Case Report: Surgical Correction of Transposition of the Great Arteries with Aortopulmonary Window

Introduction:Transposition of the great arteries(TGA)with aortopulmonary window is a rare type of congenital heart disease with limited experience.We reported a neonate aged 25 days receiving the arterial switch operation and assisted with extracorporeal membrane oxygenation.Conclusion:TGA with aortopulmonary window can be safely correctly with the arterial switch operation.

Anatomic Correlates of Mitral Systolic Anterior Motion in Transposition of the Great Arteries Following Atrial Switch Operation

Introduction:We sought to investigate whether the development of sub-pulmonic systolic anterior motion(SAM)may be inherent to the anatomy of the the mitral valve(MV)or affected by external factors,such as a dilated right ventricle or chest abnormalities in d-looped transposition of the great arteries post atrial switch operation(d-TGA/AtS).Methods:Analysis was performed of clinical and cardiac imaging studies acquired on 19 adult patients with d-TGA/AtS(age 42±6 years old,56%male)between 2015–2019.Echocardiography data included mitral apparatus anatomy,and CT/MRI data included biventricular dimensions,function,and Haller index(HI)for pectus deformity.Results:Patients with leaflet SAM(n=6)compared to patients without SAM(n=13)had higher MV protrusion height(2.3±0.5 vs.1.5±0.4 cm,p≤0.01)and longer anterior MV leaflet length(3.1±0.4 cm vs.2.6±0.3 cm p≤0.05),when compared to those without.CT/MRI showed higher sub-pulmonic left ventricular ejection fraction(LVEF)in the SAM group(71%±8%vs.54%±7%,respectively).RV size and function,significant chest deformity(HI>3.5),presence of a ventricular lead pacemaker,and septal thickness did not play a role in development of SAM.Conclusions:An elongated mitral apparatus is associated with the development of SAM,and the development of left ventricular outflow tract obstruction(LVOTO),in d-TGA/AtS.LV hyperkinesia is associated with SAM.Systemic RV dimensions,septal thickness,and degree of chest deformity did not differ significantly between subjects with SAM and those without.

Isolation of the Left Subclavian Artery in D-Transposition of the Great Arteries with Right Aortic Arch

Abnormal branching of the aorta associated with the right aortic arch(RAA)has been reported as isolation of left subclavian artery(ILSA),isolation of left common carotid artery,isolation of brachiocephalic artery.ILSA is a rare aortic branch anomaly that originates in the left subclavian artery from the pulmonary artery via ductus arteriosus.Several reports have described ILSA associated with 22q11.2 deletion syndrome and tetralogy of Fallot.Here,we present a very unusual case of RAA with ILSA associated with D-transposition of the great arteries and inferior vena cava interrupted with azygos continuation.

ARTERIAL SWITCH OPERATION FOR COMPLETE D-TRANSPOSITION OF THE GREAT ARTERIES: SURGICAL RESULTS AND ANALYSIS

Objective To review and analyze the surgical results of arterial switch operations in complete D-transposition of the great arteries （TGA）. Methods A total of 113 patients underwent arterial switch operation from Jan. 2001 to Dec. 2005. There were 60 patients with transposition of the great arteries and intact ventricular septum （TGA/IVS） and 53 patients with transposition of the great arteries and ventricular septal defect （TGA/VSD）. The lowest body weight was 2. 3 kg and the smallest age was 6 h. The arterial switch operation was performed under deep hypothermia with circulatory arrest and low-flow perfusion. Results The total mortality was 9. 73%. There were 5 deaths in TGA/IVS （8.3%）, 6 deaths in TGA/VSD （ 11.3% ）. With the development of surgical technique, peri-operative management and cardiopulmonary bypass, the total mortality significantly decreased from 16. 65% in the early times to 5. 5% lately. Conclusion The vital risk of the operation is coronary artery malformation. The incidence of coronary artery malformation is higher in TGA/VSD than in TGA/ [VS. The positional relationship of the great arteries does not affect the results of the operation. The arterial switch operation should be prevented when the pressure ratio between the left and right ventricle is less than 0.6, otherwise it is great likely to cause severe low cardiac output postoperatively.

