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Arrhythmic Risk in paediatric Patients Undergoing Surgical Repair for Pulmonary Atresia with Intact Ventricular Septum

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Congenital Heart Disease 2021年第1期16卷 85-94页

作者： Pietro Paolo Tamborrino Corrado Di Mambro Cecilia Marcolin Walter Vignaroli Giulia Cafiero Gianluca Brancaccio Sonia Albanese Massimo Stefano Silvetti Adriano Carotti Fabrizio Drago paediatric cardiology and Cardiac Arrhythmia unit Department of Paediatric Cardiology and Cardiac SurgeryBambino GesùChildren’s HospitalIRCCSRomeItaly European Reference Network for Rare and Low Prevalence Complex Disease of the Heart paediatric Cardiac Surgery unit Department of Paediatric Cardiology and Cardiac SurgeryBambino GesùChildren’s HospitalIRCCSRomeItaly Sport Medicine unit Department of Paediatric Cardiology and Cardiac SurgeryBambino GesùChildren’s HospitalIRCCSRomeItaly

Introduction:While previous studies only focused on the arrhythmic risk associated with specific correction strategies,this study evaluates this risk in a large cohort of paediatric patients with all phenotypes of PA-IVS after surgical ***:In this single centre observational cohort study,we retrospectively evaluated 165 patients with a diagnosis of PA-IVS and we excluded those with an exclusively percutaneous treatment,patients lost or with insufficient follow-up and those affected by other arrhythmic *** history and clinical outcomes were ***:86 patients were included in the study(54 male[62.8%],mean age 16.4±6.1 years),with median follow-up from definitive repair of 12.8 years(6.4–18.9 years).They underwent three different final repairs:23 patients(26.7%)univentricular palliation,43(50%)biventricular correction,and 20(23.3%)one and a half ventricle *** patients(15%)developed arrhythmia:6 patients(all the subgroups)sinus node disfunction(SND);2(biventricular repair)premature ventricular complexes;2(one and a half ventricle repair)non-sustained ventricular tachycardia;1(biventricular repair)intra-atrial re-entrant tachycardia;1(one and a half ventricle repair)supraventricular tachyarrhythmia;1(biventricular repair)atrial *** patients with SND needed a pacemaker *** Fontan circulation showed an association with SND,while the other two groups heterogeneous types of ***:The low arrhythmic risk is related to surgical repair,it does not appear to be associated with native cardiomyopathy,and it appears to increase with length of follow *** follow-up in specialized centres is necessary to make an early diagnosis and to manage the potential haemodynamic impact at medium-long term.

关键词： Pulmonary atresia with intact ventricular septum arrhythmic risk univentricular palliation Fontan circulation biventricular repair one and a half repair

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Longitudinal Evaluation of Right Ventricle Function after Right Ventricle-Pulmonary Artery Shunt ***-Taussig Shunt

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Congenital Heart Disease 2021年第1期16卷 27-37页

作者： Nunzia Borrelli Jolanda Sabatino Martina Avesani Josefa Paredes Manjit Josen Alain Fraisse Paolo Guccione Guido Michielon Giovanni Di Salvo paediatric cardiology Department Royal Brompton HospitalLondonSW36NPUK paediatric cardiology Department Bambino GesùPaediatric HospitalRome00165Italy cardiology Department “Magna Graecia”UniversityCatanzaro88100Italy paediatric cardiology Department University of PaduaPadua35128Italy

Background:Still little is known about the impact on right ventricle function of the 2 main approaches to Norwood palliation in Hypoplastic left heart syndrome,the right ventricle-pulmonary artery shunt(RVPAS)and the modified Blalock-Taussig shunt(mBTS).Methods:The cohort included 27 patients with Hypoplastic left heart syndrome(10 in the mBTS group,17 in the RVPAS group).Longitudinal strain,tricuspid annulus peak systolic excursion and fractional area change were evaluated before Norwood and in four different breakpoints in the steady-state after Norwood procedure(30-days,90-days,140-days and 200-days after Norwood).Results:No significant differences were found in all standard echocardiographic functional parameters between the two groups at any ***,when we compared right ventricle function before and after Norwood procedure,longitudinal strain significantly improved in mBTS group compared to pre-surgical assessment(after 90-days:mBTS+27,35%±43,47 ***-8,20%±25,25,p=0,03;after 200-days:mBTS+10.19%±36.58 ***-21.64%±30.43,p=0.04).Conclusion:The mBTS group,which did not receive any ventriculotomy,showed a significant increase in right ventricle longitudinal strain during *** data support the use of longitudinal strain in Hypoplastic left heart syndrome evaluation and may suggest potential value in terms of cardiac mechanics in using mBTS,preserving the right ventricle integrity.

