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Acute myocarditis (AM) is a rare but severe disease affecting patients of all age. Large multicentric data comparing children and adults are lacking. We aimed to elucidate differences in presentation, management, and outcomes of AM across age groups. A comprehensive French national cohort study, encompassing 53 pediatric and adult units from March 2020 to November 2021, was analysed. Baseline characteristics and evolution, management and in-hospital complications were collected. Major cardiovascular events (MACE) within 30 days included all-cause death, cardiogenic shock, cardiac arrest, ventricular arrhythmias, or complete AV block. We included 745 AM patients (328 children and 417 adults), mainly male (73.4%) with a median age of 19.8 years [IQR: 12.5–30.9]. Multisystem inflammatory syndrome (MIS) was more prevalent among pediatric cases (69.8%), whereas infectious aetiology dominated in adults (13.4 vs. 52.4%). Children exhibited a more severe clinical presentation, with increased risk of heart failure (15.9 vs. 7.2%) and cardiogenic shock (14.4 vs. 6.9%), requiring higher use of inotropes (25.0 vs. 9.4%), vasopressors (12.0 vs. 6.2%), and ventilatory support (12.5 vs. 7.7%). Cardiac treatments such as beta-blockers (33.4 vs. 84.4%) or ACE/ARB (37.3 vs. 63.1%) were less often used in children whereas corticosteroids (68.3 vs. 14.3%) and immunomodulators (65.1 vs. 4.5%) were more often used. MACE occurrence was substantial but not significantly different between children and adults (18.1 vs. 13.4%). Extra-cardiac manifestations at admission were significant predictors of MACE (aOR: 2.40 [1.43–4.38]), regardless of multisystem inflammatory syndrome (MIS) status. AM exhibits variations in presentation, aetiologies, and management, but shares a comparable 30-day prognosis in children and adults. [ABSTRACT FROM AUTHOR]

Acute myocarditis (AM) is a rare but severe disease affecting patients of all age. Large multicentric data in children and comparison with adult are lacking. We aim to compare AM presentation, management, and outcomes according to age of the patients at admission and to identify factors associated with 30-day prognosis. All cases of AM were included between March 2020 and November 2021 in 64 adults and children centres in France. We collected baseline data, AM presentation and evolution, in-hospital management and in-hospital complications. All-cause death and major cardiovascular events (MACE) (acute pulmonary oedema, cardiogenic shock, or cardiac arrest) were investigated with a 30-day systematic follow-up. NCT04375748 In total, 745 AM patients (417 adults and 328 children) were included, mainly men (73.4%) with a median age of 19.8 [12.5–30.9] years old. Children had more extra-cardiac manifestations (78.3% vs. 24.0%, P < 0.01), more clinical signs of heart failure (14.9% vs. 7.0%, P < 0.01) and shock (47.9% vs. 12.7%, P < 0.01). They also had lower left and right ventricular function on TTE. The main aetiology of AM was viral myocarditis (32.6% of the overall population) with a predominance in adults (50 vs. 20% in children). Children had less toxic myocarditis (0 vs. 2.6%, P < 0.01), immunologic myocarditis (1.0 vs. 7.0%, P < 0.01), vaccine-related myocarditis (5.0 vs. 9.0%, P < 0.01) and idiopathic AM (9.0 vs. 28.0%, P < 0.01). Multisystem inflammatory syndrome was predominant in children (66.0 vs. 1.0%, P < 0.01). Cardiac treatments as beta blockers (84.4 vs. 33.4%, P < 0.01) or ACE/ARB2 (63.1 vs. 37.3%, P < 0.01) were significantly more often used in adults. On the contrary, corticosteroids (68.3 vs. 14.3%, P < 0.01) and immunomodulators including antiviral and/or IGIV were significantly more used in children (65.1 vs. 4.5%, P < 0.01). In-hospital complications occurred more in children (23.8 vs. 14.3, P < 0.01) especially acute kidney injury (11.0 vs. 5.3%, P < 0.01) and cardiogenic shock (15.2 vs. 8.1%, P < 0.01). At 30-day, adults presented more composite endpoint including death, cardiac arrest, cardiogenic shock, and acute pulmonary oedema (3.4 vs. 0.9% respectively in adults and children, P < 0.03) AM presentation, aetiologies and management differs according to age. Despite higher rate of in-hospital complications in children, the 30-day prognosis is worse in adult. [ABSTRACT FROM AUTHOR]