Transposition of the great arteries-a phenotype associated with 16p11.2 duplications?

Genetic analyses of patients with transposition of the great arteries have identified rare copy number variations,suggesting that they may be significant to the aetiology of the disease.This paper reports the identification of a 16 p11.2 microduplication,a variation that has yet to be reported in association with transposition of the great arteries.The 16 p11.2 microduplication is associated with autism spectrum disorder and developmental delay,but with highly variable phenotypic effects.Autism and attention deficit disorders are observed more frequently in children with congenital heart disease than in the general population.Neonatal surgery is proposed as a risk factor,but as yet unidentified genetic abnormalities should also be taken into account.Thus,congenital heart abnormalities may constitute a part of the phenotypic spectrum associated with duplications at 16 p11.2.We suggest chromosomal microarray be considered part of the diagnostic work-up in patients with transposition of the great arteries.

EVALUATION OF THE SURGICAL PROCEDURE OF CORRECTED TRANSPOSITION OF THE GREAT ARTERIES

ObjectiFe To evaiuate surgicai procedure of corrected transposition of the great arteries(CTGA). Methods Twenty one patients with CTGA were ropaired from September 1990 to December 1996 Eighteen pattents were corrected by biventricular repair. The ventricular septal delect (VSD) was repaired through the right atrium or anatomic left ventricle in 11 patients and through the anatomic right ventricle in 7 patients. There were atrioventricular valvular regurgitation or one side ventricular hypoplasia in 3 patients. Two patients had Fontan operation and one had bidirectional shunt. Results Five patients (45%, 5/11) had complete atrioventricular block (CAVB) in these cases with VSD repaired through the right atrium or left ventricle, and 3 patients died (27%, 3/11). One patient (14%,1/7) had CA VB which the VSD was repaired through the anatomic right ventricle with only one death. Conclusion CAVB could be prevented by VSD rePair through the anatomic right ventricle. CTGA with pulmonary hypertension must be repaired early to prevent pulmonary vascular disease. Fontan or bidirectional shunt were suggested for complex CTGA with valvular regurgitation or ventricular unbalance.

D-Transposition of the Great Arteries:A New Era in Cardiology

Before the 1950s,D-transposition of the great arteries was associated with nearly 90% mortality within the first year of life.The Mustard and Senning procedures resulted in a signifi cant increase in the lifespan of these patients but with notable long-term complications,including arrhythmias,sinus node dysfunction,chronotropic incompetence,and right ventricular systolic dysfunction.The arterial switch operation(first described by Adib Jatene)initially resulted in nearly universal death.However,the use of coronary buttons for coronary artery translocation has improved operative survival dramatically.It is now considered the treatment of choice in patients amendable to the arterial switch operation.Considered an anatomic repair,resulting in concordant ventriculoarterial connections and a systemic left ventricle,the arterial switch operation reduces the incidence of ventricular dysfunction.However,it is also associated with long-term complications,including aortic root dilatation,aortic valve regurgitation,right ventricular outfl ow tract obstructions,coronary artery stenosis/compression,and branch pulmonary artery stenosis.

Noninvasive Assessment of Autonomic Cardiovascular Function in Patients after Arterial Switch Operation for Transposition of the Great Arteries

Background: Children born with transposition of the great arteries (TGA) must undergo corrective surgery for survival, arterial switch being the standard surgical procedure. The sympathetic innervation of the heart may be damaged during the operation. This study was designed to determine whether adults who were born with TGA and who had arterial switch operation (ASO) in infancy exhibit denervation of the heart, measured as heart rate variability (HRV) with electrocardiography (ECG). Methods: Nine patients with transposition of the great arteries (four men and five women;mean age 26 ± 1 years) who underwent the ASO at a mean age of 85 ± 35 days, and nine healthy adults (five men and five women;mean age 26 ± 2) were included in the study. Cardiac autonomic nerve function was determined by the variation in RR intervals during maximal deep breathing, monitored by continuous ECG. The mean values were calculated for each group from six inspirations (I) and expirations (E), and the E:I ratios were calculated. Results: The E:I ratio did not differ between patients with an arterial switch and healthy controls (P?= 0.161). Two patients had signs of denervation of the heart up to 30 years after the arterial switch operation. Conclusions: Reinnervation of the heart?may take place in patients who have undergone the ASO in infancy, and these patients would not necessarily suffer from autonomic dysfunction. The HRV, measured by ECG, has the potential to identify arterially switched patients at risk of developing silent myocardial ischemia.