关键词： Hypoplastic left heart syndrome speckle tracking echocardiography congenital heart disease

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Is early patent ductus arteriosus ligation helpful in premature neonates? A 10-year retrospective study

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World Journal of Pediatrics 2022年第5期18卷 350-357页

作者： Anip Garg Naveen Athiraman Elizabeth Jenkins David Crossland Prakash Kannan Loganathan Neonatal unit Royal Victoria InfirmaryNewcastleUK paediatric cardiology Freeman HospitalNewcastleUK Neonatal unit James Cook University HospitalMarton RoadMiddlesbrough TS43BWUK

Background To compare short and long outcomes between early(≤28 days)(EL)and late ligation(LL)*** explore factors predicting early extubation(≤7 days)after patent ductus arteriosus(PDA)*** We conducted a single center,retrospective cohort study of preterm infants<32 weeks who underwent surgical ligation over a 10-year period(2009-2019).Results A total of 133 infants underwent PDA ligation,in the study *** groups had similar short-term outcome such as bronchopulmonary dysplasia(BPD)or death(96%vs.98%,P=0.64)and long-term clinical outcomes including Bayley's assessment at 2 years corrected *** infants in the EL group developed severe BPD(63%vs.81%,P=0.02).Age at ligation had adjusted odds ratio of 1.04 with over lapping confidence interval(95%CI 1.0-1.1,P=0.02)for severe BPD/*** was no difference in day of extubation between the EL and LL group(8 days vs.7 days,P=0.85).Left atrium/aortic root ratio of ≥1.75 would give sensitivity of 41% and 80% specificity for early extubation(area under the curve of 0.61).There was marginal reduction of hospital stay in the EL group[113(105-121)days vs.115(107-123)days;log rank P=0.026].Conclusion EL can be delivered safely with a clinically important lower incidenee of severe BPD and shorter duration of hospital stay compared to LL.

关键词： Bronchopulmonary dysplasia Extubation Patent ductus arteriosus ligation

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The Impact of a Bicuspid Aortic Valve on Aortic Geometry and Function in Patients with Aortic Coarctation:A Comprehensive CMR Study

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Congenital Heart Disease 2021年第6期16卷 551-560页

作者： Laura Schweikert Dominik Gabbert Sylvia Krupickova Inga Voges Department of Congenital Heart Disease and paediatric cardiology University Hospital Schleswig-HolsteinKielGermany Royal Brompton Hospital LondonUK

Background:An isolated bicuspid aortic valve(BAV)is associated with structural and functional abnormalities of the aorta and the left ventricle(LV).Although~50%of patients with aortic coarctation(CoA)have a BAV,less is known about its impact on LV function and aortic geometry and function in CoA *** this cardiovascular magnetic resonance imaging(CMR)study,we analysed markers of LV and aortic function as well as aortic geometry in a large cohort of CoA patients with a BAVand compared them with CoA patients with a tricuspid aortic valve(TAV).Methods:We included 48 patients with a BAV(18.4±9.3 years)and 45 patients with TAV(20.7±9.9 years).LV volumes,mass and ejection fraction as well as aortic distensibilty,pulse wave velocity(PWV)were measured from standard cine CMR and phase-contrast CMR images.2-dimensional CMR feature tracking(2DCMR-FT)was performed to measure longitudinal,circumferential and radial strain and strain rate of the *** arch geometry was classified as romanic,gothic and ***:LV volumes,mass and ejection fraction as well as aortic distensibility and PWV did not significantly differ between the BAV and the TAV *** was also no significant difference for LV global longitudinal,radial and circumferential strain and strain rate between both *** with a BAV had more commonly a gothic aortic arch compared to TAV patients,but this difference was not statistically significant(22 vs.14,p=0.2).Ascending and descending aortic distensibility correlated with LV mass in the entire patient group(p<0.001).Global longitudinal,circumferential and radial strain(GLS,GCS,GRS)and global longitudinal and circumferential strain rate(GLSR,GCSR)correlated with LV ejection fraction(p<0.001).Conclusion:Our data suggest that the presence of a BAV does not adversely impact LV and aortic function in children and young adults with *** correlation of global circumferential,longitudinal and radial strain values with LV ejection fraction dem

