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.

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.

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.

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.

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.

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.

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。

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.

Adult Congenital Heart Disease in the Veteran Population:A Case-Based Report

Simple forms of congenital heart disease can allow patients to go undiagnosed until they reach adulthood.Furthermore,improvements in care of patients with complex congenital heart disease are now allowing most patients to reach adulthood.As some patients with adult congenital heart disease can remain asymptomatic until later in life,it is possible for them to serve in the military and eventually fall under the care of Veterans Administration(VA)providers.Therefore it is important for providers,especially cardiologists at VA centers,to have fundamental understanding of the management of adult congenital heart disease.This article provides multiple cases of adult congenital heart disease experienced at a single VA medical center and reviews the anatomy,physiology,and surgical management of each condition.

一期大动脉调转术的主要问题及对策

目的总结一期大动脉调转术（ASO）治疗完全性大动脉转位（TGA）存在的主要问题并探讨其应对策略。方法对2006年5月至2007年8月收治的14例患儿实施一期大动脉调转手术。患儿平均年龄（2.6±0.8）个月（0.5-14个月）;平均体重（3.85±1.30）kg（3-9kg）。室间隔完整TGA（TGA/IVS）12例,均合并房间隔缺损（ASD）,同时合并动脉导管未闭（PDA）11例;TGA合并室间隔缺损（TGA/VSD）2例,均同时合并PDA和ASD。11例合并中度肺动脉高压,3例合并重度肺动脉高压,术前均使用前列腺素E1（PGE1）。手术在全麻、中低温、低流量体外循环下完成。在主动脉、肺动脉瓣上方横断,将左右冠状动脉开口移栽至肺动脉近端。经肺动脉分叉下方,吻合肺动脉近端和升主动脉远端。用自体心包修复主动脉近端缺失部分,并在新主动脉开放后吻合主动脉近端和肺动脉远端。结果3例TGA/IVS患儿术后肺部感染,2例（分别为13个月、3个月）患儿分别于术后10h及12h突发室颤死亡。未发生与冠状动脉移植相关并发症。术前肺动脉高压患儿,特别是重度肺动脉高压,术后肺动脉压力均明显下降。12例术后顺利恢复出院。随诊3-12个月,全部患儿心功能恢复良好,无远期并发症和死亡。结论一期大动脉调转手术对于TGA有较好早期治疗效果。针对术中主要问题作出适当的手术应对措施,可以获得满意治疗效果。

年龄超过3周室间隔完整大动脉转位婴儿的外科治疗策略

目的:讨论年龄超过3周的室间隔完整大动脉转位婴儿的外科治疗策略。方法:自2000年3月至2005年3月期间,我院共收治年龄超过3周的室间隔完整大动脉转位的婴儿共14例,平均年龄(74.6±50.8)天,平均体重(4.49±1.02)kg。合并畸形包括房间隔缺损13例,动脉导管未闭11例。9例行一期动脉调转术,5例行分期动脉调转术。结果:平均体外循环时间(150±41)min,平均主动脉阻断时间(85±22)min,12例存活,2例死亡。结论:对于年龄超过3周的室间隔完整大动脉转位患儿,参考术前超声和术中测压情况合理选择一期和分期行动脉转位术,治疗效果满意。

107例矫正型大动脉转位手术治疗中期结果分析

目的:报告并分析我院107例手术治疗矫正型大动脉转位(CCTGA)合并心内畸形患者的中期随访结果。方法:1996-01至2005-12,在我院手术治疗CCTGA合并心内畸形患者107例,其中男72例,女35例;年龄1.5～50岁,平均(11.5±8.4)岁;平均体重(31.6±4.7)kg。81例左位心,23例右位心,3例中位心。手术方式包括传统双心室功能矫治75例;单心室分期矫治14例;双心室解剖矫治18例。结果:术后早期死亡5例,死亡率为4.7%。传统双心室功能矫治死亡率为4.0%;单心室分期矫治死亡率为0%;心房动脉双调转手术死亡率为0%;心房心室双调转手术死亡率40.0%。手术死亡危险因素为复杂心内畸形合并肺动脉瓣狭窄(P<0.01),与其他危险因素相比差异有统计学意义。共随访99例,随访率为92.5%。随访期间死亡12例,死亡率为12.1%;其中10例(83.3%)属于传统双心室功能矫治患者,2例(16.7%)属于单心室矫治患者。双心室解剖矫治患者没有死亡。随访期死亡危险因素为传统功能矫治手术方式(P<0.05),心房心室双调转手术(P<0.05),与其他手术方式相比差异有统计学意义。结论:CCTGA传统手术方法的中期结果是令人失望的。对于适合单心室矫治的CCTGA病例,手术可以取得较好的中期结果。心房动脉双调转手术中期的死亡率低,并发症少,其手术结果是值得肯定的。