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.

Correction of Corrected Transposition of the Great Arteries Associated with Cardiac Anomalies

Reported in this paper are 8 cases of corrected transposition of the great arteries associated with anomalies. All the patients underwent the repair of the ventricular septal defect and atrial septal defect, the replacement of left-side tricuspid valve, the pulmonary valvotomy and the placement of extra-cardiac conduit to the pulmonary trunk respectively with no hospital death after operation.The paper also described the selection of the heart incision, the prevention of injury of the conduction system and indication of the placement of extra-cardiac conduit.

Implantation of an S-ICD in a Patient with a DDD Pacemaker and Congenitally Corrected Transposition of the Great Arteries

Subcutaneous implantable cardioverter-defi brillator(S-ICD)therapy has become a viable alternative to conventional transvenous ICD implantation.Patients with congenitally corrected transposition of the great arteries(ccTGA)have a high risk of sudden cardiac death due to malignant arrhythmia.The interaction between the S-ICD system and the transvenous pacemaker system is not fully understood.We report a case of S-ICD implantation in a patient with ccTGA and a DDD pacemaker.The patient was a 30-year-old man with a previously placed pacemaker with diagnoses of congenital heart disease,ccTGA(SLL),left atrioventricular valve insuffi ciency,and third-degree atrioventricular block.He presented with an out-of-hospital cardiac arrest,and an S-ICD was implanted to prevent sudden cardiac death.Defi brillation checks were performed successfully.We tested the compatibility of the DDD pacemaker with the S-ICD and found that there was no interference between them.In conclusion,an S-ICD system is a reasonable and safe option in patients with ccTGA.

Congenitally Corrected Transposition of the Great Arteries: Conduction Anomalies: A Case Report

Introduction: Congenitally corrected transposition of the great arteries (CCTGA) is a rare heart disease that encompasses an atrioventricular and ventriculoarterial discordance and accounts for less than 1 percent of congenital heart diseases. Objective: To present an atypical case of a man with complex congenital heart disease and conduction anomalies. Case Presentation: This is a case of a 34-year-old patient who came to the hospital with 1 week of dyspnea on exertion and episodes of lipothymia. The patient was referred to our hospital after an electrocardiogram from his primary care with evidence of blocked atrial fibrillation. During the initial evaluation blocked atrial fibrillation was confirmed with a rescue ventricular rate of 38 bpm. A magnetic resonance confirmed the presence of the atrioventricular and ventriculoarterial discordance, the aorta had a left anterior position, perimembranous ventricular septal defect with a right to left shunt, biventricular systolic dysfunction, moderate tricuspid, and mitral regurgitation, interventricular septal intramyocardial and biatrial fibrosis, left atrial dilation, and dilation of the pulmonary artery. After pacemaker placement, the patient has an improvement in his clinical symptoms and quality of life. Conclusions: Cardiac arrhythmias are CCTGA’s leading cause of death, mostly ventricular tachycardia, and atrial fibrillation. Right bundle branch block is a previously unreported and potentially very rare presentation of this disease. This, added to the fact that our patient was diagnosed at an advanced age, but without symptoms of heart failure, makes him an atypical case of CCTGA, with new potential treatment options.

A 16-day old baby with transposition of great arteries and interrupted aortic arch: a case report

We report a 16-day-old boy who had transposition of great arteries combined with interrupted aortic arch (IAA). The boy developed cyanotic lips and body bruising 2 h after birth. Color Doppler sonography showed transposition of great arteries. The patient underwent total surgical correction of the transfection and defects. We summarized our experience in the perioperative management of the patient, including maintenance of body temperature, close intraoperative monitoring, anesthesia management, cardiopulmonary bypass (CPB) management and so on.

