Publications by authors named "Damien Bonnet"

378 Publications

Cardiovascular events in perimembranous ventricular septal defect with left ventricular volume overload: a French prospective cohort study (FRANCISCO).

Cardiol Young 2021 Sep 23:1-6. Epub 2021 Sep 23.

Department of Pediatric Cardiology, centre de competence M3C, Centre Hospitalier Universitaire Charles Nicolle, Rouen, France.

The long-term prospective multi-centre nationwide (French) observational study FRANCISCO will provide new information on perimembranous ventricular septal defect with left ventricular overload but no pulmonary hypertension in children older than 1 year. Outcomes will be compared according to treatment strategy (watchful waiting, surgical closure, or percutaneous closure) and anatomic features of the defect. The results are expected to provide additional guidance about the optimal treatment of this specific population, which is unclear at present.

Background: The management of paediatric isolated perimembranous ventricular septal defect (pmVSD) with left ventricle (LV) volume overload but no pulmonary arterial hypertension (PAH) remains controversial. Three therapeutic approaches are considered: watchful waiting, surgical closure, and percutaneous closure. We aim to investigate the long-term outcomes of these patients according to anatomic pmVSD characteristics and treatment strategy.

Methods: The Filiale de Cardiologie Pediatrique et Congénitale (FCPC) designed the FRANCISCO registry, a long-term prospective nationwide multi-centre observational cohort study sponsored by the French Society of Cardiology, which enrolled, over 2 years (2018–2020), patients older than 1 year who had isolated pmVSD with LV volume overload. Prevalent complications related to pmVSD at baseline were exclusion criteria. Clinical, echocardiographic, and functional data will be collected at inclusion then after 1, 5, and 10 years. A core lab will analyse all baseline echocardiographic data to depict anatomical pmVSD features. The primary outcome is the 5-year incidence of cardiovascular events (infective endocarditis, sub-aortic stenosis, aortic regurgitation, right ventricular outflow tract stenosis, tricuspid regurgitation, PAH, arrhythmia, stroke, haemolysis, heart failure, or death from a cardiovascular event). We plan to enrol 200 patients, given the 10% estimated 5-year incidence of cardiovascular events with a 95% confidence interval of ±5%. Associations linking anatomical pmVSD features and treatment strategy to the incidence of complications will be assessed.

Conclusions: The FRANSCICO study will provide the long-term incidence of complications in patients older than 1 year with pmVSD and LV volume overload. The results are expected to improve guidance for treatment decisions.
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http://dx.doi.org/10.1017/S1047951121002717DOI Listing
September 2021

Hemodynamic and prognostic impact of the diastolic pulmonary arterial pressure in children with pulmonary arterial hypertension-a registry-based analysis.

Cardiovasc Diagn Ther 2021 Aug;11(4):1037-1047

Pediatric Heart Center, University Children's Hospital Frankfurt/Giessen, Frankfurt, Germany.

Background: Diastolic pulmonary arterial pressure (dPAP) is regarded to be less sensitive to flow metrics as compared to mean PAP (mPAP), and was therefore proposed for the assessment of a precapillary component in patients with postcapillary pulmonary hypertension (PH). To analyze the diagnostic and prognostic impact of dPAP in patients with pure precapillary PH, we purposed to compare the correlation between dPAP and mPAP, as well as hemodynamically-derived calculations [ratio of PAP to systemic arterial pressure (PAP/SAP), pulmonary vascular resistance index (PVRI), transpulmonary gradient (TPG)], using both dPAP and mPAP, at rest and during acute vasoreactivity testing (AVT) in children with idiopathic or heritable pulmonary arterial hypertension (IPAH/HPAH). Furthermore, we aimed to assess the association of these metrics (at baseline and changes after AVT) with transplant-free survival.

Methods: We conducted a retrospective analysis of the TOPP (Tracking Outcomes and Practice in Pediatric Pulmonary Hypertension) registry including 246 IPAH/HPAH patients. Of these, 45 children (18.3%) died, and 13 (5.3%) received lung transplantation during the observation period.

Results: dPAP and mPAP-derived variables showed almost linear relationship. Higher mPAP/mSAP, and dPAP-/mPAP-derived PVRI at rest was associated with time to death/transplantation. At maximum AVT-response, the decrease of dPAP and mPAP, diastolic pulmonary gradient (DPG) and TPG, as well as dPAP/dSAP and mPAP/mSAP was associated with time to death/transplantation, showing higher significance than corresponding baseline values. Remarkably, no predictive value was found for PVRI-reduction during AVT, neither dPAP- nor mPAP-derived.

Conclusions: There is a strong relationship between dPAP and mPAP-derived variables. According to our results, hemodynamics during AVT (irrespectively of dPAP- or mPAP-derived) may have more prognostic implications than resting hemodynamics in children with IPAH/HPAH, except for PVRI.
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http://dx.doi.org/10.21037/cdt-20-934DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8410506PMC
August 2021

A New Solution For Stenting Large Right Ventricular Outflow Tracts Before Transcatheter Pulmonary Valve Replacement.

Can J Cardiol 2021 Sep 11. Epub 2021 Sep 11.

Centre de Référence Malformations Cardiaques Congénitales Complexes - M3C, Hôpital Universitaire Necker-Enfants malades, AP-HP, Paris, France.

Background: Pre-stenting right ventricular outflow tracts (RVOTs) before transcatheter pulmonary valve replacement (TPVR) is essential. Optimus-XXL is a new extra-large, balloon-expandable, cobalt-chrome stent with promising technologies.

Methods: From June 2020 to November 2020, 15 congenital heart patients with dysfunctional RVOTs and target TPVR diameter ≥23 mm received Optimus-XXL stents before proceeding to TPVR using Edwards SAPIEN valve. Standard safety and outcomes were prospectively assessed.

Results: Patients' median age and weight were 25.8 years (range 10.5-63.1 years) and 58 kg (range 43.8-101 kg), respectively. Underlying diagnosis was Tetralogy of Fallot (66.7%) and RVOTs were patched (80%). 15 bare-metal stents were implanted using femoral (n=14) and jugular approaches (n=1). One conduit rupture was immediately controlled with a covered Optimus-XXL. Median stent length was 43 mm (range, 33-57 mm) and median target expansion diameter was 28 mm (range, 23-30 mm). Two procedural incidents occurred during stent delivery and were percutaneously treated. Stent stability was documented during TPVRs immediately performed in 14 patients. Median stent shortening was 13.7% and median percentage of intended stent expansion was 95.9%. There was no stent fracture on the short term follow-up (median, 4.5 months).

Conclusions: We report the first implantations of Optimus-XXL stents in dysfunctional RVOTs with excellent preliminary results. Optimus-XXL should be considered as a valuable adjunct in the armamentarium for routine and complex TPVR procedures.
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http://dx.doi.org/10.1016/j.cjca.2021.08.021DOI Listing
September 2021

Transcatheter closure of extracardiac Fontan conduit fenestration using new promising materials.

J Card Surg 2021 Aug 25. Epub 2021 Aug 25.

M3C-Necker, Hôpital Universitaire Necker-Enfants malades, Assistance Publique-Hôpitaux de Paris (AP-HP), Paris, France.