关键词： Aortic coarctation bicuspid aortic valve cardiovascular magnetic resonance feature tracking

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Ventricular Arrhythmia in the Fontan Circulation:Prevalence,Risk Factors and Clinical Implications

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Congenital Heart Disease 2023年第5期18卷 507-523页

作者： Charis Tan Diana Zannino Carley Clendenning Sophie Offen Thomas LGentles Julian Ayer David Tanous Vishva Wijesekera Leeanne Grigg David Celermajer Mark McGuire Yves d’Udekem Rachael Cordina Department of Cardiothoracic Surgery Royal Prince Alfred HospitalCamperdownAustralia Sydney Medical School University of SydneyCamperdownAustralia Clinical Epidemiology and Biostatistics unit Murdoch Children’s Research InstituteParkvilleAustralia Department of cardiology Royal Prince Alfred HospitalCamperdownAustralia Department of paediatric cardiology Starship HospitalAucklandNew Zealand Department of paediatric cardiology The Children’s Hospital at WestmeadWestmeadAustralia Department of cardiology Westmead HospitalWestmeadAustralia Adult Congenital Heart Disease unit The Prince Charles HospitalBrisbaneAustralia Department of cardiology The Royal Melbourne HospitalMelbourneAustralia Heart Research Institute CamperdownAustralia Department of Cardiac Surgery The Royal Children’s Hospital MelbourneParkvilleAustralia Department of paediatrics The University of MelbourneParkvilleAustralia

Objective:Sudden cardiac death(SCD)and malignant ventricular arrhythmia(VA)are increasingly recognized as important issues for people living with a Fontan circulation,but data are *** sought to characterize the cohort who had sudden cardiac death,most likely related to VA and/or documented VA in the Australia and New Zealand Fontan Registry including risk factors and clinical ***:A retrospective cohort study was *** criteria were documented non-sustained ventricular tachycardia,sustained ventricular tachycardia,ventricular fibrillation,resuscitated cardiac arrest or SCD>30 days post-Fontan ***:Of 1611 patients,20(1.2%)had VA;14(1.0%)had VA without SCD and 6(<1%)had SCD(6%of all deaths recorded in Registry;5 of those had documented VA at the time of arrest and 1 was presumed to be VA-associated).The median age at first VA was 20.5(14–32)years,10(50%)were females,and the median age at Fontan operation was 8(4–17)*** univariable analysis,hypoplastic left heart syndrome(p=0.03)and older age Fontan operation(p<0.001)were associated with *** Fontan era(p<0.003),atriopulmonary Fontan(p<0.001),pre-Fontan atrioventricular valve repair(p=0.013)pre-or post-Fontan atrial arrhythmia(p=0.010)were associated with *** with VA had a 3 times higher risk of death or heart transplant(HR 3.27(1.19,8.98),p=0.02).Conclusions:A proportion of people living with a Fontan circulation have malignant *** VA screening in this cohort is *** data are needed to aid risk stratification.

关键词： Fontan congenital cardiac arrhythmia sudden cardiac death ventricular tachyarrhythmia

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Efficacy,Safety and Characteristics of the Amplatzer Vascular Plug II and IV Utilization for Various Percutaneous Occlusions in Children under 10 Years

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Congenital Heart Disease 2022年第4期17卷 421-436页

作者： Hugues Lucron Alban-Elouen Baruteau Caroline Ovaert Ali Houeijeh Mélanie Brard Patrice Guerin François Bourlon Claire Dauphin Saskia Tuttle Maha Tagorti Rishika Banydeen François Godart Antilles-Guyane M3C paediatric cardiology Center University Hospital of MartiniqueFort-de-FranceFrance L’institut du Thorax INSERMCNRSUNIV NantesCHU NantesNantesFrance Department of paediatric cardiology and Pediatric Cardiac Surgery University Hospital of NantesNantesFrance Department of paediatric and Congenital Heart Disease University Hospital of MarseilleMarseilleFrance Department of paediatric and Congenital Heart Disease University Hospital of Lille-Nord de FranceLilleFrance Department of paediatric and Congenital Heart Disease Cardiothoracic Center of MonacoMonaco Department of paediatric and Congenital Heart Disease University Hospital of Clermont-FerrandClermont-FerrandFrance Clinical Research Department CHU Martinique(University Hospital of Martinique)Fort-de-FranceFrance

