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This case report discusses a 42-year-old male with dextro-transposition of the great arteries (D-TGA) status post Mustard repair and sick sinus syndrome status post dual-chamber pacemaker implant, who developed symptomatic superior vena cava (SVC) baffle stenosis. He was treated with a combined pacemaker extraction and subsequent SVC baffle stenting. The case highlights the complexities of treating SVC baffle stenosis in the presence of cardiac implantable devices and demonstrates the efficacy of this combined approach. Furthermore, the authors delve into the intricacies of D-TGA, its surgical history, and the long-term complications associated with atrial switch procedures. [ABSTRACT FROM AUTHOR]

Objective: Superior venous system stenosis (superior vena cava (SVC) - right subclavian vein - innominate vein - left subclavian vein) is a clinical situation that frequently appears in patients with long-term implanted cardiac stimulation devices, due to venous system thrombosis and in those with congenital heart disease who need corrective surgery, due to chronic complications inherent to surgical techniques. In clinical practice, venous system stenosis may manifest as a SVC syndrome. In many cases, we are not able to correct stenosis or obstructions, since it is impossible to cross them. In this article, we describe the surgical technique that we have implemented in our hospital to solve this challenge, especially in those patients with pacing/defibrillation devices who present with this pathology. Our objective was to perform an extraction of the pacemaker and defibrillation electrodes, to allow the passage of a support wire to achieve the implantation of the endovascular stent(s) to correct the SVC syndrome. Methods: We present a retrospective series of six consecutive patients with SVC syndrome studied in a single center from 2012 to 2021. Three of them presented with thrombosis related to pacing or defibrillation electrodes and the other three presented with complications derived from Mustard or Senning techniques in patients with pacemakers and D-transposition of the great arteries. Results: In all cases, a complete re-vascularization of the SVC system was achieved using a stent, and new leads could have been implanted through it. Combined treatment of lead extraction and endovascular stent implantation corrected the syndrome in all cases. Conclusions: Angioplasty and stenting of the central venous system is a standardized technique with validated results, in acute, for the recanalization of chronic occlusions secondary to transvenous devices. [ABSTRACT FROM AUTHOR]

Atrial switch procedures (Senning and Mustard) for transposition of the great arteries have largely been abandoned for arterial switch procedures. The number of surviving patients who have undergone atrial switch procedures is declining. We present a case of the oldest known survivor (aged 67 years) of the Mustard procedure. (Level of Difficulty: Beginner.)

Systemic right ventricular failure after physiologic repair for dextro‐transposition of the great arteries can be managed with durable mechanical circulatory support; however, the right ventricular morphology, such as intervening papillary muscles, presents challenges to inflow cannula positioning. Papillary muscle repositioning is an innovative technique to circumvent obstructive anatomy. [ABSTRACT FROM AUTHOR]

Background Little is known about the cause of death (CoD) in patients with transposition of the great arteries palliated with a Mustard or Senning procedure. The aim was to describe the CoD for patients with the Mustard and Senning procedure during short‐ (20 years) follow‐up after the operation. Methods and Results This is a retrospective, descriptive multicenter cohort study including all Nordic patients (Denmark, Finland, Norway, and Sweden) who underwent a Mustard or Senning procedure between 1967 and 2003. Patients who died within 30 days after the index operation were excluded. Among 968 patients with Mustard/Senning palliated transposition of the great arteries, 814 patients were eligible for the study, with a mean follow‐up of 33.6 years. The estimated risk of all‐cause mortality reached 36.0% after 43 years of follow‐up, and the risk of death was highest among male patients as compared with female patients (P=0.004). The most common CoD was sudden cardiac death (SCD), followed by heart failure/heart transplantation accounting for 29% and 27%, respectively. During short‐, mid‐, and long‐term follow‐up, there was a change in CoD with SCD accounting for 23.7%, 46.6%, and 19.0% (P=0.002) and heart failure/heart transplantation 18.6%, 22.4%, and 46.6% (P=0.0005), respectively. Conclusions Among patients corrected with Mustard or Senning transposition of the great arteries, the most common CoD is SCD followed by heart failure/heart transplantation. The CoD changes as the patients age, with SCD as the most common cause in adolescence and heart failure as the dominant cause in adulthood. Furthermore, the risk of all‐cause mortality, SCD, and death attributable to heart failure or heart transplantation was increased in men >10 years after the Mustard/Senning operation.