Fenestration transcatheter closure is widely considered to eliminate persistent right-to-left shunt after Fontan surgeries. Three consecutive children with stage III-palliated univentricular hearts recently underwent successful endovascular fenestration closure using 43 mm/Large Optimus-CVS™ that were implanted using the new Altosa-XL™ PTA balloon catheters (AndraTec GmbH). The procedure was fast with no complication and patients were discharged the following day with complete shunt closure. One-month follow-up confirmed excellent outcomes. This report aims to highlight and discuss the competitive advantages of these promising new materials in this particular intervention.
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http://dx.doi.org/10.1111/jocs.15916DOI Listing
August 2021

A monocyte/dendritic cell molecular signature of SARS-CoV-2-related multisystem inflammatory syndrome in children with severe myocarditis.

Med (N Y) 2021 Sep 14;2(9):1072-1092.e7. Epub 2021 Aug 14.

Sorbonne Université, UMS037, PASS, Plateforme de Cytométrie de la Pitié-Salpêtrière CyPS, 75013 Paris, France.

Background: Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection in children is generally milder than in adults, but a proportion of cases result in hyperinflammatory conditions often including myocarditis.

Methods: To better understand these cases, we applied a multiparametric approach to the study of blood cells of 56 children hospitalized with suspicion of SARS-CoV-2 infection. Plasma cytokine and chemokine levels and blood cellular composition were measured, alongside gene expression at the bulk and single-cell levels.

Findings: The most severe forms of multisystem inflammatory syndrome in children (MIS-C) related to SARS-CoV-2 that resulted in myocarditis were characterized by elevated levels of pro-angiogenesis cytokines and several chemokines. Single-cell transcriptomics analyses identified a unique monocyte/dendritic cell gene signature that correlated with the occurrence of severe myocarditis characterized by sustained nuclear factor κB (NF-κB) activity and tumor necrosis factor alpha (TNF-α) signaling and associated with decreased gene expression of NF-κB inhibitors. We also found a weak response to type I and type II interferons, hyperinflammation, and response to oxidative stress related to increased HIF-1α and Vascular endothelial growth factor (VEGF) signaling.

Conclusions: These results provide potential for a better understanding of disease pathophysiology.

Funding: Agence National de la Recherche (Institut Hospitalo-Universitaire Imagine, grant ANR-10-IAHU-01; Recherche Hospitalo-Universitaire, grant ANR-18-RHUS-0010; Laboratoire d'Excellence ''Milieu Intérieur," grant ANR-10-LABX-69-01; ANR-flash Covid19 "AIROCovid" and "CoVarImm"), Institut National de la Santé et de la Recherche Médicale (INSERM), and the "URGENCE COVID-19" fundraising campaign of Institut Pasteur.
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http://dx.doi.org/10.1016/j.medj.2021.08.002DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8363470PMC
September 2021

Exposure to low-dose ionising radiation from cardiac catheterisation and risk of cancer: the COCCINELLE study cohort profile.

BMJ Open 2021 08 3;11(8):e048576. Epub 2021 Aug 3.

PSE-SANTE/SESANE/Laboratory of Epidemiology, Institute for Radiological Protection and Nuclear Safety (IRSN), Fontenay-aux-Roses, Île-de-France, France.

Purpose: The COCCINELLE study is a nationwide retrospective French cohort set up to evaluate the risk of cancer in patients who undergone cardiac catheterisation (CC) procedures for diagnosis or treatment of congenital heart disease during childhood.

Participants: Children who undergone CC procedures from 1 January 2000 to 31 December 2013, before the age of 16 in one of the 15 paediatric cardiology departments which perform paediatric CC in mainland France were included. The follow-up started at the date of the first recorded CC procedure until the exit date, that is, the date of death, the date of first cancer diagnosis, the date of the 18th birthday or the 31 December 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.

Findings To Date: 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. Standardised incidence ratios (SIRs) were increased for all-cancer (SIR=3.8, 95% CI: 2.9 to 4.9), leukaemia (SIR=3.3, 95% CI: 2.0 to 5.4), lymphoma (SIR=14.9, 95% CI: 9.9 to 22.5) and solid cancers excluding central nervous system (CNS) tumours (SIR=3.3, 95% CI: 2.0 to 5.5) compared with the general population.

Future Plans: Dose reconstruction is currently underway to estimate individual cumulative doses absorbed to relevant organs, including red bone marrow and brain for respectively haematologic disorders and CNS tumours risk estimation. A dose-response analysis will be conducted with consideration to confounding factors such as age at exposure, gender, predisposing factors to cancer and other sources of medical diagnostic low-dose ionising radiation.
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http://dx.doi.org/10.1136/bmjopen-2020-048576DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8336117PMC
August 2021

Pulmonary-to-Systemic Arterial Shunt to Treat Children With Severe Pulmonary Hypertension.

J Am Coll Cardiol 2021 Aug;78(5):468-477

Congenital and Pediatric Cardiology Unit, Hospital Necker-Enfants Malades, APHP, Universitaire de Paris, Paris, France.

Background: The placement of a pulmonary-to-systemic arterial shunt in children with severe pulmonary hypertension (PH) has been demonstrated, in relatively small studies, to be an effective palliation for their disease.

Objectives: The aim of this study was to expand upon these earlier findings using an international registry for children with PH who have undergone a shunt procedure.

Methods: Retrospective data were obtained from 110 children with PH who underwent a shunt procedure collected from 13 institutions in Europe and the United States.

Results: Seventeen children died in-hospital postprocedure (15%). Of the 93 children successfully discharged home, 18 subsequently died or underwent lung transplantation (20%); the mean follow-up was 3.1 years (range: 25 days to 17 years). The overall 1- and 5-year freedom from death or transplant rates were 77% and 58%, respectively, and 92% and 68% for those discharged home, respectively. Children discharged home had significantly improved World Health Organization functional class (P < 0.001), 6-minute walk distances (P = 0.047) and lower brain natriuretic peptide levels (P < 0.001). Postprocedure, 59% of children were weaned completely from their prostacyclin infusion (P < 0.001). Preprocedural risk factors for dying in-hospital postprocedure included intensive care unit admission (hazard ratio [HR]: 3.2; P = 0.02), mechanical ventilation (HR: 8.3; P < 0.001) and extracorporeal membrane oxygenation (HR: 10.7; P < 0.001).

Conclusions: A pulmonary-to-systemic arterial shunt can provide a child with severe PH significant clinical improvement that is both durable and potentially free from continuous prostacyclin infusion. Five-year survival is comparable to children undergoing lung transplantation for PH. Children with severely decompensated disease requiring aggressive intensive care are not good candidates for the shunt procedure.
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http://dx.doi.org/10.1016/j.jacc.2021.05.039DOI Listing
August 2021

Heterotaxy: fluctuat nec mergitur.