Diagnosed corrected transposition of great arteries after cesarean section:A case report

BACKGROUND Corrected transposition of the great arteries(cTGA) is a cardiac malformation in which the ventricular and arterial-ventricular positions in the heart are doubly reversed.In general,this defect puts a load on the systemic circulation and causes heart failure,resulting in a poor prognosis.This article reports a case of cTGA detected in a patient with post-caesarean pregnancy who had undergone elective caesarean section and was experiencing an episode of acute heart failure.CASE SUMMARY This was the case of a 36-year-old gravida 3 para 1 woman.No problems were noted in the puerperal course following the previous pregnancy.The current pregnancy was also uneventful.An elective caesarean section was performed and the patient was discharged from the hospital 7 d after the operation.On postoperative day 18,the patient became aware of breathing difficulty and presented at a nearby clinic,where she was referred to our institution after bilateral pleural effusions were detected.She was then diagnosed with acute heart failure after noting the presence of a prominent pedal oedema and SpO_(2) 91%(supine position and room air);the patient was promptly hospitalised for close examination and treatment.Although chest computed tomography revealed the presence of cTGA,no other cardiac malformations were observed.Owing to improvements in both the pedal oedema and pleural effusions,the patient was discharged on day 9.CONCLUSION Close examination should be performed on the premise of congenital cardiac malformation when heart failure symptoms are noted during perinatal control.

Systematic review and meta-analysis of outcomes of anatomic repair in congenitally corrected transposition of great arteries

BACKGROUND Treatment of congenitally corrected transposition of great arteries(cc-TGA)with anatomic repair strategy has been considered superior due to restoration of the morphologic left ventricle in the systemic circulation.However,data on long term outcomes are limited to single center reports and include small sample sizes.AIM To perform a systematic review and meta-analysis for observational studies reporting outcomes on anatomic repair for cc-TGA.METHODS MEDLINE and Scopus databases were queried using predefined criteria for reports published till December 31,2017.Studies reporting anatomic repair of minimum 5 cc-TGA patients with at least a 2 year follow up were included.Metaanalysis was performed using Comprehensive meta-analysis v3.0 software.RESULTS Eight hundred and ninety-five patients underwent anatomic repair with a pooled follow-up of 5457.2 patient-years(PY).Pooled estimate for operative mortality was 8.3%[95%confidence interval(CI):6.0%-11.4%].0.2%(CI:0.1%-0.4%)patients required mechanical circulatory support postoperatively and 1.7%(CI:1.1%-2.4%)developed post-operative atrioventricular block requiring a pacemaker.Patients surviving initial surgery had a transplant free survival of 92.5%(CI:89.5%-95.4%)per 100 PY and a low rate of need for pacemaker(0.3/100 PY;CI:0.1-0.4).84.7%patients(CI:79.6%-89.9%)were found to be in New York Heart Association(NYHA)functional class I or II after 100 PY follow up.Total re-intervention rate was 5.3 per 100 PY(CI:3.8-6.8).CONCLUSION Operative mortality with anatomic repair strategy for cc-TGA is high.Despite that,transplant free survival after anatomic repair for cc-TGA patients is highly favorable.Majority of patients maintain NYHA I/II functional class.However,monitoring for burden of re-interventions specific for operation type is very essential.

D-Transposition of Great Arteries in a Primigravida of 35 Years Old-Case Report and Literature Review

This is a case report with discussion of the maternal-fetal outcome of pregnant women with uncorrected transposition of the great arteries （TGA） associated with pulmonary arterial hypertension and a large ventricular septal defect. This case draws attention to the severity of the pathology and maternal symptoms prior to gestation, and how an adequate clinical management of both obstetrics and cardiology can provide a favorable outcome for mother and fetus.