A 45-year-old man with history of Mustard repair for transposition of the great arteries, cirrhosis, and chronic hypoxemic respiratory failure presented for subacute worsening of his chronic symptoms, which were found to be secondary to a previously unrecognized baffle stenosis and leak. Percutaneous intervention resolved his ascites and hypoxia. (Level of Difficulty: Intermediate.)

Thirty four-year-old male with history of D-transposition of the great arteries (D-TGA) who underwent Mustard operation at 14 months of age presented in cardiogenic shock secondary to severe systemic right ventricular failure. Catheterization revealed significantly increased pulmonary pressures. Due to the patient's inotrope dependence and prohibitive pulmonary hypertension, he underwent implantation of a Heart Ware HVAD® for systemic RV support. Within 4 months of continuous flow ventricular assist device (VAD) implantation complete normalization of pulmonary vascular resistance (PVR) was achieved. He ultimately underwent orthotopic heart transplantation with favorable outcomes. This is the second report of complete normalization of PVR following VAD implantation into a systemic RV in

Abstract Background Ventricular assist device (VAD) is usually attached by an inflow cannula to the apex of the systemic left ventricle (LV), but very few cases with implantation of the VAD in the morphologic right ventricle (RV) have been described. Case presentation We describe the case of a 41-year-old male who developed severe systemic RV failure related to a Mustard procedure he had as an infant for treatment of TGA. His heart failure was refractory and irreversible, and therefore, he underwent VAD implantation for systemic RV support. Although the patient developed pulmonary congestion on postoperative day (POD) 5, he was discharged on POD 60. He is now looking forward to receiving heart transplantation. Conclusions Placement of a VAD for systemic RV failure could be a life-saving treatment in adult patients with heart failure due to congenital heart disease.

Introduction: In adult congenital patients with transposition of the great arteries originally treated with the Mustard (atrial switch) procedure, the most common reason for re-intervention is baffle stenosis. This may be exacerbated by permanent transvenous pacemaker lead placement across the baffle.Case Report: A 47-year-old female status post Mustard procedure performed at 15 months old presented with a high-grade stenosis of the superior vena cava (SVC) baffle from the SVC to the left atrium, with a nonfunctional permanent pacemaker lead passing through the baffle. A mechanical rotating dilator sheath was used for attempted lead extraction, relieving the baffle stenosis almost completely as a secondary effect, before the placement of a 10 × 27 mm Visipro balloon-expandable stent in the SVC baffle.Conclusions: Use of the mechanical rotating dilator sheath is an evolving treatment strategy in adult congenital heart disease to minimize the risk of bleeding, trauma to surrounding structures, and death. Its ability to fully alleviate baffle stenosis even when full lead extraction is not feasible or is associated with significant procedural risk, further demonstrates its expanded role in this patient population. A multidisciplinary approach and great diligence must be employed to avoid potential complications. [ABSTRACT FROM AUTHOR]

Atrial switch procedures (Senning and Mustard) for transposition of the great arteries have largely been abandoned for arterial switch procedures. The number of surviving patients who have undergone atrial switch procedures is declining. We present a case of the oldest known survivor (aged 67 years) of the Mustard procedure. ( Level of Difficulty: Beginner. )., Competing Interests: The authors have reported that they have no relationships relevant to the contents of this paper to disclose., (© 2023 The Authors.)

Given the improved survival in patients with corrected dextro-transposition of great arteries (D-TGA), it has evolved into an adult congenital heart defect. It is important to understand the management and complications observed in this population that eventually progresses to systemic ventricular failure requiring cardiac transplantation. Our case focuses on the rapid right ventricle (RV) deterioration of a patient with corrected D-TGA following a surgical procedure requiring systemic support. With such patients awaiting heart transplantation, there are limited assist devices available for RV support and no right ventricular assist device is approved for destination therapy yet. Current indications for implantation of the HeartWare ventricular assist device (HVAD) are limited by the Food and Drug Administration (FDA) to the left ventricle support as a bridge to transplantation. However, its use in the United States for right-sided support has rarely been described for adult congenital defects. In this case, a HeartWare assist device was used to provide systemic support as a bridge to cardiac transplant. The size and implantation design of the HVAD makes it a promising option for patients with this challenging patient population and RV failure as a late complication. [ABSTRACT FROM AUTHOR]