Cardiol Young 2021 Jul;31(7):1200-1201

The International Pediatric and Congenital Cardiac Code (IPCCC) states that visceral heterotaxy is defined as "a congenital malformation in which the internal thoraco-abdominal organs demonstrate abnormal arrangement across the left-right axis of the body. By convention, in congenital cardiology, heterotaxy syndrome does not include patients with complete mirror-imaged arrangement of the internal organs along the left-right axis also known as "total mirror imagery" or "situs inversus totalis"." [www.ipccc.net]In patients with heterotaxy, it is important to describe both the cardiac relations and the junctional connections of the cardiac segments, with documentation of the arrangement of the atrial appendages, the ventricular topology, the nature of the unions of the segments across the atrioventricular and the ventriculoarterial junctions, the infundibular morphologies, and the relationships of the arterial trunks in space. Particular attention is required for the venoatrial connections, since these are so often abnormal. The relationship and arrangement of the remaining thoraco-abdominal organs, including the lungs, the spleen, the liver, and the intestines, also must be described separately, because, although common patterns of association have been identified, there are frequent exceptions to these common patterns. Therefore, in patients with heterotaxy, it is important to describe each thoracic and abdominal organ independently.
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http://dx.doi.org/10.1017/S1047951121002961DOI Listing
July 2021

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

Health Qual Life Outcomes 2021 Jul 28;19(1):187. Epub 2021 Jul 28.

Cardiology Department of Cardiology, Regional Reference Centre for Inherited Cardiac Arrhythmia, Montpellier University Hospital, Montpellier, France.

Background: Advances in paediatric cardiology have improved the prognosis of children with inherited cardiac disorders. However, health-related quality of life (QoL) and physical activity have been scarcely analysed in children with inherited cardiac arrhythmia or inherited cardiomyopathy. Moreover, current guidelines on the eligibility of young athletes with inherited cardiac disorders for sports participation mainly rely on expert opinions and remain controversial.

Methods: The QUALIMYORYTHM trial is a multicentre observational controlled study. The main objective is to compare the QoL of children aged 6 to 17 years old with inherited cardiac arrhythmia (long QT syndrome, Brugada syndrome, catecholaminergic polymorphic ventricular tachycardia, or arrhythmogenic right ventricular dysplasia), or inherited cardiomyopathy (hypertrophic, dilated, or restrictive cardiomyopathy), to that of age and gender-matched healthy subjects. The secondary objective is to assess their QoL according to the disease's clinical and genetic characteristics, the level of physical activity and motivation for sports, the exercise capacity, and the socio-demographic data. Participants will wear a fitness tracker (ActiGraph GT3X accelerometer) for 2 weeks. A total of 214 children are required to observe a significant difference of 7 ± 15 points in the PedsQL, with a power of 90% and an alpha risk of 5%.

Discussion: After focusing on the survival in children with inherited cardiac disorders, current research is expanding to patient-reported outcomes and secondary prevention. The QUALIMYORYTHM trial intends to improve the level of evidence for future guidelines on sports eligibility in this population. Trial registration ClinicalTrials.gov Identifier: NCT04712136, registered on January 15th, 2021 ( https://clinicaltrials.gov/ct2/show/NCT04712136 ).
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http://dx.doi.org/10.1186/s12955-021-01825-6DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8317438PMC
July 2021

Late Pediatric Mechanical Thrombectomy for Embolic Stroke as Bridge Reinforcement From LVAD to Heart Transplantation.

JACC Case Rep 2021 Apr 24;3(4):686-689. Epub 2021 Feb 24.

Department of Neuroradiology, University of Paris, INSERM UMR 1266 IMA-BRAIN, University Hospital Group, GHU Paris, Paris, France.

Although the left ventricular assist device is an important bridge to heart transplantation for patients with end-stage heart failure, it can also be a source of embolic stroke. We present a case of late intracranial mechanical thrombectomy performed for embolic stroke beyond the recommended 6 h, thus allowing for heart transplantation 4 days after intracranial mechanical thrombectomy. ().
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http://dx.doi.org/10.1016/j.jaccas.2020.10.028DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8302778PMC
April 2021

Transcatheter patent arterial duct closure in premature infants: A new technique to ease access to the patent arterial duct, with particular benefit for the tricuspid valve.

Arch Cardiovasc Dis 2021 Jun-Jul;114(6-7):482-489. Epub 2021 Jul 24.

Centre de Référence Malformations Cardiaques Congénitales Complexes-M3C, Hôpital Necker Enfants Malades, 75015 Paris, France.

Background: Transcatheter patent arterial duct (PAD) closure in premature infants has been shown to be feasible. Since our early transcatheter PAD closure procedures in premature infants at Hôpital Necker Enfants Malades, we have changed our technique several times to advance the guidewire through the right heart to avoid tricuspid valve damage.

Aim: To describe the technique we have been using since May 2019, to report our results with a particular focus on tricuspid leaks and to analyse the potential mechanisms of tricuspid lesion development with previous methods.

Methods: All premature infants weighing<2kg who underwent transcatheter PAD closure with this new technique were included. Demographic data, procedural data, outcome and procedural complications were reviewed, with particular attention to the occurrence of tricuspid regurgitation.

Results: Between May 2019 and May 2020, 33 patients were included. Median gestational age was 25 weeks. Median birth weight and procedural weight were 690g (range 490-1065g; interquartile range [IQR] 620-785g) and 1160g (range 900-1900g; IQR 1030-1300g), respectively. Median age at procedure was 35 (IQR 30-46) days. PAD anatomy was evaluated on transthoracic echocardiography only. The median duct diameter was 3 (IQR 2.5-3.2) mm at the pulmonary end. Success rate was 100% (defined as successful closure without residual shunt). One patient had a renal vein thrombosis, which fully resolved with low-molecular-weight heparin anticoagulation. No tricuspid regurgitation or stenosis of the left pulmonary artery or the aorta was seen. One patient died of a superior caval vein obstruction with bilateral chylothorax related to a central catheter thrombosis 56 days after the procedure, unrelated to the catheter procedure.

Conclusion: In this prospective study, we describe a new technique to avoid tricuspid valve damage and facilitate delivery of the PAD device.
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http://dx.doi.org/10.1016/j.acvd.2021.06.002DOI Listing
September 2021

A retrospective analysis of discordances between international normalized ratio (INR) self-testing and INR laboratory testing in a pediatric patient population.

Int J Lab Hematol 2021 Jul 8. Epub 2021 Jul 8.

Hematology Laboratory, Hôpital universitaire Necker-Enfants Malades, AP-HP, Paris, France.

Introduction: The lack of quality control procedures for home point-of-care (POC) international normalized ratio (INR) devices is a concern. Concomitant laboratory and POC INR testing may be proposed to overcome the lack of quality control. However, a difference between the POC INR and the laboratory INR is not necessarily due to failure of the POC device. This study aimed to identify variables associated with a significant deviation between the POC INR and the laboratory INR.

Methods: Children included in this retrospective cohort study performed at least one concomitant laboratory and POC INRs. Clinical and laboratory variables were assessed for an association with significant deviation within pairs of INR.

Results: A significant deviation was noted for 30 (15.3%) of the 196 pairs of INR measurements from 124 children. Relative to patients without deviations, patients with deviations were younger (odds ratio =0.91; P = .020), less experienced in the use of POC INR devices (odds ratio =0.89; P = .098), and more likely to have received an INR result from a laboratory using animal thromboplastin (odds ratio =2.81 vs. 0.37 for laboratories using human thromboplastin; P = .016). In a multivariate analysis, younger age and the laboratory's use of animal thromboplastin were associated with significant deviations.

