Stent supported angioplasty for coronary arterial stenosis following the arterial switch operation.

We present a case of a patient who underwent stent-supported angioplasty for left main bifurcation disease following the arterial switch operation. Prior to this procedure, the patient underwent surgical repair of the left coronary ostium and subsequently coronary artery bypass surgery with an arterial graft to the left anterior descending artery. We could not find a similar report in the literature.     

完全性大动脉转位行大动脉调转术16例麻醉分析

目的总结完全性大动脉转位行大动脉调转术的麻醉处理体会。方法回顾性分析16例完全性大动脉转位行大动脉调转术的麻醉资料。全组患儿均在中低温低流量体外循环下行大动脉调转术及冠状动脉移植术。人室后肌注氯胺酮行基础麻醉，麻醉药物选用芬太尼、咪达唑仑和维库溴铵，术中监测心电图、动脉血压、中心静脉压、左房压、体温、经皮血氧饱和度、呼气末二氧化碳、尿量和血气分析等，主动脉开放后给予多巴胺、肾上腺素、硝酸甘油、米力农、异丙肾上腺素等血管活性药以维持血流动力学稳定，根据中心静脉压和左房压控制体外循环后液体回输速度。结果麻醉经过平稳，无麻醉并发症。术中2例死于重度低心排，不能脱离体外循环。结论完全性大动脉转位患儿年龄小，全身情况及心功能差，充分的术前准备、平稳的麻醉诱导及维持、正性肌力药及血管扩张药的合理使用是顺利完成手术的关键。     

大动脉调转术22例的初步体会

目的：探讨临床应用大动脉调转术的经验。方法：2000年1月至2001年12月对22例患儿施行大动脉调转术。出生后1个月内手术12例，9h至6岁10例。TGA/IVS10例、TGA/VSD11例、DORV(Taussig-Bing)1例。3例施行姑息性大动脉调转术。结果：手术死亡9例，手术死亡率为40.9％。手术死亡原因为：术中无法脱离体外循环3例、低心排综合征4例、心律失常2例。结论：目前施行大动脉调转术的手术死亡率较高。只有通过严格把握手术适应证、充分进行术前准备，改进大动脉移植技术和严密的术后监护处理等综合措施，才可望提高手术成功率。     

Development of quality metrics for ambulatory pediatric cardiology: Transposition of the great arteries after arterial switch operation

Objective

To develop quality metrics (QMs) for the ambulatory care of patients with transposition of the great arteries following arterial switch operation (TGA/ASO).

Design

Under the auspices of the American College of Cardiology Adult Congenital and Pediatric Cardiology (ACPC) Steering committee, the TGA/ASO team generated candidate QMs related to TGA/ASO ambulatory care. Candidate QMs were submitted to the ACPC Steering Committee and were reviewed for validity and feasibility using individual expert panel member scoring according to the RAND‐UCLA methodology. QMs were then made available for review by the entire ACC ACPC during an “open comment period.” Final approval of each QM was provided by a vote of the ACC ACPC Council.

Patients

Patients with TGA who had undergone an ASO were included. Patients with complex transposition were excluded.

Results

Twelve candidate QMs were generated. Seven metrics passed the RAND‐UCLA process. Four passed the “open comment period” and were ultimately approved by the Council. These included: (1) at least 1 echocardiogram performed during the first year of life reporting on the function, aortic dimension, degree of neoaortic valve insufficiency, the patency of the systemic and pulmonary outflows, the patency of the branch pulmonary arteries and coronary arteries, (2) neurodevelopmental (ND) assessment after ASO; (3) lipid profile by age 11 years; and (4) documentation of a transition of care plan to an adult congenital heart disease (CHD) provider by 18 years of age.

Conclusions

Application of the RAND‐UCLA methodology and linkage of this methodology to the ACPC approval process led to successful generation of 4 QMs relevant to the care of TGA/ASO pediatric patients in the ambulatory setting. These metrics have now been incorporated into the ACPC Quality Network providing guidance for the care of TGA/ASO patients across 30 CHD centers.     

一期大动脉调转术治疗完全性大动脉转位

目的:探讨一期大动脉调转术治疗完全性大动脉转位(TGA)的手术适应证及手术效果.方法:2006-05-2007-08对15例患儿[年龄0.5～14(2.51±0.76)个月,体重3～9(3.73±0.86)kg]实施一期大动脉调转手术.15例中室间隔完整TGA(TGA/IVS)13例,均并发房间隔缺损(ASD),同时并发动脉导管未闭(PDA)11例;TGA 并发室间隔缺损(TGA/VSD)2例,均同时并发PDA和ASD.12例并发中度肺动脉高压,3例并发重度肺动脉高压,术前均使用前列腺素E1.手术在全麻、中低温、低流量体外循环下完成.在2大动脉瓣上方横断,将左、右冠状动脉开口移植至肺动脉近端.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个月龄)患儿术后12 h内突发心室颤动死亡,病死率为13.3%.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.13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有较好早期效果.对于年龄超过4个月部分患儿,即使并发肺动脉压,仍可取得满意治疗效果.     