Cardiac catheterization (CC) has largely improved the diagnosis and treatment of congenital heart disease in children over the last decades, but it leads to exposure to low doses of X-rays. Children have a greater radiation sensitivity, however, little is known about the long-term radiation associated cancer risks. This study aims to assess the risk of lympho-hematopoietic malignancies among children diagnosed and/or treated with CC. The COCCINELLE cohort includes children from 15 French paediatric cardiology departments who underwent a first CC between 2000–2013, before 16 years old. The cohort was linked with national registries to identify cancer cases and collect information on predisposing factors to cancer (PF). Individual cumulative doses to active bone marrow (ABM) were calculated with the PCXMC software, based on detailed dosimetric information retrieved from reports of 1,139 CC procedures. Standardised incidence ratios (SIRs) and relative risks (RR) of haematological malignancies associated with the 2-year lagged ABM dose were calculated. The cohort included 17,104 children, followed 6.5 years on average. 22,227 CC procedures were collected, with a mean individual ABM cumulative dose of 3.0 mGy. 38 lympho-hematopoietic malignancies were counted (23 lymphoma and 15 leukemia). A significant excess of cancer incidence was observed (SIR = 3.8 [95% confidence interval 2.9; 4.9]), which wasn't anymore observed after excluding patients with PF (SIR = 1.3 [0.6; 2.7]). The ABM dose wasn't significantly associated with the risk of lympho-hematopoietic malignancies (RR/mGy = 1.00 [0.88; 1.10]) or lymphoma (RR/mGy = 1.03 [0.90; 1.14]) after adjustment for attained age, gender and PF. No significant increase of cancer incidence was observed after excluding patients with PF and no dose-response relationship was observed between the risk of lympho-hematopoietic malignancies or lymphoma and cumulative ABM dose arising from CC procedure. The extension of the COCCINELLE study and the European Harmonic project will help to better assess health effects of CC exposure during childhood. [ABSTRACT FROM AUTHOR]

Although perinatal complications during pregnancy in women with Congenital Heart Disease (ACHD) are well known, the pathophysiological mechanisms are still to be elucidated. As the placenta is the interface between mother and fetus, its analysis might help to better understand their interactions and identify risk factors for complications. We hypothesize that abnormal findings would be more frequent in placentas of women with CHD than in those without cardiac disease. The main objective of this study is to compare the incidence of placental abnormalities (PA) in ACHD versus controls women. The present proposal is to conduct a prospective, multicentric, national, 2-years (2023–2024) cohort study (research involving human type III), in order to analyze Doppler imaging and histological placentas, and compare findings in a group of women with ACHD versus a group without. The secondary objectives of the study are to assess the association between PA and the occurrence of maternal cardiovascular, fetal and/or perinatal complications, and to assess risk factors for PA or maternal/fetal events (Fig. 1). Placentas of ACHD would have significantly (P < 0.05) more abnormalities and these would correlate with the occurrence of complications. Description of PA could help to elucidate the pathophysiological mechanisms and eventually propose preventive therapeutic strategies of fetal and/or neonatal complications. Risk factors could be stratified according to the type of heart disease (as the mWHO score) and could allow to propose a novel risk-score of pregnancy in women with CHD. [ABSTRACT FROM AUTHOR]

Anomalous aortic origin of a coronary artery, particularly in the presence of inter-arterial course between the great arteries, has been found to be associated with sudden death in young people. This study reports a single-center experience in the management of anomalous aortic origin of a coronary artery, with or without inter-arterial course, by focusing specifically on presentation, diagnosis, and patient outcome. From March 1993 to February 2018, 100 patients (70 males) were diagnosed with anomalous aortic origin of a coronary artery, including 27 left coronary artery from the right sinus, 60 right coronary artery from the left sinus, and 13 other anomalies. Patients with inter-arterial course between the great arteries presented more frequently with syncope and/or sudden death compared to patients without (23.4% vs. 0%, P = 0.026). Diagnosis was reached using first-line transthoracic echocardiography in 65% of cases. Surgical repair was performed in 61 patients (61%). All operated patients (60 direct implantations, 1 unroofing) had been diagnosed with inter-arterial course between the great arteries, and were asymptomatic at a mean (±SD) postoperative follow-up of 4.9 ± 5.3years. An inter-arterial course of the anomalous right or left coronary arteries arising from the opposite sinus is associated with life-threatening events. Direct reimplantation of coronary artery is reliable, and should be discussed even in asymptomatic patients. [ABSTRACT FROM AUTHOR]

