PH induced by hyperoxia. [ABSTRACT FROM AUTHOR]

Agenesis of the ductus venosus is a rare anomaly that may be responsible of cardiomegaly and hydrops in fetuses. We report 2 cases in combination with pulmonary artery hypertension (PAH). Agenesis of ductus venosus was diagnosed in 2 boys at the second term of pregnancy. None had associated hydrops. Chromosomal karyotyping was normal in both. Both of them had extracardiac malformation: one had oesophageal atresia (requiring surgery at day 1), the second had malformation of the ear and micropenis. Both had neonatal PAH. The first patient received bosentan at the second month of life. He had persistent PAH (PA pressure of 119/48, mean 77mm Hg) and underwent closure of a small VSD at the age of 7 months. Two years and half after surgery, he remains in NYHA class II with similar PA pressure despite bosentan therapy. He is awaiting for combination therapy with sildenafil. The second presented pulmonary distress at birth requiring mechanical ventilation. He was placed immediately under a combination therapy of epoprostenol and NO for 4 days, received then treprostinil for 15 days and sildenafil. He could be weaned off the ventilator after 3 days. One month and half after birth, he is doing well under sildenafil but has persistent moderated PAH (tricuspid regurgitation velocity of 3 m/s). The antenatal existence of high grade arteriovenous shunt could explain high pulmonary artery pressure. However, the persistence of PAH in these patients remains unclear. In combination with research of chromosomal anomaly and congenital malformation, infants with agenesis of the ductus venosus should benefit after birth of serial ultrasound examination to rule out PAH. [ABSTRACT FROM AUTHOR]

Purpose: When isolated and non immune, prevalence of congenital and childhood atrioventricular blocks (AVB) is extremely low and little is known about their natural history. Methods: A multicentric study retrospective from 1980 to 2009 allowed inclusion of 141 children from 13 French referral centers. Included children presented an AVB diagnosed in utero, at birth or during childhood before the age of 15 years, without structural heart abnormalities and without maternal antibodies. Results: 26 congenital and 114 childhood AVB were included. Symptoms lead to diagnosis in 15,6% whereas AVB was asymptomatic in 84,4%. 73% AVB were complete and 26,2% AVB were incomplete at first presentation. 21 of these incomplete blocks (56,7%) progressed to permanent complete AVB. Narrow QRS complexes were found in 69,2% congenital and 91,2% childhood AVB. In the 112 (79,4%) implanted children, mean duration between AVB diagnosis and pacemaker implantation was 35 months. Pacemaker primo-implantation occurred during the first year of life for 18 children and 90 children (63.8%) were paced before 10 years old. Pacing was required for symptomatic bradycardia in 37,5% whereas prophylactic cardiac pacing accounted for 61,6%. The median follow-up was 96 months (from 6 to 384 months). 85,1% experienced no complication and neither dilated cardiomyopathy nor death had occurred at last follow-up. Pacemaker-related complications appeared in 11,6%. Conclusion: We describe the largest reported experience with isolated and non immune congenital and childhood AVB. Such a block is a nodal damage from unknown origin that may postnatally progress in incomplete forms. Outcome is not influenced by age at diagnosis. Prognosis is very good with no late-onset dilated cardiomyopathy, a few pacemaker-related complications in the modern technological era and no death at last follow-up. [ABSTRACT FROM AUTHOR]

Purpose: When isolated and non immune, prevalence of congenital and childhood atrioventricular blocks (AVB) is extremely low and little is known about their natural history. Methods: a multicentric study retrospective from 1980 to 2009 allowed inclusion of 141 children from 13 French referral centers. Included children presented an AVB diagnosed in utero, at birth or during childhood before the age of 15 years, without structural heart abnormalities and without maternal antibodies. Results: 26 congenital and 114 childhood AVB were included. Symptoms lead to diagnosis in 15,6% whereas AVB was asymptomatic in 84,4%. 73% AVB were complete and 26,2% AVB were incomplete at first presentation. 21 of these incomplete blocks (56,7%) progressed to permanent complete AVB. Narrow QRS complexes were found in 69,2% congenital and 91,2% childhood AVB. In the 112 (79,4%) implanted children, mean duration between AVB diagnosis and pacemaker implantation was 35 months. Pacemaker primo-implantation occurred during the first year of life for 18 children and 90 children (63.8%) were paced before 10 years old. Pacing was required for symptomatic bradycardia in 37,5% whereas prophylactic cardiac pacing accounted for 61,6%. The median follow-up was 96 months (from 6 to 384 months). 85,1% experienced no complication and neither dilated cardiomyopathy nor death had occurred at last follow-up. Pacemaker-related complications appeared in 11,6%. Conclusion: We describe the largest reported experience with isolated and non immune congenital and childhood AVB. Such a block is a nodal damage from unknown origin that may postnatally progress in incomplete forms. Outcome is not influenced by age at diagnosis. Prognosis is very good with no late-onset dilated cardiomyopathy, a few pacemaker-related complications in the modern technological era and no death at last follow-up. [ABSTRACT FROM AUTHOR]

Introduction Infantile haemangioma (IH) is a very common vascular tumour that affects up to 10% of newborns. Since 2008, oral propranolol is used to treat complicated IH, like haemangioma that obstruct vital structures or ulcerated haemangioma. Objective The aim of this study was to investigate, by a retrospective review, the therapeutic results and effects of propranolol on cardiovascular and biological parameters in infants treated for complicated infantile haemangioma and to assess its safety. Results All paediatric patients with complicated IH who started systemic propranolol from February 2009 to December 2014 were included. 218 patients (155 girls and 63 boys) were treated by propranolol. The mean weight was 6780g (2115g to 20000g). Median age at beginning of treatment was 4.7 months (10 days to 6 years). The most frequent localisation of IH was facial (63 patients), palpebral (52 patients), perineal (20 patients), labial (14 patients), airway obstruction (8 patients) and 1 PHACE syndrome. Median length of therapy was 7.5 months for facial IH, 6 months for palpebral, 5.6 months for perineal IH and 7 months for subglottic localisation. Adverse events were observed: hypoglycemia (n=11 patients aged less 6 months), arterial hypotension (n=103 patients, especially at the second and third dose with dose titration), bradycardia (n=120). Transthoracic echocardiography was realised in 158 patients: 19 pathologies were found (8 PDA, 4 ASD, 4 mitral regurgitations, 2 VSD and 1 coarctation). Other adverse events occurred in 49 patients (wheezing, acrocyanosis, diarrhoea, sleep disturbance) leading to modification in management. Complete response was observed for all but 11 (partial or no response). Conclusion In appropriated patients, propranolol therapy is effective for severe or complicated IH. We must be aware of frequent adverse events under beta-blocker in these patients. [ABSTRACT FROM AUTHOR]