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

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 repair.Methods: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 syndromes.Surgical history and clinical outcomes were reviewed.Results: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 correction.Thirteen 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 fibrillation.Three patients with SND needed a pacemaker implantation.Only Fontan circulation showed an association with SND,while the other two groups heterogeneous types of arrhythmias.Conclusions: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 up.Continuous follow-up in specialized centres is necessary to make an early diagnosis and to manage the potential haemodynamic impact at medium-long term.

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

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 infections.The hallmark of the syndrome is the presence of an enlarged anomalous pulmonary vein draining into the inferior vena cava.Speckle 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).

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

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 patients.Theoretically,the use of 3.0T CMRA should improve the visualisation of the coronary arteries.The 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 group.Material 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 SSFP.Image 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 identified.No 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 cm.CNR 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 origins.Inter-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(0.5)in the left anterior descending artery,but lacking in the other origins though with a good agreement on Bland-Altman plots.Conclusions:Our findings indicate a better,more reproducible image quality with Gd-FLASH than with non-contrast SSFP CMRA on 3.0T for evaluation of the coronary artery origins in ASO TGA children and adolescents.

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

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 years.Methods:Observational retrospective multicenter(2007–2020,6 centers)review of paediatric procedures using AVP II or IV.Results: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 10.Such devices represent an alternative“off label”use for well selected paediatric patients.

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

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 lacking.We 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 outcomes.Methods:A retrospective cohort study was performed.Inclusion criteria were documented non-sustained ventricular tachycardia,sustained ventricular tachycardia,ventricular fibrillation,resuscitated cardiac arrest or SCD>30 days post-Fontan completion.Results: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)years.On univariable analysis,hypoplastic left heart syndrome(p=0.03)and older age Fontan operation(p<0.001)were associated with VA.Earlier 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 SCD.Patients 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.Routine VA screening in this cohort is essential.More data are needed to aid risk stratification.

常规胎儿超声检测出生前房室隔缺损的灵敏度较低

Objective: To report the antenatal detection rate in a consecutive series of l iveborn infants with atrioventricular septal defect (AVSD). Design: Review and a nalysis of referrals for detailed fetal echocardiography and postnatal diagnosis of AVSD. Setting: Tertiary referral centre for congenital heart disease centre with data prospectively collected between 1996 to 2001. Results: 92 consecutivel y liveborn infants with AVSDs were identified of which 27 (29%) were detected b y routine obstetric antenatal ultrasound. The antenatal diagnosis rate was worse for liveborn infants with trisomy 21(12 of 49(25%) v 15 of 43 (35%) chromosom ally normal children) and for infants with AVSD without other structural heart d isease (18 of 74 (24%) v 9 of 18 (50%) infants with associated structural hear t disease). Conclusion: Despite the potential ability of fetal ultrasound to det ect AVSDs, the antenatal diagnosis rate is poor. This is particularly true for i nfants with trisomy 21 and is of importance when counselling parents with an app arently normal fetal ultrasound scan.

Percutaneous pulmonary and tricuspid valve implantations: An update

The field of percutaneous valvular interventions is one of the most exciting and rapidly developing within interventional cardiology.Percutaneous procedures focusing on aortic and mitral valve replacement or interventional treatment as well as techniques of percutaneous pulmonary valve implantation have already reached worldwide clinical acceptance and routine interventional procedure status.Although techniquesof percutaneous pulmonary valve implantation have been described just a decade ago,two stent-mounted complementary devices were successfully introduced and more than 3000 of these procedures have been performed worldwide.In contrast,percutaneous treatment of tricuspid valve dysfunction is still evolving on a much earlier level and has so far not reached routine interventional procedure status.Taking into account that an "interdisciplinary challenging",heterogeneous population of patients previously treated by corrective,semi-corrective or palliative surgical procedures is growing inexorably,there is a rapidly increasing need of treatment options besides redo-surgery.Therefore,the review intends to reflect on clinical expansion of percutaneous pulmonary and tricuspid valve procedures,to update on current devices,to discuss indications and patient selection criteria,to report on clinical results and finally to consider future directions.