The aim of the study was to assess the long-term outcome of patients with ALCAPA. Methods Retrospective single-center analysis of patients who underwent surgery for ALCAPA from 1980 to 2012. Surgical techniques, demographics, echocardiographic parameters and outcomes were collected. Patients were divided into group I<2years at diagnosis, group II:>2years). Results Forty-eight patients (28 females) were included, median age 6 months (min 4 months, max 65 years), median weight 6kg (min 1.9kg): 36 in group I and 12 in II. HF was patent in 39 patients (mean age 4 years), 9 were asymptomatic (mean age 10 years), Qwave on ECG was present in 87% of cases. Mean LVSF=24.2% (group I=26.6% vs II=37.7%, and 16.6% in patients<6 months of age), LVEDD and LVESD Z-scores were respectively +2 and +5 in groups I and II; 73% had MR: severe in 6%, moderate in 46%, mild in 21%. Left coronary artery ostium located in the left posterior sinus in 31cases, right posterior sinus in 12 and in right pulmonary branch in 4. Direct coronary artery reimplantation was performed in 71%, Takeuchi technique in 6%, Meyer technique in 20% and LCA ligation in 3%. Mean age at surgery was 29 months, mean weight 9kg. Postoperative mechanical circulatory support was required in 3 cases, who had more severe HF, lower LVSF and longer bypass duration. Mean FU was 81 months (6 to 312 months). Freedom from reoperation was 100% at 1 year, 91% at 10 years and 88% at 20 years. LVSF increased by 20% in the early postoperative course and 36% at late FU. MR improved significantly in most of the cases. Overall mortality was 33% (15 in group I died before day 30, none in group II), decreasing over time from 55% to 11%, and was lower in patients who had direct reimplantation. Q wave disappeared in 82% of the cases; 93% of survivors were asymptomatic at latest evaluation. Conclusion ALCAPA patients have good long-term survival and outcome. Age>2 years at diagnosis and direct implantation are factors of favorable prognosis. [ABSTRACT FROM AUTHOR]

In the context of recent change in guidelines for prophylaxis of infective endocarditis (IE), the objective of this study was to assess the features and outcomes of IE in children and adults with non-significant ventricular septal defect (VSD). Methods A retrospective analysis of records of patients with non-significant VSD. Clinical, echocardiographic and microbiological data, and outcomes were assessed. Results From 1980 to 2013, 57 IE occurred (1 to 4 per year), in patients aged 14.2±11.3 years (med. 12.1), 29 males (51%) and 39 were<18 years of age. VSD was membranous in all cases, isolated (39=68.5%) or associated with mild aortic regurgitation or pulmonary stenosis. VSD was native in 39 (68.5%) and not diagnosed before IE occurred in 4cases (7%). The cause of infection was unknown in 36% of the cases, while 23% were from dental, 13% from cutaneous, 9% from ENT or digestive origin, and 19% occurred in the early postoperative course of patch closure, i.e. 81% of the cases occurred in native mild VSD. Streptococcus from dental origin was the most frequent causal agent (54.5%), staphylococcus was found in 35% of cases, Gram-negative bacillus in 3.5%. Hemocultures were negative in 7% of the cases. Vegetation was the most frequent echographic lesion, and located either on VSD, and/or tricuspid valve and/or RV free wall and/or pulmonary valve. Aortic valve location occurred in 8cases. Embolic event occurred in 28 cases (49%): multiple pulmonary embolia in 21 (37%), systemic embolia in 6. Eighteen patients were operated (31.6%): early surgery in 11 (19.3%), delayed patch closure in 7. Six patients died (10.5%). Death was not related to early surgery. FU was 13.4±11.2 years (med. 10.2 years). Conclusion Infective endocarditis impairs prognosis of mild membranous VSD and dental events are the most frequent origin of infection. Preventive surveillance and management of any dental lesion are probably to be emphasized in these patients. [ABSTRACT FROM AUTHOR]

