A 63-Year-Old Male with D-Transposition of the Great Arteries Who Had an Early Form of the Arterial Switch Operation

We describe a 63-year-old male who appears to have undergone an early form of the arterial switch operation for D-transposition of the great arteries performed in the mid-1960s.We review the clinical and imaging data that support our conclusion.He had a diagnostic cardiac catheterization which demonstrated severe pulmonary hypertension responsive to epoprostenol and oxygen.Our case may represent one example of the experimental surgical work done prior to Dr.Adibe Jatene’s description of thefirst successful arterial switch performed in 1975.

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.

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.

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.

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.

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.

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.

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.

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.

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.

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.

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.

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.

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.

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.

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.

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.

Quantitative Analysis of Microstructural Components of Human Coronary Arteries

The anterior interventricular branch and circumflex of the left coronary artery,the right coronary artery,the internal thoracic artery,and the great saphenous vein were excisedfrom 7 human adult autopsy subjects.Paraffin slices of these specimens were prepared and stainedwith different dyes in order to reveal the elastin,collagen and smooth muscle in the walls of thesevessels.The slices were scanned with a microspectrophotometer and the relative contents of thecomponents mentioned above were represented with the mean absorbance respectively.It was found that no significant difference existed in the contents of the above mentioned com-ponents between the left and right coronary arteries(P】0.05).The internal thoracic artery con-tains relatively higher elastin than the others(P【0.01).The contain a highcollagen/elastin ratio(P【0.01),which makes the coronary arteries be suitable to carry out thespecial functional needs of the heart.