Coronary Artery Anomalies in D-Transposition of the Great Artery Following Arterial Switch Operation

Background:The survival rate of patients following arterial switch operation(ASO)exceeds 95%,but coronary artery anomalies(CAA)contribute to a 2%incidence of sudden cardiac arrest later in life.Therefore,we aimed to assess abnormal findings of coronary arteries in post-ASO patients.Methods:Coronary computed tomography angiography(CCTA)is performed on post-ASO patients who meet institutional criteria.Intraoperative findings of coronary artery patterns were retrospectively reviewed and categorized using the Leiden classification system.Coronary artery anomalies were detected by CCTA and associations with coronary artery compromise were explored.Results:Forty-three patients who had CCTA with a median age of 15.6 years(12–21.3 years)were included in the study.Unusual coronary patterns were identified in 20(46%)patients before ASO.CCTA identified 25 CAA in 22 patients(eleven with prepulmonic course,nine with interarterial course,three with acute take-off angle,and two with significant stenosis).Postoperative CAA was more common in patients with unusual coronary patterns(90%vs.17.4%;p<0.001).Nine patients experienced chest pain and two patients required coronary artery bypass graft.A common ostium of RCA and LAD or LMCA were associated with significant chest pain(OR 14.3%,95%CI 2.5 to 82.3).Conclusions:Coronary artery anomalies in post-ASO are common.All post-ASO patients should have coronary artery imaging before participating in competitive sport and when they reach adolescence.Patients with unusual preoperative coronary artery patterns should undergo coronary artery imaging when feasible.Follow-up imaging studies are indicated in patients with post-operative coronary artery abnormalities.

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.

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.

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.

Systemic right ventricle complications in levo-transposition of the great arteries: A case report and review of literature

BACKGROUND Congenitally corrected levo-transposition of the great arteries(L-TGA)is a congenital heart disease in which the ventricles and great arteries are transposed from their typical anatomy.In L-TGA,the double discordance,atrioventricular and ventriculoarterial,create an acyanotic milieu which allows patients to survive their early decades,however,progressive systemic right ventricle(sRV)dys-function creates complications later in life.sRV dysfunction and remodeling predisposes patients to intracardiac thrombus(ICT)formation.CASE SUMMARY A 40-year-old male with L-TGA presented with symptoms of acute decom-pensated heart failure.In childhood,he had surgical repair of a ventricular septal defect.In adulthood,he developed sRV dysfunction,systemic tricuspid valve(sTV)regurgitation,and left-bundle branch block for which he underwent cardiac resynchronization therapy.Transthoracic echocardiogram showed a sRV ejection fraction of 40%,severe sTV regurgitation,and a newly identified sRV ICT.ICT was confirmed by ultrasound-enhancing agents and transesophageal echocardio-graphy.Our patient was optimized with guideline-directed medical therapy and diuresis.Anticoagulation was achieved with a vitamin K antagonist(VKA)and he was later referred for evaluation by advanced heart failure and heart transplant services.CONCLUSION Anticoagulation with VKA is the mainstay of treatment in the absence of conclusive data supporting direct oral anticoagulant use in ICT in patients with congenital heart disease.This case illustrates the natural history of L-TGA and highlights the importance of surveillance and monitoring with dedicated cardiac imaging to identify complications.

Calcific aorta and coronary artery： two cases of calcific ascending aorta and descending aorta

Calcific aorta is a disease of old age and is an independent risk factor for morbidity and mortality. Here, we present two patients with calcific aorta at different levels. One with a descending porcelain aorta, and modified Bentall＇s procedure was done. Second is a patient who is having a calcific ascending aorta and coronary artery. Coronary artery bypass grafting from left internal mammary artery to left anterior descending was done for the patient. The calcification and its morbidity had been discussed briefly.

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.

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.

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.

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.

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.

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.

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.