主动脉缩窄儿科患者随访中磁共振血流检测的价值:一项再评价

背景:先前研究提示,利用相位对比MR成像(PC-MRI)无创性定量检测血管跨狭窄压力阶差(ΔP)是可行的。

荷兰儿童中埋藏式心脏复律除颤器的植入

To evaluate the indications, underlying cardiac disorders, efficacy and complications involved with implantable cardioverter- defibrillators (ICDs) in paediatric patients in The Netherlands, the records of all patients aged 18 years or younger who underwent ICD placement were reviewed retrospectively. Between January 1995 and September 2002, 23 patients (11 males, 12 females; median age 12 years, range 6 months to 16 years) underwent ICD implantation. The ICD was implanted for aborted sudden cardiac death ( n =14), syncope ( n =5) or for primary prevention of sudden cardiac death ( n =4). Underlying cardiac disorders were electrical diseases ( n =16), hypertrophic or dilated cardiomyopathy ( n =4) and congenital cardiac malformations ( n =3). Five patients had an epicardially placed ICD, while 18 underwent a transvenous approach. The generator was placed in an abdominal position in eight patients, whereas it was placed in the subpectoral region in 15. There was no early mortality. Median hospital stay was 5 days (range 2- 30 days). Median follow-up time was 29 months (range 1 month to 7 years). Seven patients experienced an inappropriate shock after a median period of 7 months; five patients an appropriate shock after a median period of 3 months. The reasons for inappropriate shock were supraventricular tachycardia ( n =1), sinus tachycardia ( n =4) or T-wave sensing ( n =2). One patient with malignant long QT syndrome died of intractable arrhythmias and irreversible cardiomyopathy. Generator replacement was necessary in four patients after 28, 44, 51 and 54 months respectively. Conclusion:Implantable cardioverter-defibrillator implantation in paediatric patients is a safe procedure with a good medium-term outcome. The most serious problem is the occurrence of a significant number of inappropriate shocks.

主动脉弓偏移和锁骨下动脉畸形是否为22q11.2缺失综合征的可靠标志

多种心脏先天性畸形包括永存交通动脉干、Fallot四联征、肺动脉闭锁伴室间隔缺损及主动脉弓间断等常被总称为锥干性心脏畸形。在有这些心脏先天性畸形的患者中,常发现其22号染色体上有一常见的微小缺失,即所谓的22q11.2单体。本研究的目的是确定这些患者其主动脉弓偏移或存在锁骨下动脉畸形(SAA)是否是22q11.2单体的标志。

扩大的主动脉弓修补术治疗主动脉缩窄的效果

Objectives: To assess survival and long term arch patency rates in a consecutive group of children after extended arch repair for coarctation of the aorta. Methods: Review of 191 consecutive children(154(81% ) under 1 year of age) operated on between 1990 and 2002 by a single surgeon using extended arch reconstructive techniques. For assessment of survival patients were divided into three groups: 1, coarctation alone, n=104; 2, coarctation and ventricular septal defect, n=38; and 3, coarctation in association with complex intracardiac anomalies, n=49. A prospective and systematic clinical and echocardiographic evaluation of the aortic arch was undertaken. Results: Median time to follow up was 4.2 years(range 1-10.6 years). Overall actuarial survival was 92% , 88% , and 88% at two, five, and 10 years. Mortality was significantly higher in those patients with complex intracardiac anatomy. Arch obstruction recurred in seven of 165(4.2% ) patients: four of 139(2.9% ) term and three of 10(30% ) premature infants(p < 0.001). Conclusions: Survival after extended arch reconstruction for coarctation is excellent. At long follow up recurrent arch obstruction is rare, with prematurity the only risk factor.

低能量射频导管消融术治疗早产新生儿室上性心动过速

A premature neonate with hydrops was born at 32 weeks of gestation after successful direct fetal amiodarone therapy via cordocentesis for incessant supraventricular tachycardia. After birth the tachycardia could not be controlled despite high doses of amiodarone and flecainide and the patient developed severe respiratory and circulatory failure. After 3 weeks, weighing 2 kg, he underwent successful and uncomplicated catheter ablation of a left free-wall accessory pathway using low-energy radiofrequency. Conclusion:radiofrequency catheter ablation is rarely used in neonates, but when used with caution may provide the optimal treatment.