This study was to: 1) analyze impact of antenatal echocardiography, 2) assess pronostic factors of outcome, in neonates and infants with coarctation of aorta (COA). Methods Single-center analysis of clinical and echocardiographic data of fetus with suspicion of, and neonates and infants<1year of age with COA. Groups DAN (antenatal diagnosis) and DPN (postnatal diagnosis) were compared. Results 286 cases were included, 106 in DAN group and 180 in DPN. Among 106 antenatal suspected COA, 11 were not confirmed after birth (sensitivity=34.5%). Antenatal ascending aorta diameter (AAO) and AO/PA ratio were predictors of postnatal COA. Heart failure (HF) was the main symptom in DPN group, absent in DAN (P<0.0001). Antenatal echocardiography was associated with lower diameter of isthmus (AOI), transverse arch (TrAO) and AAO. DPN had lower LV systolic fraction (LVSF) than DAN. Preoperative HF was associated with narrowest AOI, AAO, TrAO and decreased LVSF. PGE was administred in 53% preoperatively and mechanical ventilation in 39%. Among 275 confirmed COA (95 DAN and 180 DPN), 272 underwent Crafoord operation, at mean age 29days (median 12days) and mean time after admission 3.4days. Age at surgery was lower and time to surgery longer in DAN than DPN group. Residual postoperative aortic gradient was observed in 18 cases (6.7%), and spontaneously disappeared in the majority of them within 2 postoperative days. Median hospital stay was 11days. Survival was 98.5%. Restenosis occurred in 20 cases(7%), was related to postoperative AOI diameter and gradient, and AAO, and was more frequent in patients<1month at surgery (8.5%). Mortality was associated with Shone complex and parachute mitral valve (P<0.0001), LV diameters (P=0.02), ICU stay (P<0.0001), hospital stay (P=0.02). Conclusion Antenatal diagnosis impacts on early outcomes of neonates with COA. Postoperative isthmus diameter and gradient, and <1month age at surgery impact on restenosis. [ABSTRACT FROM AUTHOR]

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Methods Retrospective single-centre analysis of long-term posttransplant outcome, with chart collection of clinical and paraclinical data [this study assessed the long-term outcome of heart (HTx) and heart-lung transplantation (HLTx) in patients with congenital heart disease (CHD) and children with non-congenital cardiac or pulmonary disease From 1984 to 2013, 111 first-HTx, 5 HLTx and 6 re-HTx were performed (62 males), in patients aged 11.7 "0.25" ± "0.25" 8.2 "0.25" y: 96 (79%) aged "0.25" < "0.25" 18 "0.25" y. Cardiopathy included 61 cardiomyopathies (50.8%), 50 CHD (41.7%), 6 retransplants (5%). HLTx included 1 Eisenmenger, 1 PPHT, and 2 pulmonary diseases. Patients with cardiomyopathy were younger than CHD (8.7 "0.25" y vs. 14.9 "0.25" y). Seventeen (14%) patients had circulatory mechanical support as bridge to transplant. Acute rejection occurred more frequently within the first year post-transplant or "0.25" > "0.25" 5th year in non-compliant teenagers. Overall, 33 patients died (27%), 3.5 "0.25" ± "0.25" 4.6 "0.25" y post-Tx (1 "0.25" day to 16.4 "0.25" y, med 1.5 "0.25" months), due to early multivisceral failure in 6 (18%), pulmonary hypertension in 3 (9%), acute rejection in 7 (21%), graft coronary disease in 6 (18%), sepsis in 5 (15%) and miscellaneous in 6. Graft coronary disease occurred in 15 (12.4%): 4 had re-HTx, 6 died and 5 are alive. Five lymphoma occurred, 4 "0.25" months to 14 "0.25" y after HTx, cured in 4 (1 died). Patients survival was 85% at 1 "0.25" y, 81% at 5 "0.25" y, 70% at 10 "0.25" y and 61% at 20 "0.25" y post-transplant. Graft survival rates were respectively 82%, 68% and 52% at 5 "0.25" y, 10 "0.25" y and 20 "0.25" y post-transplant. Survival did not differ with pretransplant disease, age, gender, pretransplant mechanical support. Mortality was higher in patients with coronary disease (40%) than those free from (25%). Long-term prognosis after HTx and HLTx is favourable. Graft coronary disease is the main cause of failure, less frequent than in the adult non-CHD heart-transplanted population. [ABSTRACT FROM AUTHOR]