A Rare Case of Late LAD Reimplantation after Arterial Switch Operation

Arterial switch operation(ASO)is a complex neonatal operation in which transfer of the coronary arteries origins is the key to success.Coronary events after a successful ASO are not uncommon.We describe a rare case of a child who underwent an ASO in the neonatal period with one coronary(LAD)described as atretic left in place.At age seven,he developed myocardial ischemia due to retrograde flow with a steal phenomenon from the LAD into the pulmonary artery.The patient underwent a late LAD reimplantation.This case underscores that even very small ostia should be translocated at the time of ASO.

Systemic venous atrium stimulation in transvenous pacing after mustard procedure

We present the case of a young woman corrected with a Mustard procedure undergoing successful transvenous double chamber pacemaker implantation with the atrial lead placed in the systemic venous channel. The case presented demonstrates that, when the systemic venous atrium is separate from the left atrial appendage, the lead can be easily and safely placed in the systemic venous left atrium gaining satisfactory sensing and pacing thresholds despite consisting partially of pericardial tissue.

Six-Year Outcome after Valve Replacement and Resynchronization Therapy in TGA Patient

Patients with complete transposition of the great arteries(TGA)treated by the Senning procedure have a higher risk of developing heart failure due to:a)additional work load of the systemic(morphologic right)ventricle(sRV),b)arrhythmias,mainly caused by surgical implications at the atria as well as c)worsening of systemic tricuspid regurgitation.We present a unique case of a female patient who developed all these complications,who was successfully treated and was able to carry out a twin pregnancy.This breakthrough approach was based on:1.detecting reversibility potential of myocardial systolic dysfunction in a severe valvular lesion combined with continuous systemic afterload settings and permanent tachyarrhythmia,and 2.prevention of subsequently iatrogenic worsening of systemic ventricular function due to permanent pacing.Surgical replacement of systemic tricuspid valve(sTV)and cardiac resynchronization device(CRT)implantation after nodal ablation resulted in recovering of the systolic function and a positive remodeling of the sRV.The reversal of a further decline in systolic function was achieved by permanent arrhythmia control,synchronous pacing with epicardial leads of CRT,sTV replacement as well as echocardiographic monitoring during pregnancy to determine the right time for delivery.Two years after delivery,the patient remains in NYHA Class I.

Quantitative analysis of host cells growing into canine homograft valved aortic and pulmonary artery

Background Cryopreserved conduit valved homografts （CVH） have been widely used in surgical treatment of cardiac disease. This study aimed to determine the extent of host cell ingrowth and the durability and immunogenicity of CVH,and to compare the performance of CVH stored at 4℃ and CVH cryopreserved in liquid nitrogen at -196℃.Methods Heterotopic transplants of canine CVH stored at 4℃ （n=14） and cryopreserved in liquid nitrogen （n=14） were made onto the abdominal aorta of recipient dogs. Animals were sacrificed at 7 and 15 days and at 1, 3, 6, 9, and 12months after transplantation to excise the implanted CVHs. Tissue DNA extraction and quantitative polymerase chain reaction （PCR） were performed to calculate the ratio of donor cells and host cells in the CVH. The tissue viability of CVH after implantation was analyzed by detecting alkaline fibroblast growth factor 2 （FGF-2） using immunohistochemical staining and by observation under transmission electron microscope and scanning electron microscope.Results All the animals survived and recovered well. There were few repopulating host cells （0.04-0.83%） in the implanted CVH at 7 or 15 days. The ratio of ingrowing host cells into the CVH continued rising after implantation and reached 40%-47% in the 12th month postoperation. Histology, transmission electron microscopy and FGF-2immunohistochemical staining indicated that fibroblasts and the host＇s endothelial cells were the main cellular elements invading the CVH. There were no significant differences in results between CVH stored at 4℃ and CVH cryopreserved in liquid nitrogen.Conclusions Host cells growing into CVH are very important for maintaining the long-term structure and function of the implanted CVH. There is no significant difference between CVH storing at 4℃ or in liquid nitrogen in regard to the ingrowth of host cells or of morphologic features after CVH allografting.

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.