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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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Modified ultrafiltration for paediatric cardiac surgical patients: additional benefits and concerns

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中华医学杂志(英文版) 2004年第2期000卷 160-161页

作者： Shahzad G.Raja Department of paediatric cardiac surgery Alder Hey Children's HospitalLiverpool L12 2AP United Kingdom

I read with great interest the article by Huang et al and have a few comments which I am sure will not only substantiate the findings of the authors but will also give the readers additional information on use of modi... 详细信息

关键词： additional benefits and concerns Modified ultrafiltration for paediatric cardiac surgical patients

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surgery for Residual Inferior Left-to-Right Atrial Shunt

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Congenital Heart Disease 2021年第1期16卷 39-43页

作者： Francesco Bertelli Claudia Cattapan Alvise Guariento Vladimiro L.Vida paediatric and Congenital cardiac surgery unit Department of CardiacThoracicVascular Sciences and Public HealthUniversity of PaduaPaduaItaly Department of Cardiovascular surgery The Hospital for Sick ChildrenUniversity of TorontoTorontoCanada

We report the case of three female patients who were scheduled for surgical correction of residual left-to-right shunt after initial repair of sinus venosus atrial septal defect(SV-ASD)during *** excluding the possibility of an hemodynamic intervention,all three patients underwent a successful surgical closure through a right mini sub-axillary approach by using total peripheral cannulation for cardiopulmonary bypass and leaving the inferior vena cava completely un-snared allowing for an optimal visualization of the residual atrial septal communication and avoiding extensive dissection of mediastinal structures.

关键词： Residual sinus venosus-ASD un-snared vena cavae minimally invasive cardiac surgery inferior ASD

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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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A Comparative Study on Safety and Efficacy of Caudal, Thoracic Epidural and Intra Venous Analgesia in paediatric cardiac surgery: A Double Blind Randomised Trial

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World Journal of Cardiovascular surgery 2020年第7期10卷 101-114页

作者： Mahesh Vakamudi Rajesh Kumar V. Kodali Ranjith B. Karthekeyan Periyasamy Thangavel Kamalakannan G. Sambandham Department of Anaesthesiology Sri Ramachandra Institute of Higher Education and Research (SRIHER) Chennai India Department of cardiac Anaesthesiology Sri Ramachandra Institute of Higher Education and Research (SRIHER) Chennai India HOD Department of cardiac surgery Sri Ramachandra Institute of Higher Education and Research (SRIHER) Chennai India

Introduction:Regional anaesthesia combined with general anaesthesia reduces stress response to surgery, duration of ventilation, intensive care unit (ICU) stay and promotes early recovery. Studies on thoracic epidural, caudal analgesia along with general anaesthesia (GA) in paediatric cardiac surgery are limited hence we aimed to compare efficacy and safety of caudal, thoracic epidural and intravenous analgesia in paediatric cardiac surgery. Methodology: This study was conducted in the Department of Anaesthesiology in a tertiary care teaching hospital in southern India from February 2019 to December 2019. 90 children were randomised into group A, group B, group C. Children in group A received caudal analgesia along with GA. Group B children received thoracic epidural along with GA. Group C patients received intravenous analgesia along with GA. Rescue analgesia 1 mcg/kg fentanyl given in all 3 groups if pain score is more than 4. Primary outcome assessed was post-op pain scores. Secondary outcome assessed was duration of ventilation, duration of intensive care unit stay. Results: All patients were comparable in terms of age, sex, weight, mean RACHS score, baseline heart

关键词： Caudal Analgesia Thoracic Epidural Analgesia Pain Scores paediatric cardiac surgery

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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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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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3.0T MR Coronary Angiography after Arterial Switch Operation for Transposition of The Great Arteries-Gd-FLASH Versus Non-Enhanced SSFP.A Feasibility Study

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Congenital Heart Disease 2021年第2期16卷 107-121页

作者： Kathrine Rydén Suther Charlotte de Lange Henrik Brun Rolf Svendsmark Bac Nguyen Stig Larsen Bjarne Smevik Arnt Eltvedt Fiane Harald Lauritz Lindberg Einar Hopp Department of Radiology Division of Radiology and Nuclear MedicineOslo University HospitalRikshospitaletOsloNorway Department of paediatric Radiology and Clinical Physiology Queen Silvia Children’s hospitalSahlgrenska University HospitalGothenburgSweden Department of paediatric Cardiology Division of Paediatric and Adolescent MedicineOslo University HospitalOsloNorway Norwegian University of Life Sciences Faculty of Veterinary MedicineCentre for Epidemiology and BiostatisticsCampus AdamstuenOsloNorway Faculty of Medicine University in OsloOsloNorway Department of Cardiothoracic surgery Oslo University HospitalRikshospitaletOsloNorway