Conclusions: Although most children had coherent pairs of INR values, the occurrence of deviations raises the question of the origin of the thromboplastin used in the laboratory and emphasizes the need to provide specific quality control procedures for POC INR devices.
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http://dx.doi.org/10.1111/ijlh.13652DOI Listing
July 2021

Efficacy and safety of tadalafil in a pediatric population with pulmonary arterial hypertension: phase 3 randomized, double-blind placebo-controlled study.

Pulm Circ 2021 Jul-Sep;11(3):20458940211024955. Epub 2021 Jun 23.

Eli Lilly and Company, Indianapolis, IN, USA.

This study evaluated the efficacy and safety of tadalafil in pediatric patients with pulmonary arterial hypertension. This phase-3, international, randomized, multicenter (24 weeks double-blind placebo-controlled period; two-year, open-labeled extension period), add-on (patient's current endothelin receptor antagonist therapy) study included pediatric patients aged <18 years with pulmonary arterial hypertension. Patients received tadalafil 20 mg or 40 mg based on their weight (heavy-weight: ≥40 kg; middle-weight: ≥25 to <40 kg) or placebo orally once daily for 24 weeks. Primary endpoint was change from baseline in six-minute walk distance in patients aged ≥6 years at Week 24. Sample size was amended from 134 to ≥34 patients, due to serious recruitment challenges. Therefore, statistical significance testing was not performed between treatment groups. Results showed that patient demographics and baseline characteristics ( = 35; tadalafil = 17; placebo = 18) were comparable between treatment groups; median age was 14.2 years (6.2-17.9 years) and majority (71.4%,  = 25) of patients were in the heavy-weight cohort. Least square mean (standard error) changes from baseline in six-minute walk distance at Week 24 was numerically greater with tadalafil versus placebo (60.48 (20.41) vs 36.60 (20.78) meters; placebo-adjusted mean difference (standard deviation) 23.88 (29.11)). Safety of tadalafil treatment was as expected without any new safety concerns. During study Period 1, two patients (one in each group) discontinued due to investigator's reported clinical worsening, and no deaths were reported. In conclusion, the statistical significance testing was not performed between the treatment groups due to low sample size; however, the study results show positive trend in improvement in non-invasive measurements, commonly utilized by clinicians to evaluate the disease status for children with pulmonary arterial hypertension. Safety of tadalafil treatment was as expected without any new safety signals.
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http://dx.doi.org/10.1177/20458940211024955DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8226239PMC
June 2021

Echocardiographic Changes and Long-Term Clinical Outcomes in Pediatric Patients With Pulmonary Arterial Hypertension Treated With Bosentan for 72 Weeks: A Analysis From the FUTURE 3 Study.

Front Pediatr 2021 16;9:681538. Epub 2021 Jun 16.

Department of Pediatric Cardiology, Children's Hospital Colorado, Denver, CO, United States.

FormUlation of bosenTan in pUlmonary arterial hypeRtEnsion (FUTURE) 3 was a 24-week open-label, prospective, and randomized phase 3 study that assessed the pharmacokinetics of bosentan 2 mg/kg b.i.d. or t.i.d. in children with pulmonary arterial hypertension (PAH). We report findings from a analysis that explored the prognostic value of echocardiographic changes during FUTURE 3 in relation to clinical outcomes observed during the 24-week core study and 48-week extension. Patients aged ≥3 months to <12 years ( = 64) received oral doses of bosentan 2 mg/kg b.i.d. or t.i.d. (1:1) for 24 weeks, after which they were eligible to enter the extension with continued bosentan administration. Echocardiographic evaluations were performed at baseline, Week 12, and 24 of the core study via central reading, and analyzed for correlation with clinical outcomes (time to PAH worsening, time to death, and vital status). Sixty-four patients were randomized in the core study [median (IQR) age 3.8 (1.7-7.8) years]; and 58 patients (90.6%) entered the 48-week extension. Most of the patients (68.8%) were receiving ≥1 PAH medication at baseline. Echocardiographic changes during the core study were small but with high variability. There were statistically significant associations at Week 24 between worsening of the parameters, systolic left ventricular eccentricity index (LVEIS) and E/A ratio mitral valve flow, and the outcomes of time to death and time to PAH worsening. Additional studies that utilize simple and reproducible echocardiographic assessments are needed to confirm these findings and subsequently identify potential treatment goals in pediatric PAH.
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http://dx.doi.org/10.3389/fped.2021.681538DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8242164PMC
June 2021

Long-Term Neurodevelopmental Outcomes of Children with Congenital Heart Defects.

J Pediatr 2021 Jun 19. Epub 2021 Jun 19.

Université de Paris, CRESS, INSERM, INRA, F-75004, Paris, France.

Objective: To assess whether children with symptomatic congenital heart defects (CHDs) at birth (cyanosis and/or heart failure) are at greater risk of adverse neurodevelopmental outcomes at 8 years of age.

Study Design: From a prospective population-based cohort study of newborns with CHDs (EPICARD), we included 473 children with available neurodevelopmental assessments at 8 years of age. We grouped the CHD based on symptoms at birth and need for early neonatal intervention. Ventricular septal defects that closed spontaneously within the first year of life were considered the control group. Neurodevelopmental outcomes were assessed using the Kauffman Assessment Battery Test for Children, Second Edition, for IQ (mean 100 ± 15), and the Developmental NEuroPSYchological Assessment Battery, Second Edition, for detailed assessment of specific neurocognitive domains (mean 10 ± 3). Multivariable regression analysis was used to compare the outcomes across the CHD groups after considering potentially confounding variables.

Results: Compared with the control group, children with cyanotic CHD without heart failure had lower scores for IQ, -7.2 (95% CI -13.4 to -1.2). Children with noncyanotic CHD with heart failure had lower scores in the specific domains of language -1.5 (95% CI -2.2 to -0.7), and memory and learning -1.3 (95% CI -2.4; -0.3). Those with both cyanotic CHD and heart failure had lower scores for IQ, -7.6 (95% CI -13.5 to -1.8), as well as the specific domains of language and memory and learning, -2.0 (95% CI -2.9 to -1.0) and -1.1 (95% CI -2.3 to -0.1), respectively.

Conclusions: Children with symptomatic CHD at birth are at greater risk of adverse neurodevelopmental outcomes at 8 years of age, with the greatest risk for those who were born with both cyanosis and heart failure.
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http://dx.doi.org/10.1016/j.jpeds.2021.06.032DOI Listing
June 2021

Prevalence of Growth Restriction at Birth for Newborns With Congenital Heart Defects: A Population-Based Prospective Cohort Study EPICARD.

Front Pediatr 2021 28;9:676994. Epub 2021 May 28.

Université de Paris, CRESS, INSERM, INRA, Paris, France.