Objectives:We aim to describe the efficacy,safety,and characteristics of the Amplatzer Vascular Plug(AVP)II and IV“off-label”use for multiple cardiovascular occlusions in children under 10 ***:Observational retrospective multicenter(2007–2020,6 centers)review of paediatric procedures using AVP II or ***:A total of 125 children(49.6%aged≤1 year,147 lesions)underwent 136 successive procedures(success rate:98.5%)using 169 devices(109 AVP IV,60 AVP II).The mean device diameter was 7.7±3.2 mm(4–20 mm).The median AVP size to vessel diameter ratio was 1.3(0–2).The median age and weight at implantation were 1.0 year(0.01–9.98)and 8.4 kg(1–69).Procedures were heterogeneous(55 patent ductus arteriosus(PDA),28 collaterals,18 sequestrations,22 arteriovenous/veinovenous/coronary fistulas,6 vertical veins,6 conduits,5 ventricular septal defects,7 miscellaneous).Day 1 and 6-month occlusion rates were respectively 94.8%and 98.5%.Major adverse events(MAE)occurred in 5.2%of cases(no procedure-related deaths),and more frequently in weight≤5 kg(p=0.01),younger patients(p=0.03)during PDA closure(p=0.02)of tubular types(p=0.02)using larger devices(p=0.03)and AVP II(p=0.003).Independent predictor of MAE risk was a higher AVP diameter to patient weight ratio(Odds-ratio:2.33,95%confidence interval 1.31–4.13,p=0.004,optimal cut off:1.45).Conclusions:Both AVPs are safe and effective for percutaneous occlusions in children under *** devices represent an alternative“off label”use for well selected paediatric patients.

关键词： Amplatzer vascular plug(AVP)II and IV cardiovascular occlusions off-label use children under 10 years efficacy safety

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Frequency and individual severity of arterial blood pressure changes in children and adolescents with attentiondeficit/hyperactivity disorder treated with methylphenidate hydrochloride: a prospective non-interventional study

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General Psychiatry 2020年第2期33卷 80-87页

作者： Dorothee Busold-Hagenbeck Julia Elmenhorst Christoph Irtel von Brenndorff Reinhard Hilgers Martin Hulpke-Wette University Medical Centre Gottingen GottingenLower SaxonyGermany German Heart Centre Munich MunichBavariaGermany 3Medical Practice for Paediatric CardiologyBaden-Baden Baden-Wurttemberg Germany Departm ent of Medical Statistics University Medical Centre GottingenGottingenLower SaxonyGermany Medical Practice for paediatric cardiology GottingenLower SaxonyGermany

Background Attention-deficit/hyperactivity disorder(ADHD)is a common behavioural disorder in *** psychostimulant methylphenidate hydrochloride(MPH)is one of the major pharmacological options for *** is known to result,on average,in a small increase in arterial blood pressure(BP).However,there are few clinical data regarding theindividual influences of MPH on BP among children and adolescents with *** to the European Union-wide standardised patient information sheet for MPH,BP changes>10 mm Hg compared with baseline values are Common'(ie,≥1%topaediatric cardiology centres in Germany and *** After beginning MPH therapy,34%of participants(99%Cl 15.52%to 52.66%)had BP increases/decreases>10 mm *** mean changes in systolic BP and diastolic BP were 0.87 mm Hg(95%Cl-1.75 mm Hg to 3.48 mm Hg)and 1.96 mm Hg(95%Cl 0.21 mm Hg to 3.7 mm Hg),*** proportion of participants with initial prehypertension/hypertension was 54.55%.Conclusions In our sample with a high baseline rate of prehypertension/hypertension,BP changes>10 mm Hg during MPH therapy were more frequent than those indicated by the patient information ***,individual BP changes,including increases and decreases>10 mm Hg,resulted in a small average BP increase in the sample,thus reflecting n