Background:Patency of the coronary arteries is an issue after reports of sudden cardiac death in patients with transposition of the great arteries(TGA)operated with arterial switch(ASO).Recent studies give rise to concern regarding the use of ionising radiation in congenital heart disease,and assessment of the coronary arteries with coronary MR angiography(CMRA)might be an attractive non-invasive,non-ionising imaging alternative in these ***,the use of 3.0T CMRA should improve the visualisation of the coronary *** objective of this study was to assess feasibility of 3.0T CMRA at the coronary artery origins by comparing image quality with non-contrast CMRA in ASO TGA patients to healthy age-matched controls,and by comparing image quality with non-contrast CMRA to contrast enhanced CMRA in the patient *** and methods:Twelve patients,9-15 years(mean 11.9 years,standard deviation 1.5 years),and 12 age-matched controls(mean 12.7 years,standard deviation 1.7 years)were examined with 3D balanced steady-state free precession(SSFP).Nine of twelve patients had Gadolinium-enhanced fast low-angle shot(Gd-FLASH)performed after *** quality at the coronary artery origins was evaluated subjectively with a 10 cm figurative visual analogue scale(fVAS)and objectively by signal-to-noise and contrast-to-noise ratio(SNR,CNR).Results:All,but one,coronary artery origins were *** significant difference in image quality scores was found between patients and controls with SSFP(mean values 6.5 cm—9.1 cm in patients and 7.0 cm—8.0 cm in controls,p-values>0.1).With SSFP,intra-observer fVAS mean score was 6.7 cm—8.6 cm and with Gd-FLASH 7.7 cm—8.7 *** was higher with Gd-FLASH(p<0.03).Intra-observer agreement index(AI)with SSFP was moderate-to-good(0.43–0.71)and with Gd-FLASH good(0.64–0.79)in all ***-observer AI was good in the left main stem(LMS)with SSFP(0.65).With Gd-FLASH inter-observer AI was good in LMS(0.78)and moderate(0

关键词： Magnetic resonance imaging coronary angiography gadolinium transposition of great vessels arterial switch operation

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Congenital Absence of Pericardium:The Largest Systematic Review in the Field on 247 Worldwide Cases(1977-Now)

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Congenital Heart Disease 2023年第6期18卷 595-610页

作者： Pier Paolo Bassareo Aurelio Secinaro Paolo Ciliberti Massimo Chessa Marco Alfonso Perrone Kevin Patrick Walsh Colin Joseph Mcmahon Mater Misericordiae University Hospital National Adult Congenital Heart Disease ServiceDublinD07 R2WYIreland Children’s Health Ireland Crumlin DublinIreland School of Medicine University College of DublinDublinIreland Advanced Cardiovascular Imaging unit Bambino GesùChildren’s HospitalIRCCSRomeItaly Department of cardiac surgery CardiologyHeart and Lung Transplantation Bambino Gesu’Children’s HospitalIRCCSRomeItaly Adult Congenital Heart Disease unit Paediatric and Adult Congenital Heart CentreIRCCS-Policlinico San DonatoSan Donato MilaneseMilanItaly School of Medicine Vita Salute San Raffaele UniversityMilanItaly Division of Cardiology and CardioLab Department of Clinical Sciences and Translational MedicineUniversity of Rome Tor VergataRomeItaly

Background:Congenital absence of pericardium(CAP),also known as pericardial agenesis,represents an uncommon cardiac abnormality and mostly incidental *** can be subdivided into complete and partial(left or right-sided)*** of its infrequency,just case reports and a few case series have been released so *** paper represents the largest systematic review in the *** features(age at diagnosis,type,gender,clinical presentation,electrocardiography,imaging(ultrasounds,CT/MRI),concomitant cardiac defects,and outcome)were ***:The electronic database PubMed was investigated from its establishment up to July 15th,*** case reports and case series were *** studies,papers that were not in English,Spanish,and Italian,and those manuscripts not reporting at least seven of the nine analysed *** ruled *** analysed data were reported mostly in terms of ***:One hundred eighty studies were included encompassing 247 *** than half of reviewed CAP cases were in males(63.2%).The mean age at diagnosis was 31.8±19.3 years;a range of 32 weeks of gestation-81 years).23.5%of the patients did not report any *** most common clinical presentations were chest pain(35.2%)and dyspnoea(29.2%).The most commonly seen ECG changes were right axis deviation(28.7%)and right bundle branch block(23.9%).CAP was suspected or diagnosed by echocardiography in 20.1%of *** diagnosis was made by CT and/or MRI in 61.9%of *** was left-sided in 71.2%,complete in 23.1%,and right-sided in 5.7%.A concomitant congenital heart defect was found in 22.7%,especially in the form of atrial septal defect(6.5%)and patency of ductus arteriosus(2.8%).The pericardial repair was required in 12.9% of the incomplete forms of the *** did the complete form require surgical *** outcome appeared favourable in the vast majority of cases,with just 18 deaths(7.3%).Discussion:The main limitation of this syste

关键词： Congenital absence pericardium pericardial agenesis electrocardiography echocardiography computed tomography cardiac magnetic resonance imaging

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Echocardiography in children with Down syndrome

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World Journal of Clinical Pediatrics 2013年第4期2卷 36-45页

作者： Mohammed A Al-Biltagi paediatric Department Faculty of Medicine Tanta University Tanta 31527 Egypt

Congenital heart disease is a common problem in children with Down syndrome(DS). Echocardiography plays an important role in the detection of both structural and functional abnormalities in this group of patients. Fetal echocardiography can help in the early recognition of DS by detecting soft markers of DS, but its main role is to define the exact nature of the suspected cardiac problem in the fetus. Postnatal echocardiography is mandatory in the first month of life for all neonates with DS. It is also indicated before any cardiac surgery and for serial follow-up after cardiac *** this article, we discuss the types and mechanism of cardiac abnormalities in DS children and the role of both fetal and postnatal echocardiography in the detection of these abnormalities.

关键词： Fetal Echocardiography Congenital heart disease Down syndrome

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