Congenital heart defects (CHD) and growth restriction at birth are two major causes of childhood and adult morbidity and mortality. The aim of this study was to assess the overall risk of growth restriction at birth, as measured by its imperfect proxy small (< 10th percentile) for gestational age (SGA), for newborns with CHD. Using data from a population-based cohort of children born with CHD, we assessed the risk of growth restriction at birth using SGA and severe SGA (3rd percentile). To compare the odds of SGA and severe SGA across five specific major CHD, we used ordinal logistic regression using isolated, minor (non-operated) ventricular septal defect (VSD) as the control group. The overall proportion of SGA for "isolated" CHD (i.e., those not associated with other anomalies) was 13% (95% CI, 12-15%), which is 30% higher than what would be expected in the general population (i.e., 10%). The risk of severe SGA was 5% (95% CI, 4-6%) as compared with the expected 3% in the general population. There were substantial differences in the risk of overall SGA and more so severe SGA across the different CHD. The highest risk of SGA occurred for Tetralogy of Fallot (adjusted OR 2.7, 95% CI, 1.3-5.8) and operated VSD (adjusted OR 2.1, 95% CI, 1.1-3.8) as compared with the control group of minor (non-operated) VSD. The overall risks of both SGA and severe SGA were higher in isolated CHD than what would be expected in the general population with substantial differences across the subtypes of CHD. These results may provide a clue for understanding the underlying mechanisms of the relation between alterations in fetal circulation associated with different types of CHD and their effects on fetal growth.
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http://dx.doi.org/10.3389/fped.2021.676994DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8192794PMC
May 2021

Outcomes of sustained fetal tachyarrhythmias after transplacental treatment.

Heart Rhythm O2 2021 Apr 9;2(2):160-167. Epub 2021 Mar 9.

Obstetric and Maternal Fetal Medicine and EA7328.

Background: Fetal tachyarrhythmia is a condition that may lead to cardiac dysfunction, hydrops, and death. Despite a transplacental treatment, failure to obtain or maintain sinus rhythm may occur.

Objective: We aimed to analyze the perinatal outcomes of sustained fetal tachyarrhythmias after in utero treatment.

Methods: We performed a retrospective evaluation of 69 cases with sustained fetal tachyarrhythmia. We compared the perinatal and long-term outcomes of prenatally converted and drug-resistant fetuses. Tachyarrhythmia subtypes were also evaluated.

Results: Conversion to sinus rhythm was obtained in 74% of cases; 26% of cases were drug-resistant and delivered arrhythmic. Three perinatal deaths occurred in both groups (6.7% vs 17%, = .34). Neonates delivered arrhythmic were more frequently admitted to neonatal intensive care units (75% vs 31%, < .01), and their hospital stay was longer (20.9 vs 6.64 days, < .001). Multiple neonatal recurrences (81% vs 11%, < .001), temporary hemodynamic dysfunction or heart failure (50% vs 6.7%, < .001), and postnatal use of a combination treatment (44% vs 13%,  = .028) were also more frequent in this population. Beyond the neonatal period, rates of recurrences within the first 16 months were higher in drug-resistant fetuses (HR = 16.14, CI 95% [4.485; 193.8], < .001). In this population, postnatal electrocardiogram revealed an overrepresentation of rare mechanisms, especially permanent junctional reciprocating tachycardia (PJRT) (31%).

Conclusion: Prenatal conversion to stable sinus rhythm is a major determinant of perinatal and long-term outcomes in fetal tachyarrhythmias. The underlying electrophysiological mechanisms have a major role in predicting these differential outcomes with an overrepresentation of PJRT in the drug-resistant population.
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http://dx.doi.org/10.1016/j.hroo.2021.02.006DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8183966PMC
April 2021

Abnormal origin of the left pulmonary artery from the descending aorta and heterotaxy syndrome: an undescribed phenotypic association.

Cardiol Young 2021 Jul 4;31(7):1193-1196. Epub 2021 Jun 4.

M3C-Necker, Hôpital Necker-Enfants Malades, AP-HP, Paris, France.

Extensive screening in a newborn with prenatal suspicion of VACTERL syndrome identified an anomalous origin of the left pulmonary artery from the descending aorta with an arterial duct and left aortic arch, and normal intra-cardiac anatomy. Other anatomical anomalies suggested heterotaxy syndrome. At one-month-old, re-implantation of the 3.5 mm left pulmonary artery was performed by direct tension-low anastomosis. Post-operative course was complicated by severe left pulmonary atelectasis, and the patient died 20 days later.
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http://dx.doi.org/10.1017/S1047951121002183DOI Listing
July 2021

Endothelial Dysfunction as a Component of Severe Acute Respiratory Syndrome Coronavirus 2-Related Multisystem Inflammatory Syndrome in Children With Shock.

Crit Care Med 2021 Jun 1. Epub 2021 Jun 1.

Department of Biological Hematology, Necker Hospital, AP-HP Centre-Universiteé de Paris, Paris, France. HITh, UMR_S 1176, INSERM, Univ. Paris-Saclay, Le Kremlin-Bicêtre, France. Emergency Department, Georges Pompidou European Hospital, AP-HP Centre Universiteé de Paris, Paris, France. Universiteé de Paris, PARCC, INSERM, Paris, France. Pediatric Intensive Care Unit, Necker Hospital, AP-HP Centre Universiteé de Paris, Paris, France. Hematology Department and Biosurgical Research Lab (Carpentier Foundation), Georges Pompidou European Hospital, AP-HP Centre Universiteé de Paris, Paris, France. Universiteé de Paris, Innovative Therapies in Hemostasis, INSERM, Paris, France. Universiteé de Paris, EA7323, Paris, France. M3C-Necker, Congenital and Pediatric Cardiology, Necker Hospital, AP-HP Centre-Universiteé de Paris, Paris, France. Department of General Pediatrics and Pediatric Infectious Diseases, Necker Hospital, AP-HP Centre-Universiteé de Paris, Paris, France. Pediatric Emergency Department, Necker Hospital, AP-HP Centre Universiteé de Paris, Paris, France. INSERM, Centre de Recherche des Cordeliers, UMRS 1138, Sorbonne Université, Université de Paris, Paris, France.

Trial Registration: NCT04420468.

Objectives: Severe acute respiratory syndrome coronavirus 2-related multisystem inflammatory syndrome in children is frequently associated with shock; endothelial involvement may be one of the underlying mechanisms. We sought to describe endothelial dysfunction during multisystem inflammatory syndrome in children with shock and then assess the relationship between the degree of endothelial involvement and the severity of shock.

Design: Observational study.

Setting: A PICU in a tertiary hospital.

Patients: Patients aged under 18 (n = 28) with severe acute respiratory syndrome coronavirus 2-related multisystem inflammatory syndrome in children and shock, according to the Centers for Disease Control and Prevention criteria.

Interventions: None.

Measurements And Main Results: Correlations between endothelial marker levels and shock severity were assessed using Spearman coefficient. The median (interquartile range) age was 9 years (7.5-11.2 yr). Sixteen children presented with cardiogenic and distributive shock, 10 presented with cardiogenic shock only, and two presented with distributive shock only. The median left ventricular ejection fraction, troponin level, and lactate level were, respectively, 40% (35-45%), 261 ng/mL (131-390 ng/mL), and 3.2 mmol/L (2-4.2 mmol/L). Twenty-five children received inotropes and/or vasopressors; the median Vasoactive and Inotropic Score was 8 (5-28). Plasma levels of angiopoietin-2 (6,426 pg/mL [2,814-11,836 pg/mL]), sE-selectin (130,405 pg/mL [92,987-192,499 pg/mL]), von Willebrand factor antigen (344% [288-378%]), and the angiopoietin-2/angiopoietin-1 ratio (1.111 [0.472-1.524]) were elevated and significantly correlated with the Vasoactive and Inotropic Score (r = 0.45, p = 0.016; r = 0.53, p = 0.04; r = 0.46, p = 0.013; and r = 0.46, p = 0.012, respectively).