大动脉调转术手术时机对室间隔完整的完全性大动脉转位患儿预后的影响

目的评价大动脉调转术（anerial switch operation，ASO）手术时机对室间隔完整的完全性大动脉转位（transposition of the great arteries with intact ventricular septum，TGA／IVS）患儿预后的影响。方法选取2005年1月至2009年12月在广东省人民医院行ASO的TGA／IVS患儿72例，依手术时年龄分为3组：I组年龄≤7d，Ⅱ组年龄7～14d，Ⅲ组年龄〉14d。回顾性分析各组术后围术期并发症、早期死亡、中期死亡及再次手术干预等情况。结果各组患儿在术后低心排血量综合征、膈肌麻痹、肺不张、肾功能衰竭、切口愈合不良等并发症发生率比较，差异无统计学意义（P〉0．05）。3组早期病死率分别为30-8％（8／26），11．1％（3／27）及0％，3组比较差异有统计学意义（P〈0．005）。共61例出院患儿，随访率为91．8％（56／61）。3组各有1例因术后肺动脉狭窄需再次手术干预，3组再次手术干预率比较，差异无统计学意义（P〉0．05）。I、Ⅱ组患者均无中期死亡，Ⅲ组中期病死率为6．7％（1／15）。结论TGA／IVS患儿ASO术后早期病死率仍较高，中期预后好；手术年龄不是TGA／IVS手术时机的决定性因素。     

快速二期动脉调转术应用的体会

目的:报告采用快速二期动脉调转术(ASO),治疗完全性大动脉转位(TGA)合并完整室间隔畸形的初步体会。方法:2006年1月至2007年1月,采用快速两期ASO治疗4例室间隔完整型TGA患儿。年龄1.2个月~20个月;体重4.0~9.5 kg。一期手术主要采用肺动脉环缩及Blalock-Taussig分流术进行左心室功能训练。至二期ASO前,左心室心肌质量、舒张末径和容积、室间隔和左心室后壁厚度分别较左心室训练前增长了102%~120%、30.3%~37.5%、79.1%~143.6%、5.5%~26.7%和20%~50%。室间隔位置由偏向左心室侧转为偏向右心室侧或居中。二期手术间隔5~19 d。结果:术后早期均无心肌缺血及明显低心排出量综合征表现。存活3例,另1例在二期手术体外循环过程中及术后早期无尿,术后28h死于肾功能衰竭。结论:对于年龄错过新生儿期室间隔完整型TGA患儿,在根治手术前采用肺动脉环缩先对左心室进行功能训练,为一种可选择的、有效的方法。     

Right ventricular hypertrophy and diastolic dysfunction in arterial switch patients without pulmonary artery stenosis

Objective

To assess pulmonary flow dynamics and right ventricular (RV) function in patients without significant anatomical narrowing of the pulmonary arteries late after the arterial switch operation (ASO) by using magnetic resonance imaging (MRI).

Methods

17 patients (mean (SD), 16.5 (3.6) years after ASO) and 17 matched healthy subjects were included. MRI was used to assess flow across the pulmonary trunk, RV systolic and diastolic function, and RV mass.

Results

Increased peak flow velocity (>1.5 m/s) was found across the pulmonary trunk in 14 of 17 patients. Increased RV mass was found in ASO patients: 14.9 (3.4) vs 10.0 (2.6) g/m² in normal subjects (p<0.01). Delayed RV relaxation was found after ASO: mean tricuspid valve E/A peak flow velocity ratio = 1.60 (0.96) vs 1.92 (0.61) in normal subjects (p = 0.03), and E‐deceleration gradients = −1.69 (0.73) vs −2.66 (0.96) (p<0.01). After ASO, RV mass correlated with pulmonary trunk peak flow velocity (r = 0.49, p<0.01) and tricuspid valve E‐deceleration gradients (r = 0.35, p = 0.04). RV systolic function was well preserved in patients (ejection fraction = 53 (7)% vs 52 (8)% in normal subjects, p = 0.72).

Conclusions

Increased peak flow velocity in the pulmonary trunk was often observed late after ASO, even in the absence of significant pulmonary artery stenosis. Haemodynamic consequences were RV hypertrophy and RV relaxation abnormalities as early markers of disease, while systolic RV function was well preserved.     