REV手术在复杂先天性心脏病中的应用

目的:介绍REV手术在治疗合并室间隔缺损(ventricular septal defect,VSD),或同时合并肺动脉狭窄(pulmonary stenosis,PS)的复杂型大动脉转位(transposition of the great arteries,TGA)和矫正型大动脉转位中的经验。方法:2012年5—7月中南大学湘雅医院心胸外科收治了4例TGA患者。2例单纯合并VSD的患者(年龄分别为1个月和3个月)采用动脉调转术治疗。1例同时合并VSD及PS的患者(年龄6个月)采用REV手术,另1例同时合并VSD及PS的矫正型大动脉转位患者(年龄7岁)采用Senning术+REV术进行矫正。结果:4例患者痊愈出院,单纯REV手术治疗的患者体外循环时间及主动脉阻断时间最短。分别随访1个月和3个月,超声心动图检查提示心脏畸形矫治满意,心脏功能恢复良好。结论:REV手术对治疗合并VSD,PS的TGA是一种较为简便的手术方法。

大动脉转位的磁共振成像

目的 探讨磁共振成像 (MRI)对先天性大动脉转位 (TGA)的诊断价值。方法 本组病例 14例 ,男 8例 ,女 6例 ,年龄 2月～ 12岁 ,平均 5 .4± 3.6岁。应用超导磁共振仪 ,选用心电图门控自旋回波技术 (SE)、电影磁共振 (cine MRI)及磁共振造影 (MRA)进行诊断 ,所有病例均做二维彩色多普勒超声心动图检查 ,10例同时做心血管造影 (Angio)检查 ,2例经手术治疗。结果 MRI准确诊断本组病例包括完全性大动脉转位 13例 (92 .8% ) ,纠正性大动脉转位 1例(7.2 % )。 14例中 ,合并室间隔缺损 12例 (85 .6 % ) ,室间隔完整 2例 (14.4% ) ,右室双出口 4例 (2 8.8% ) ,心脏位置异常 5例 (35 .7% ) ,肺动脉狭窄 6例 (4 3.2 % )。14例中 ,10例经Angio或 /和手术证实。结论 MRI对TGA的诊断具有重要临床价值 。

保留肺动脉瓣的改良REV手术治疗复杂型大动脉转位——附3例报道及文献综述

目的 介绍一种改良REV手术治疗合并室间隔缺损（VSD）、肺动脉瓣狭窄（PS）的复杂型大动脉转位或右心室双出口（DORV）。方法 2005年9月～2006年2月我院收治3例复杂型先天性心脏病患者，在经典REV手术的基础上，我们改良了REV手术，保留了肺动脉瓣和瓣环进行手术治疗。结果 2例痊愈，1例同时用体外膜式氧合器（ECMO）进行抢救后死亡。痊愈患者分别随访4个月和1个月，超声心动图检查提示肺动脉瓣跨瓣压差分别为15mmHg和5mmHg，结果满意。结论 保留肺动脉瓣的改良REV手术对治疗合并VSD、PS的复杂大动脉转位是一种较为理想的手术方法。

产前超声筛查在胎儿完全性大动脉转位的诊断价值及意义

目的 探讨产前超声心动图对胎儿完全性大动脉转位的诊断价值。方法 回顾性分析2010年-2014年产前诊断16例胎儿完全性大动脉转位的超声心动图表现,总结大动脉转位胎儿在四腔心切面、左右流出道切面、三血管切面、主动脉弓切面的声像图特征。结果 16例胎儿完全性大动脉转位在流出道切面上：均显示两条大动脉前后平行走行。主动脉弓切面上：15例曲度都有不同程度的增大,1例曲度基本正常。三血管切面上均出现异常：12例仅显示两条血管,另4例排列异常。四腔切面上：14例正常,仅有2例出现异常（1例合并右心室发育不良,1例合并心内膜垫缺损）。结论 完全性大动脉转位的产前超声表现具有一定的特异性。产前超声多个切面联合扫查有助于完全性大动脉转位的产前诊断。

矫正型大动脉转位形态三尖瓣置换术及中远期结果

目的探讨矫正型大动脉转位（cTGA）患者行形态三尖瓣置换术的手术适应证及其术后中远期结果。方法1997年9月至2007年9月,阜外心血管病医院收治cTGA患者18例,男15例,女3例;年龄16~51岁（33.3±12.8岁）,体重47~90 kg（60.9±14.7 kg）。单纯形态三尖瓣关闭不全10例,合并室间隔缺损3例,合并室间隔缺损及肺动脉瓣狭窄2例,室间隔缺损修补术后形态三尖瓣关闭不全2例,形态三尖瓣置换术后机械瓣功能不良1例。术前形态右心室射血分数为56.2%±11.6%,心功能分级（NYHA）Ⅱ级12例,Ⅲ级6例。18例cTGA患者均施行形态三尖瓣置换术。术后随访患者的心功能、形态右心室射血分数等指标。结果手术死亡1例,死于术后低心排血量综合征。术后发生瓣周漏2例,分别于术后7 d和30 d行瓣周漏修补术后治愈。随访16例,随访时间57.0±40.7个月;随访时形态右心室射血分数与术前比较差异无统计学意义（52.8%±9.2%vs.56.2%±11.6%;t=2.062,P〉0.05）,心功能分级（NYHA）Ⅰ~Ⅱ级12例,Ⅲ级4例,与术前比较心功能分级Ⅲ级者所占百分比差异无统计学意义（χ2=1.532,P〉0.05）。结论cTGA形态三尖瓣置换术能防止形态三尖瓣关闭不全对形态右心室功能的进一步损害;其中远期结果满意;随访期间,形态右心室功能基本正常。