Conclusions: Endothelial dysfunction is associated with severe acute respiratory syndrome coronavirus 2-related multisystem inflammatory syndrome in children with shock and may constitute one of the underlying mechanisms.
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http://dx.doi.org/10.1097/CCM.0000000000005093DOI Listing
June 2021

Continuous positive airway pressure improves work of breathing in pediatric chronic heart failure.

Sleep Med 2021 07 19;83:99-105. Epub 2021 Apr 19.

Pediatric Noninvasive Ventilation and Sleep Unit, Hôpital Necker-Enfants Malades F-75015, Paris, France; Université de Paris, VIFASOM F-75004, Paris, France.

Background: Sleep disordered breathing (SDB) is common in adults with chronic heart failure (CHF), but its prevalence in children remains unclear. Continuous positive airway pressure (CPAP) is the treatment of SDB but deleterious hemodynamic effects have been reported.

Methods: We prospectively analyzed SDB in children with CHF and the effect of CPAP on work of breathing (WOB) and cardiac index (CI). Children aged 6 months to 18 years old with CHF due to: 1) dilated cardiomyopathy (DM) with an ejection fraction < 45%, 2) functional single ventricle (SV) or 3) aortic or mitral valve disease awaiting surgery (VD) were eligible for the study. A polysomnography (PSG), measurement of WOB and CI during spontaneous breathing (SB) and CPAP (6, 8 and 10 cmHO) were performed.

Results: Thirty patients with mean age of 6.4 ± 5 years were included (16 DM 16, 10 SV, 4 LV). Twenty (73%) patients had a normal sleep efficiency. Median apnoeas hypopnea index (IAH) was within normal range at 1.6 events/h (0, 14) events/hour. Only one patient had central sleep apnoeas, none had Cheyne-Stokes respiration, and 3 patients had an obstructive AHI between 5 and 10 events/hour. Optimal CPAP level decreased WOB (p = 0.05) and respiratory rate (p = 0.01).

Conclusions: Severe SDB was uncommon in children with CHF. However, CPAP may be beneficial by decreasing WOB and respiratory rate without deleterious effects on CI.
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http://dx.doi.org/10.1016/j.sleep.2021.04.003DOI Listing
July 2021

Lung and heart-lung transplantation for children with PAH: Dramatic benefits from the implementation of a high-priority allocation program in France.

J Heart Lung Transplant 2021 07 26;40(7):652-661. Epub 2021 Mar 26.

Service de Chirurgie Thoracique, Vasculaire et Transplantation Cardio-pulmonaire, Groupe hospitalier Marie-Lannelongue -Saint Joseph, Le Plessis-Robinson, France; Université Paris-Saclay, Le Kremlin Bicêtre, France; UMR_S 999, Université Paris-Sud, INSERM, Groupe hospitalier Marie-Lannelongue -Saint Joseph, Le Plessis-Robinson, France.

Purpose: Pulmonary arterial hypertension (PAH) is rare but remains fatal in infants and children despite the advance of targeted therapies. Lung transplantation (LTx), first performed in pediatric patients in the 1980s, is, with the Potts shunt, the only potentially life-extending option in patients with end-stage PAH but is possible only in tightly selected patients. Size-matching challenges severely restrict the donor organ pool, resulting-together with peculiarities of PAH in infants-in high waitlist mortality. We aimed to investigate survival when using a high-priority allocation program (HPAP) in children with PAH listed for double-LTx or heart-LTx.

Methods: We conducted a single-center, retrospective, before-after study of consecutive children with severe Group 1 PAH listed for double-LTx or heart-LTx between 1988 and 2019. The HPAP was implemented in France in 2006 and 2007 for heart-LTx and double-LTx, respectively.

Results: Fifty-five children with PAH were listed for transplantation. Mean age at transplantation was 15.8±2.8 years and 72% had heart-lung transplantation. PAH was usually idiopathic (65%) or due to congenital heart disease (25%). HPAP implementation resulted in the following significant benefits: Decreased cumulative incidence of waitlist death within 1 and 2 years (p < 0.0001); increased cumulative incidence of transplantation within 6 months, from 44% to 67% (p < 0.01); and improved survival after listing (at 1, 3, and 5 years: 61%, 50%, and 44% vs. 92%, 84%, and 72% before and after HPAP implementation, respectively; p = 0.02).

Conclusion: HPAP implementation was associated with significant improvements in access to transplantation and in survival after listing in children with end-stage PAH.
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http://dx.doi.org/10.1016/j.healun.2021.03.013DOI Listing
July 2021

Catheter ablation in adults with congenital heart disease: A 15-year perspective from a tertiary centre.

Arch Cardiovasc Dis 2021 Jun-Jul;114(6-7):455-464. Epub 2021 Apr 10.

Adult Congenital Heart Disease Medico-Surgical Unit, Georges Pompidou European Hospital, 75015 Paris, France; Paediatric and Congenital Heart Disease Department, Necker Hospital, 75015 Paris, France.

Background: With the growing adult congenital heart disease (ACHD) population, the number of catheter ablation procedures is expected to dramatically increase. Data reporting experience and evolution of catheter ablation in patients with ACHD, over a significant period of time, remain scarce.

Aim: We aimed to describe temporal trends in volume and outcomes of catheter ablation in patients with ACHD.

Methods: This was a retrospective observational study including all consecutive patients with ACHD undergoing attempted catheter ablation in a large tertiary referral centre over a 15-year period. Acute procedural success rate and freedom from recurrence at 12 and 24 months were analysed.

Results: From November 2004 to November 2019, 302 catheter ablations were performed in 221 patients with ACHD (mean age 43.6±15.0 years; 58.9% male sex). The annual number of catheter ablations increased progressively from four to 60 cases per year (P<0.001). Intra-atrial reentrant tachycardia/focal atrial tachycardia was the most common arrhythmia (n=217, 71.9%). Over the study period, acute procedural success rate increased from 45.0% to 93.4% (P<0.001). Use of irrigated catheters (odds ratio [OR] 4.03, 95% confidence interval [CI] 1.86-8.55), a three-dimensional mapping system (OR 3.70, 95% CI 1.72-7.74), contact force catheters (OR 3.60, 95% CI 1.81-7.38) and high-density mapping (OR 3.69, 95% CI 1.82-8.14) were associated with acute procedural success. The rate of freedom from any recurrence at 12 months increased from 29.4% to 66.2% (P=0.001). Seven (2.3%) non-fatal complications occurred.

Conclusions: The number of catheter ablation procedures in patients with ACHD has increased considerably over the past 15 years. Growing experience and advances in ablative technologies appear to be associated with a significant improvement in acute and mid-term outcomes.
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http://dx.doi.org/10.1016/j.acvd.2020.12.005DOI Listing
September 2021

Transplantation for pulmonary arterial hypertension with congenital heart disease: Impact on outcomes of the current therapeutic approach including a high-priority allocation program.

Am J Transplant 2021 Apr 12. Epub 2021 Apr 12.

UMR-S 999, Inserm, Hôpital Marie Lannelongue, Faculté de Médecine Paris-Saclay, Université Paris-Saclay, Plessis-Robinson, France.