Cardiorespiratory exercise function after the arterial switch operation for transposition of the great arteries.

AIMS: This study compares the functional outcome and cardiorespiratory response to exercise, in patients who have undergone arterial switch for transposition of the great arteries, with normal controls and patients who have undergone atrial switch operation. METHODS AND RESULTS: Fifteen patients who had undergone arterial switch (mean age 8.5+/-2.9 years) were compared to 32 patients who had undergone atrial switch (9.2+/-1.8 years) and 27 normal controls (8.5+/-2.1 years). Exercise testing was performed on a treadmill and gas exchange measured breath-by-breath. Aerobic capacity, assessed by determination of the ventilatory anaerobic threshold, averaged 91+/-7.8% of normal (95% confidence limits: 92-108%) for arterial switch and 75.1+/-13.1% for atrial switch (P<0.001 patients vs normals). Aerobic exercise function was evaluated by calculation of the slope of oxygen uptake vs exercise intensity. The mean value for this slope was 2.0+/-0.25 for arterial switch, 2.5+/-0.46 for normals and 1.7+/-0.80 for atrial switch (P<0.05; patients vs normals). Efficiency of the pulmonary gas exchange was assessed by calculation of the slope of ventilation vs carbon dioxide output during exercise. This averaged 38.7+/-14.7 for arterial switch, 48.1+/-14.1 for atrial switch and 30.3+/-7.6 for normals (P<0.001; patients vs normals). CONCLUSION: Cardiorespiratory exercise function is at, or slightly below, the lower limit of normal in patients with arterial switch, while the lowest values were observed for those who had undergone atrial switch.     

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.     

Management of anomalous circumflex coronary artery from the neo‐pulmonary artery in an adolescent following neonatal arterial switch operation

A 15‐year‐old male with transposition of the great arteries presented with exertional chest pain. He was found to have a circumflex coronary artery from the neo‐pulmonary artery that had not been transferred during his arterial switch operation. The circumflex coronary artery, fed through collaterals from a re‐implanted single coronary artery, resulted in coronary steal. This report describes a management pathway to treat this rare anomaly. © 2014 Wiley Periodicals, Inc.     

Coronary arterial origins in transposition of the great arteries: factors that affect outcome. A morphological and clinical study

OBJECTIVE: Transfer of the coronary arteries is crucial during the arterial switch operation for transposition, but little attention has been paid to the position of their orifices relative to the valvar sinuses. The objective of this study was to determine the factors which are important for effective transfer and to determine potential surgical significance. DESIGN: Morphological and clinical study. SETTING: Two national centres for neonatal cardiac surgery. PATIENTS: 277 patients with transposition of the great arteries. One group comprised 88 necropsy specimens (ages ranging from 17 weeks of fetal life to 17 years old), and the other comprised 189 children undergoing surgery. The coronary artery orifices were inspected relative to the depth of the aortic sinuses (vertical origin), relative to the commissures between the valvar leaflets (radial origin), and their angle of exit from the aortic wall (angle of origin). The data were compared with the surgical results. RESULTS: In the necropsy specimens, the vertical origin of the arteries was at, or above, the sinutubular junction in 20%, the radial origin was paracommissural in 3%, and the angle of origin was not orthogonal in 7%. Those with high take off and paracommissural origin were all intramural. In the clinical cases, those children with high take off, paracommissural origin or tangential origin had an increased risk at surgery. CONCLUSIONS: In 20% of hearts, high take off, paracommissural orifice, or tangential origin of coronary arteries is found. This may be recognised preoperatively by echocardiography and may cause technical difficulty in transfer during the arterial switch procedure.     

Delineation of intra-atrial reentrant tachycardia circuits after mustard operation for transposition of the great arteries using biatrial electroanatomic mapping and entrainment mapping