经导管房间隔缺损封堵术后心肌肌钙蛋白的释放依赖于封堵器的大小并非患者的年龄

Objective: To examine whether transcatheter closure of secundum atrial septal defect(ASD) with the Amplatzer septal occluder leads to more myocardial injury in children than in adults. Design: In a prospective study with children and adults cardiac troponin I(cTnl) serum concentrations were determined by immunoassay(AxSYM, Abbott Laboratories) before, during, and up to 20 months after surgical or transcatheter ASD closure. Patients: Four groups of patients were studied: transcatheter ASD closure(group 1: 22 children, age range 3.26-14.7 years; group 2: 22 adults, 18.0-67.3 years), surgical ASD closure(group 3:18 children, 3.12-13.5 years), and diagnostic catheterisation(group 4: 12 children, 2.68-15.0 years). Results: cTnl concentrations were significantly increased after occluder implantation with higher serum concentrations in children than in adults(immediately after implantation: group 1, 3.2(4.4)-μg/l; group 2, 1.1(4.2) μg/l; four hours after implantation: group 1, 4.8(5.0) μg/l; group 2, 1.7(2.3) μg/l; both p< 0.01, group 1 v group 2; one day after implantation: group 1, 3.0(5.7) μg/l; group 2, 2.2(5.2) μg/l) but were less than 20%of those after surgical ASD closure(group 3; p< 0.001) where the highest cTnl concentration was found(37.1(26.3) μg/l). Diagnostic catheterisation(group 4) was not associated with detectable cTnl increase. From the cTnl concentrations the total amount of cTnl released after ASD closure was estimated for each patient. This was dependent on the size of the occluder(p< 0.05) but not on the patient’s age or procedural duration. Conclusion: In regard to interventional ASD closure our data do not provide evidence that the child’s myocardium is more vulnerable. Transcatheter ASD closure induces minor myocardial lesion, the extent of which depends on the size of the Amplatzer septal occluder but is irrespective of the patient’s age.

儿童的风湿性心脏病和冠脉系统血管炎

Rheumatic fever is a systemic inflammatory disease which may also cause vasculitis in multiple organ systems.Conclusion:Two children with rheumatic mitral valve disease and mitral valve replacement developed myocardial ischaemia due to coronary vasculitis.One of them had neurological findings that may be attributed to cerebral vasculitis.

出院前不能诊断新生儿危重心脏畸形是否是一个日益严重的问题

Aim: To determine what proportion of newborns with critical heart defects are discharged undiagnosed from the maternity ward. Methods: Data were retrieved retrospectively for infants born in our referral area from 1993 to 2001 and undergoing surgical or catheter- based intervention before 2 mo of age because of critical heart defects. Results: 259 full- term infants had critical heart defects. Duct- dependent systemic circulation was present in 129, duct- dependent pulmonary circulation in 106, and 24 infants were not duct dependent but critically ill. In 51 infants (20% ) the heart defect was not suspected before discharge from the maternity ward. Such late detection occurred more often in infants with duct- dependent systemic circulation (30% ) and in children with defects that were not duct dependent (38% ) than in children with duct- dependent pulmonary circulation (4% ) (p < 0.001). The proportion detected after discharge from the maternity ward increased from 13% in 1993- 1995 and 21% in 1996- 1998 to 26% in 1999- 2001 (p < 0.05). Conclusion: Many infants with critical congenital heart defects are not diagnosed before discharge from the neonatal maternity ward. The proportion discharged undiagnosed has increased. Neonatal screening fails mainly in children with duct- dependent systemic circulation.

先天性心脏病患者支架置入后的早期并发症:一项多中心研究

目的:对于先天性心脏病,支架置入术已成为一种非常成熟的心脏病学介入措施。尽管大部分支架置入术能成功完成,但并发症时有发生。此项多中心研究旨在评估先天性心脏病患者支架置入后的早期并发症。

Application of stent implantation in congenital heart disease

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.

Paediatric primary care in Germany during the early COVID-19 pandemic:the calm before the storm

Objectives Globally,the COVID-19 pandemic has a major impact on healthcare provision.The effects in primary care are understudied.This study aimed to explore changes in consultation numbers and patient management during the COVID-19 pandemic,and to identify challenges for patient care.Design Survey of paediatric primary care practices on consultation numbers and patient management changes,and semistructured interviews to identify challenges for patient care.Surveys and interviews were partially linked in an explanatory sequential design to identify patient groups perceived to be at higher risk for worse care during the pandemic.Setting In and around Düsseldorf,a densely populated area in Western Germany.The primary care facilities are spread over an area with approximately 2 million inhabitants.Participants Primary care in Germany is provided through practices run by self-employed specialist physicians that are contracted to offer services to patients under public health insurance which is compulsory to the majority of the population.The sample contained 44 paediatric primary care practices in the area,the response rate was 50%.Results Numbers of consultations for scheduled developmental examinations remained unchanged compared with the previous year while emergency visits were strongly reduced(mean 87.3 less/week in March-May 2020 compared with 2019,median reduction 55.0%).Children dependent on developmental therapy and with chronic health conditions were identified as patient groups receiving deteriorated care.High patient numbers,including of mildly symptomatic children presenting for health certificates,in combination with increased organisational demands and expected staff outages are priority concerns for the winter.Conclusions Primary care paediatricians offered stable service through the early pandemic but expected strained resources for the upcoming winter.Unambiguous guidance on which children should present to primary care and who should be tested would help to allocate resources appropriately,and this guidance needs to consider age group specific issues including high prevalence of respiratory symptoms,dependency on carers and high contact rates.