Patients with end-stage pulmonary arterial hypertension due to congenital heart disease have limited access to heart-lung transplantation or double-lung transplantation. We aimed to assess the effects of a high-priority allocation program established in France in 2007. We conducted a retrospective study to compare waitlist and posttransplantation outcomes before versus after implementation of the high-priority allocation program. We included 67 consecutive patients (mean age at listing, 33.2 ± 10.5 years) with pulmonary arterial hypertension due to congenital heart disease listed for heart-lung transplantation or double-lung transplantation from 1997 to 2016. At one month, the incidences of transplantation and death before transplantation were 3.5% and 24.6% in 1997-2006, 4.8% and 4.9% for patients on the regular list in 2007-2016, and 41.2% and 7.4% for patients listed under the high-priority allocation program (p < .001 and p = .0001, respectively). Overall survival was higher in patients listed in 2007-2016 (84.2% and 61.2% at 1 and 10 years vs. 36.8% and 22.1%, p = .0001). Increased incidence of transplantation, decreased waiting list mortality, and improved early and long-term outcomes were observed in patients with pulmonary arterial hypertension due to congenital heart disease listed for transplantation in the recent era, characterized by implementation of a high-priority allocation program.
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http://dx.doi.org/10.1111/ajt.16600DOI Listing
April 2021

Preliminary Experience With the New Amplatzer™ Trevisio™ Delivery System in Transcatheter Atrial Septal Defect Closures in Children.

Front Pediatr 2021 11;9:641742. Epub 2021 Mar 11.

M3C-Necker, Hôpital Universitaire Necker-Enfants Malades, Assistance Publique-Hôpitaux de Paris (AP-HP), Paris, France.

To evaluate safety, efficacy, and technical advantages of Amplatzer™ Trevisio™ intravascular delivery system (ATIDS) in percutaneous atrial septal defect (ASD) closure in children. The Trevisio™ is a novel delivery system designed for accurate and facilitated implantation of Amplatzer™ devices. There are no published clinical reports so far. During September 2020, 9 children with anatomically challenging ASDs underwent attempted transcatheter closure using ATIDS to deliver Amplatzer™ Septal occluders (ASO). All interventions were performed under general anesthesia, trans-esophageal echocardiography (TOE), and fluoroscopic guidance. Standard safety, immediate, and 60-days outcomes were prospectively assessed. The median age was 8.1 (5.1-16.9) years and the median bodyweight was 30 (18-63) kg. Six patients had isolated secundum-type ASDs with absent anterosuperior rims including one with an aneurysmal septum. Three patients had unclassical defects associated with complex congenital heart anomalies. Eight devices were delivered from the femoral vein and the jugular vein was accessed in one patient with interrupted inferior caval vein and azygos continuation. All implantations were successful. The shape, position, and orientation of the ASO were identical before and after release on TOE and fluoroscopy. There was no device embolization or serious complication following closure. Complete shunt closure was confirmed on follow-up. We report the first clinical experience with ATIDS in transcatheter ASD pediatric closures. Safety and efficacy were witnessed in our case-series. The major advantage of reduced-tension deployment and reliable precision in device positioning is highly beneficial in challenging anatomies and unusual access.
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http://dx.doi.org/10.3389/fped.2021.641742DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8006412PMC
March 2021

Association between prophylactic angiotensin-converting enzyme inhibitors and overall survival in Duchenne muscular dystrophy-analysis of registry data.

Eur Heart J 2021 05;42(20):1976-1984

Service d'Explorations Fonctionnelles, Hôpital Raymond Poincaré, Garches, FranceINSERM Université de Versailles Saint Quentin en Yvelines, France.

Aims: To estimate the effect of prophylactic angiotensin-converting enzyme inhibitors (ACEi) on survival in Duchenne muscular dystrophy (DMD).

Methods And Results: We analysed the data from the French multicentre DMD Heart Registry (ClinicalTrials.gov: NCT03443115). We estimated the association between the prophylactic prescription of ACEi and event-free survival in 668 patients aged 8 to 13 years, with normal left ventricular function, using (i) a Cox model with intervention as a time-dependent covariate, (ii) a propensity-based analysis comparing ACEi treatment vs. no treatment, and (iii) a set of sensitivity analyses. The study outcomes were overall survival and hospitalizations for heart failure (HF) or acute respiratory failure. Among the 668 patients included in the DMD Heart Registry, 576 (mean age 6.1 ± 2.8 years) were eligible for this study, of whom 390 were treated with ACEi prophylactically. Death occurred in 53 patients (13.5%) who were and 60 patients (32.3%) who were not treated prophylactically with ACEi, respectively. In a Cox model with intervention as a time-dependent variable, the hazard ratio (HR) associated with ACEi treatment was 0.49 [95% confidence interval (CI) 0.34-0.72] and 0.47 (95% CI 0.31-0.17) for overall mortality after adjustment for baseline variables. In the propensity-based analysis, 278 patients were included in the treatment group and 834 in the control group, with 18.5% and 30.4% 12-year estimated probability of death, respectively. ACEi were associated with a lower risk of death (HR 0.39; 95% CI 0.17-0.92) and hospitalization for HF (HR 0.16; 95% CI 0.04-0.62). All other sensitivity analyses yielded similar results.

Conclusion: Prophylactic ACEi treatment in DMD was associated with a significantly higher overall survival and lower rates of hospitalization for HF.
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http://dx.doi.org/10.1093/eurheartj/ehab054DOI Listing
May 2021

Impact of aortic arch curvature in flow haemodynamics in patients with transposition of the great arteries after arterial switch operation.

Eur Heart J Cardiovasc Imaging 2021 Jan 31. Epub 2021 Jan 31.

Paediatric Cardiology, Evelina London Children's Hospital, St. Thomas' Hospital, Westminster Bridge Road, London SE1 7EH, UK.

Aims : In this study, we will describe a comprehensive haemodynamic analysis and its relationship to the dilation of the aorta in transposition of the great artery (TGA) patients post-arterial switch operation (ASO) and controls using 4D-flow magnetic resonance imaging (MRI) data.

Methods And Results : Using 4D-flow MRI data of 14 TGA young patients and 8 age-matched normal controls obtained with 1.5 T GE-MR scanner, we evaluate 3D maps of 15 different haemodynamics parameters in six regions; three of them in the aortic root and three of them in the ascending aorta (anterior-left, -right, and posterior for both cases) to find its relationship with the aortic arch curvature and root dilation. Differences between controls and patients were evaluated using Mann-Whitney U test, and the relationship with the curvature was accessed by unpaired t-test. For statistical significance, we consider a P-value of 0.05. The aortic arch curvature was significantly different between patients 46.238 ± 5.581 m-1 and controls 41.066 ± 5.323 m-1. Haemodynamic parameters as wall shear stress circumferential (WSS-C), and eccentricity (ECC), were significantly different between TGA patients and controls in both the root and ascending aorta regions. The distribution of forces along the ascending aorta is highly inhomogeneous in TGA patients. We found that the backward velocity (B-VEL), WSS-C, velocity angle (VEL-A), regurgitation fraction (RF), and ECC are highly correlated with the aortic arch curvature and root dilatation.