INTRODUCTION: Intra-atrial reentrant tachycardia (IART) circuits after Mustard operation remain incompletely understood due to the complex atrial anatomy after extensive surgical procedures. The aim of this study was to delineate IART circuits and their relations to the individual anatomic boundaries in Mustard patients. METHODS AND RESULTS: Twelve patients (10 men and 2 women; age 29 +/- 4.6 years) with atrial tachyarrhythmias after Mustard operation were included in this study. During 14 IARTs and 2 focal atrial tachycardias, electroanatomic mapping and entrainment mapping were performed in both the systemic venous atrium and the pulmonary venous atrium. The latter was accessed via a retrograde transaortic approach. Thirteen IARTs used a single-loop reentrant circuit, and 1 IART used a dual-loop reentrant circuit. Ten (77%) of 13 single-loop reentrant circuits used the tricuspid annulus (TA) as their central barrier. The remaining 3 IARTs rotated around the inferior vena cava (IVC) (n = 2) or ostium of the right upper pulmonary vein (n = 1). In 6 (60%) of the 10 peritricuspid IARTs, both pulmonary venous atrium and systemic venous atrium components of the mid-portion of the TA-IVC isthmus were demonstrated to be part of the reentry. Overall, 12 (86%) of 14 IARTs in 10 patients were successfully ablated by bridging two barriers that constrained the reentrant circuit. Eight (80%) of 10 peritricuspid circuits were abolished by linear ablation connecting the TA to the IVC (n = 4), incisional scar (n = 2), patch (n = 1), and atriotomy (n = 1). CONCLUSIONS: In Mustard patients, the TA serves as the most frequent central barrier of IART. Biatrial electroanatomic mapping combined with entrainment mapping facilitates delineation of IART circuits in relation to their anatomic barriers and enables the design of individual ablation strategies to achieve high success.     

D-Transposition of the Great Arteries after Arterial Switch Operation: Usefulness of 3D-Echocardiography for Left Ventricle Function Evaluation

Objective: The objective of this study was to assess left ventricle (LV) function and remodeling by three-dimensional echocardiography (3DE) in patients who underwent arterial switch procedure (ASO) for transposition of great arteries (TGA) in long-term follow-up. Methods and Results: We studied 54 asymptomatic patients (39 male) who have undergone single-stage ASO for TGA, aged 13.7 ± 4.7 years, with a normal LV ejection fraction (EF), compared to healthy peers. We evaluated LV volume and function in asymptomatic patients with normal ejection fraction by 3DE. All patients had normal EF, measured by modified Simpson’s method (mean 60.9 ± 3.5%) and by 3D method (mean 62.3 ± 3.8%). No statistically significant differences were documented between 2D and 3D measures of age-related LV volumes. Comparison of 3D volumes with reference ones was performed only in pediatric patients (<18 years old). In this subgroup (n = 42) 3D volumes were significantly higher than reference values from the age of 9 years (End-diastolic volume: 9–12 years 79.61 ± 20.29 ml vs.53.52 ± 13.94 ml, p < 0.001; 13–17 years 107.30 ± 23.28 ml vs. 81.78 ± 26.44 ml, p = 0.0038). Conclusions: Children and young adults late after ASO demonstrate normal ejection fraction, but present subclinical signs of ventricular and myocardial remodeling, such as increased LV dimensions, when using 3D echocardiography. Our findings support the usefulness of 3DE to detect LV remodeling precociously.     

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 originsis the key to success. Coronary events after a successful ASO are not uncommon. We describe a rare case of achild 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 LADinto the pulmonary artery. The patient underwent a late LAD reimplantation. This case underscores that evenvery small ostia should be translocated at the time of ASO.     

Left ventricular torsion abnormalities in patients after the arterial switch operation for transposition of the great arteries with intact ventricular septum

Background

The arterial switch operation (ASO) is currently the treatment of choice for infants with transposition of the great arteries (TGA). Little is known, however, about the alteration of anatomic left ventricular (LV) torsional mechanics after the operation. This study sought to evaluate LV torsion in patients of transposition of the great arteries with intact ventricular septum (TGA/IVS) using speckle tracking echocardiography.

Methods

Echocardiographic images were prospectively acquired in 32 infants (age range, 0.5–60 months) who successfully underwent ASO repair at about 1 month of age and in 48 normal controls. They were divided into early and late categories according to the age at the time of the study. The LV peak systolic torsion and systolic twisting and diastolic untwisting velocities were determined by speckle tracking. Mitral inflow velocity obtained by Pulsed-wave Doppler and mitral annular velocities drawn by septal tissue Doppler were also analyzed.

Results

Compared with controls, the early postoperative group (TGA1) had significantly higher septal E/e′ (P = 0.000). In contrast, septal e′ velocity (P = 0.000), LV peak apical rotation (P = 0.01), twist (P = 0.02) and peak untwisting velocity (PUV) (P = 0.001) were lower in patients than in controls. For the normal younger group (Control1), PUV correlated positively with e′ (r = 0.68, P < 0.001). No significant difference in LV twisting and untwisting was noted between the TGA2 and Control2.

Conclusions

Two dimensional speckle tracking echocardiography may sensitively detect impaired LV torsional mechanics in patients with TGA/IVS early after ASO, and the impairment of LV relaxation leads to increased LV filling pressure which is consistent with higher E/e′. However, all patients recovered well thereafter and the overall midterm outcome of ASO is satisfactory.     

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.