关键词： severity prospective routine

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Outcomes of Transcatheter Closure in Outlet-Type Ventricular Septal Defect after 1 Year

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Congenital Heart Disease 2023年第2期18卷 169-182页

作者： Supaporn Roymanee Nantawan Su-angka Worakan Promphan Kanjarut Wongwaitaweewong Jirayut Jarutach Rujira Buntharikpornpun Pimpak Prachasilchai Department of paediatrics Division of Paediatric CardiologyFaculty of MedicinePrince of Songkla UniversitySongkhla90110Thailand Department of Medical Services Paediatric Heart CentreQueen Sirikit National Institute of Child HealthMinistry of Public HealthBangkok10400Thailand College of Medicine Rangsit UniversityBangkok10200Thailand

Background:Ventricular septal defect(VSD)is the most common congenital heart *** VSD closure is an effective treatment for patients with muscular and perimembranous ***,there is a limit data for outlet VSD,especially impact to the aortic valve leaflet after transcatheter *** study aims to assess the outcomes of transcatheter closure of the outlet-type ventricular septal defect(OVSD)after 1 postoperative ***:A retrospective study was performed including 50 patients who underwent transcatheter(n=25)and surgical(n=25)OVSD closure during the exact time frame at two medical ***:The median age and body weight of patients in the transcatheter group were significantly higher than those of patients in the surgical group(7.0 vs.2.8 years;27.0 vs.11.4 kg;p<0.01).The defect size in the surgical group was significantly larger than that in the transcatheter group(5.0 vs.3.0 mm;p<0.01).All OVSD patients have successful transcatheter closure(100%)as effective as surgical *** than small residual shunt was present 20%and 8%immediately after the procedure in the transcatheter and surgical groups(p=0.50),which decreased to 12%and 4%at the 1-year follow-up(p=0.61),*** incidence of complete atrioventricular block and other complications was observed in both groups,and no significant differences were noted in the new onset or worsening of the aortic regurgitation in both groups(p=1.0).Conclusions:Transcatheter treatment could be effectively and safely achieved for OVSD closure at 1-year follow-up.

关键词： Outlet ventricular septal defect aortic valve prolapse aortic regurgitation transcatheter closure outcome intervention

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Application of stent implantation in congenital heart disease

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Chinese Medical Journal 2014年第21期127卷 3814-3824页

作者： Chau Kai Tung Adolphus Department of paediatric cardiology Queen Mary Hospital University of Hong Kong Hong Kong SAR China

Vascular stenoses and hypoplasia in children are associated with congenital heart disease or occur as residual lesions or complications after surgical repair. These obstructions can occur in arteries or veins of both the pulmonary and systemic circulations. Untreated vascular stenoses cause significant mortality and morbidity and will severely affect surgical outcome.

关键词： children congenital heart disease stent implantation transcatheter intervention treatment

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Scimitar Syndrome:Role of Right Atrial Longitudinal Strain.A Case Report

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Congenital Heart Disease 2021年第4期16卷 411-416页

作者： Isabella Leo Jolanda Sabatino Sabrina La Bella Antonio Strangio Iolanda Aquila Concetta Procopio Carmen Anna Maria Spaccarotella Maria Petullà Salvatore De Rosa Ciro Indolfi Division of cardiology Department of Medical and Surgical SciencesMagna Graecia UniversityCatanzaroItaly Cardiovascular Research Center Magna Graecia UniversityCatanzaroItaly Department of paediatric cardiology Royal Brompton HospitalLondonUK Department of Radiology Magna Graecia UniversityCatanzaroItaly Mediterranea Cardiocentro NapoliItaly

We describe a case of a rare congenital heart disorder,scimitar syndrome,diagnosed in an adult woman presenting with dyspnea on exertion,chest pain and recurrent episodes of pulmonary *** hallmark of the syndrome is the presence of an enlarged anomalous pulmonary vein draining into the inferior vena *** tracking echocardiography,including the often-forgotten atrial strain evaluation,is a sensitive parameter that should be routinely used for a better clinical and prognostic evaluation of patients with congenital heart disease(CHD).

关键词： Scimitar syndrome RV overload congenital heart disease myocardial strain

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