Conclusion : We have identified six potential biomarkers (B-VEL, WSS-C, VEL-A, RF, and ECC), which may be helpful for follow-up evaluation and early prediction of aortic root dilatation in this patient population.
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http://dx.doi.org/10.1093/ehjci/jeaa416DOI Listing
January 2021

Evolution of acute myocarditis in a pediatric population: An MRI based study.

Int J Cardiol 2021 04 25;329:226-233. Epub 2020 Dec 25.

Unité médico-chirurgicale de cardiologie congénitale et pédiatrique, centre de référence des maladies cardiaques congénitales complexes - M3C, Hôpital universitaire Necker-Enfants Malades, Université de Paris, France and Laboratory of Embriology and Genetic Malformation, INSERM UMR 1163, Imagine Institute, F-75015 Paris, France.

Background: Cardiac Magnetic Resonance (CMR) data regarding myocarditis presentation and disease course is still lacking in pediatric patients. We evaluate baseline CMR and evolution of functional and tissue abnormalities in children with acute myocarditis.

Methods: CMR was performed in 125 patients with clinical diagnosis of acute myocarditis. Clinical follow-up was performed for a median of 498 (214-923) days.

Results: LVEF was depressed (<55%) in 56 cases (45%) upon baseline CMR. LGE was found in 93 patients (77%) of cases. LGE was exclusively subepicardial in 29 patients (23%), while other LGE patterns (midwall/mixed) were present in 64 (51%). CMR was repeated in 92 (74%) patients. 67% presented recover of function at a median of 170 (70-746) days after onset of symptoms. Midwall/mixed LGE pattern had a statistically significant correlation with absent recover of function (OR 0.20 p 0.036). Thirteen patients (16%) had recovery from LV dysfunction but with persistence of LGE. Sub-epicardial pattern of LGE (OR 3.33, 95% CI 1.08-10.2, p = 0.036) and the presence of fever at admission (OR 4.67, 95% CI 1.16-18.7, p = 0.03) were associated with a significantly higher likelihood of complete normalization while midwall/mixed LGE pattern was associated with non-recovery.

Conclusions: In pediatric myocarditis, midwall/mixed LGE pattern is associated with absent recover of function. Patients with recover of function may still have persistence of LGE, while a complete recovery from functional and tissue abnormalities is found only in a third of patients. Midwall/mixed pattern of LGE at first MRI was associated to worse outcome.
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http://dx.doi.org/10.1016/j.ijcard.2020.12.052DOI Listing
April 2021

Safety and efficacy of rivaroxaban in pediatric cerebral venous thrombosis (EINSTEIN-Jr CVT).

Blood Adv 2020 12;4(24):6250-6258

Bayer AG, Wuppertal, Germany.

Anticoagulant treatment of pediatric cerebral venous thrombosis has not been evaluated in randomized trials. We evaluated the safety and efficacy of rivaroxaban and standard anticoagulants in the predefined subgroup of children with cerebral venous thrombosis (CVT) who participated in the EINSTEIN-Jr trial. Children with CVT were randomized (2:1), after initial heparinization, to treatment with rivaroxaban or standard anticoagulants (continued on heparin or switched to vitamin K antagonist). The main treatment period was 3 months. The primary efficacy outcome, symptomatic recurrent venous thromboembolism (VTE), and principal safety outcome, major or clinically relevant nonmajor bleeding,were centrally evaluated by blinded investigators. Sinus recanalization on repeat brain imaging was a secondary outcome. Statistical analyses were exploratory. In total, 114 children with confirmed CVT were randomized. All children completed the follow-up. None of the 73 rivaroxaban recipients and 1 (2.4%; CVT) of the 41 standard anticoagulant recipients had symptomatic, recurrent VTE after 3 months (absolute difference, 2.4%; 95% confidence interval [CI], -2.6% to 13.5%). Clinically relevant bleeding occurred in 5 (6.8%; all nonmajor and noncerebral) rivaroxaban recipients and in 1 (2.5%; major [subdural] bleeding) standard anticoagulant recipient (absolute difference, 4.4%; 95% CI, -6.7% to 13.4%). Complete or partial sinus recanalization occurred in 18 (25%) and 39 (53%) rivaroxaban recipients and in 6 (15%) and 24 (59%) standard anticoagulant recipients, respectively. In summary, in this substudy of a randomized trial with a limited sample size, children with CVT treated with rivaroxaban or standard anticoagulation had a low risk of recurrent VTE and clinically relevant bleeding. This trial was registered at clinicaltrials.gov as #NCT02234843.
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http://dx.doi.org/10.1182/bloodadvances.2020003244DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7756994PMC
December 2020

Prenatal diagnosis of anomalous connection of the inferior caval vein to the left atrium associated with common arterial trunk.

J Anat 2021 05 20;238(5):1255-1258. Epub 2020 Dec 20.

M3C-Necker Enfants malades, AP-HP, Paris, France.

Anomalous connection of the inferior caval vein to the left atrium is exceedingly rare, and has even been considered by some authors an anatomic and embryologic impossibility. This study demonstrates for the first time the existence of this rare malformation, diagnosed on prenatal echo, and confirmed on post-mortem examination in a 24 WG fetus, in association with a common arterial trunk.
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http://dx.doi.org/10.1111/joa.13378DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8053582PMC
May 2021

Outcomes after common arterial trunk repair: Impact of the surgical technique.

J Thorac Cardiovasc Surg 2021 10 28;162(4):1205-1214.e2. Epub 2020 Nov 28.

M3C-Necker Enfants malades, AP-HP, Université de Paris, Paris, France.

Objectives: We compared the risk of mortality and reintervention after common arterial trunk (CAT) repair for different surgical techniques, in particular the reconstruction of the right ventricle outflow tract with left atrial appendage (LAA) without a monocusp.

Methods: The study population comprised 125 patients with repaired CAT who were followed-up at our institution between 2000 and 2018. Statistical analysis included Cox proportional hazard models.

Results: Median follow-up was 10.6 years. The 10-year survival rate was 88.2% (95% confidence interval [CI], 80.6-92.4) with the poorest outcome for CAT type IV (64.3%; 95% CI, 36.8-82.3; P < .01). In multivariable analysis, coronary anomalies (hazard ratio [HR], 11.63 [3.84-35.29], P < .001) and CAT with interrupted aortic arch (HR, 6.50 [2.10-20.16], P = .001) were substantial and independent risk factors for mortality. Initial repair with LAA was not associated with an increased risk of mortality (HR, 0.37 [0.11-1.24], P = .11). The median age at reintervention was 3.6 years [7.3 days-13.1 years]. At 10 years, freedom from reintervention was greater in the group with LAA repair compared with the valved conduit group, 73.3% (95% CI, 41.3-89.4) versus 17.2% (95% CI, 9.2-27.4) (P < .001), respectively. Using a valved conduit for repair (HR, 4.79 [2.45-9.39], P < .001), truncal valve insufficiency (HR, 2.92 [1.62-5.26], P < .001) and DiGeorge syndrome (HR, 2.01 [1.15-3.51], P = .01) were independent and clinically important risk factors for reintervention.

Conclusions: For the repair of CAT, the LAA technique for right ventricle outflow tract reconstruction was associated with comparable survival and greater freedom from reintervention than the use of a valved conduit.
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http://dx.doi.org/10.1016/j.jtcvs.2020.10.147DOI Listing
October 2021
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