Comparative analysis of early and middle outcomes of the arterial switch operation in children with complete transposition of the great arteries with ventricular septal defect and severe pulmonary artery hypertension

Background The best age for the arterial switch operation （ASO） in complete transposition of great arteries with ventricular septal defect is usually considered to be within six months. This is because of severe pulmonary arterial hypertension and pulmonary arterial obstructive pathological changes. There are few reports on ASO surgery in children older than three years old. Methods We studied 41 children, including 24 males and 17 females, from January 2010 to December 2011. They were divided into three groups by operation age; 15 patients were 〈1 year old, 13 were 1-3 years old, and 13 were 〉3 years old. Associated cardiac abnormalities included patent ductus arteriosus in six cases, atrial septal defect in five cases, and mitral regurgitation in two cases. All the patients had echocardiography before the operation. Seventeen patients underwent a coronary computed tomography examination and five patients underwent right heart catheterization. All ASO surgeries were performed under inhalation anesthesia and hypothermic cardiopulmonary bypass. Results Three operative deaths occurred. Two were in the 〈1 year old group, who died from severe postoperative low cardiac output. The other was two years old and died of postoperative multiple organ failure. There was no significant difference in postoperative mortality and the recent mid-term survival rate among the three groups. Thirty-eight cases were followed up for an average of 11.2 months, ranging 6-20 months. One seven years old patient died of acute diarrhea and electrolyte disturbance arrhythmia caused by food poisoning. Three patients more than three years old still had residual pulmonary arterial hypertension. Conclusion Children older than three years old can still undergo the ASO procedure, but residual pulmonary hypertension is present.

超声心动图在矫正型大动脉转位患儿二期双调转术中的应用价值

目的 探讨超声心动图在矫正型大动脉转位(CTGA)患儿二期双调转术(DS)中的应用价值。方法 选取于外科行二期DS的17例CTGA患儿,分析超声心动图对其肺动脉环扎(PAB)术前诊断情况,比较PAB术前、后左心室形态及血流动力学情况;应用超声心动图观察DS术前、后左心室血流动力学变化。结果 17例CTGA患儿,PAB术前超声心动图准确诊断15例(88.24%);误诊2例(11.76%),均误诊为房室连接不一致型右心室双出口。PAB术后肺动脉内径、三尖瓣反流束宽均缩小,左心室舒张末期内径、左心室收缩末期内径、左心室后壁舒张末期厚度、室间隔舒张期厚度、左心室质量及肺动脉血流速度均明显增大,反流程度减轻,与PAB术前比较差异均有统计学意义(均P<0.05)。DS术后左心室心肌做功指数(MPI)明显增大,右心室MPI、左心室射血分数均明显减低,与DS术前比较差异均有统计学意义(均P<0.05);6例合并心功能不全(3例死亡,3例随访中)患儿中,室间隔完整3例,合并室间隔缺损3例(缺损直径分别为0.13 cm、0.15 cm、0.18 cm);11例心功能正常患儿室间隔缺损直径平均(0.37±0.18)cm。结论 超声心动图可准确诊断CTGA,CTGA患儿PAB术后左心室质量明显提高,可行DS;DS术后近期易合并心功能不全及主动脉瓣反流。

Surgical Treatment of Complete Transposition of the Great Arteries in Newborn

INTRODUCTION Complete transposition of the great arteries （TGAs）is a common cyanotic congenital heart defect,with an incidence rate of 0.02%,accounting for 5-7% of congenital heart diseases.[1] After birth,infants with TGA largely rely on ventricular septal defect （VSD）,atrial septal defect （ASD）,and patent ductus arteriosus （PDA）to sustain life.Without systemic-pulmonary shunt and surgical correction,these infants will probably die on the day after birth.

动脉调转术后新主动脉瓣反流及根部扩张成因、转归及预防

动脉调转术(ASO)是治疗完全性大动脉转位(TGA)及Taussig-Bing畸形(TBA)的首选术式。ASO手术发展至今,大量术后患儿已至成年,因此ASO的晚期并发症逐渐引起关注。新主动脉瓣反流(NAVR)和新主动脉根部扩张(NARD)是ASO术后再次手术的第二大原因。本文拟对现有研究进行回顾,总结ASO术后NAVR及NARD的原因和预后,为临床实践提供参考。