Background: Atrial switch repair (AtrSR) was the initial operation method in patients with D-transposition of the great arteries (D-TGA) constituting the right ventricle as a systemic one. Currently, it has been replaced with arterial switch operation (ASO), but the cohort of adults after AtrSR is still large and requires strict cardiological management of late complications. For this reason, we aimed to evaluate potential long-term mortality risk factors in patients with D-TGA after AtrSR (either Mustard or Senning procedures) Methods: We searched the MEDLINE database for suitable trials. We included 22 retrospective and prospective cohort studies of patients with D-TGA with at least 5 years mean/median follow-up time after Mustard or Senning procedures, with an endpoint of non-sudden cardiac death (n-SCD) and sudden cardiac death (SCD) after at least 30 days following surgery., Results: A total of 2912 patients were enrolled, of whom 351 met the combined endpoint of n-SCD/SCD. The long-term mortality risk factors were New York Heart Association (NYHA) class ≥III/heart failure hospitalization (odds ratio [OR], 7.25; 95% confidence interval [CI], 2.67-19.7), tricuspid valve regurgitation (OR, 4.64; 95% CI, 1.95-11.05), Mustard procedure (OR, 2.15; 95% CI, 1.37-3.35), complex D-TGA (OR, 2.41; 95% CI, 1.31-4.43), and right ventricular dysfunction (OR, 1.94; 95% CI, 0.99-3.79). Supraventricular arrhythmia (SVT; OR, 2.07; 95% CI, 0.88-4.85) and pacemaker implantation (OR, 2.37; 95% CI, 0.48-11.69) did not affect long-term survival in this group of patients. In an additional analysis, SVT showed a statistically significant impact on SCD (OR, 2.74; 95% CI, 1.36-5.53) but not on n-SCD (OR, 1.5; 95% CI, 0.37-6.0)., Conclusions: This meta-analysis demonstrated that at least moderate tricuspid valve regurgitation, NYHA class ≥III/heart failure hospitalization, right ventricular dysfunction, complex D-TGA, and Mustard procedure are risk factors for long-term mortality in patients after AtrSR.

Although sleep-disordered breathing has been extensively studied in patients with left-ventricular dysfunction, little is known of its prevalence in adults with congenital heart disease. Patients with d-looped transposition of the great arteries (d-TGA) who have undergone atrial switch procedures often develop progressive heart failure. The objective of this study was to determine the prevalence of patients at risk for sleep-disordered breathing in adults with d-TGA and atrial switch procedures compared with a control population. Thirty-two patients with d-TGA (66 % males, median age 31) were compared with 32 healthy controls. Baseline demographics and clinical characteristics were documented. The snoring, tiredness during daytime, observed apnea, and high blood pressure (STOP) questionnaire was used to identify subjects at risk for obstructive sleep apnea (OSA). There was no difference in baseline demographics between subjects and controls. For the STOP questionnaire, 14 subjects with d-TGA had scores predictive of OSA compared with three in the control group (44 vs. 9 %, p = 0.0038). There was no difference in functional status between d-TGA patients with or without OSA. There is a greater prevalence of risk for sleep disordered breathing in adults with d-TGA compared with controls. Further prospective investigation with sleep studies will be valuable to confirm these findings. [ABSTRACT FROM AUTHOR]

OBJECTIVES The Senning or Mustard procedure combined with the arterial switch operation (ASO) (± VSD and no left ventricular (LV) outflow tract obstruction) or the Rastelli operation (VSD and LV outflow tract obstruction) has become the preferred strategy over conventional repair as it is thought to prevent long-term dysfunction of the right ventricle (RV). More recently, hemi-Mustard rerouting of blood from the inferior vena cava to the RV in combination with bidirectional cavopulmonary anastomosis (BCPA) has been adopted by some centres for potential benefits over the classic atrial switch procedure. The aim of this study was to analyse our experience with hemi-Mustard and BCPA as part of an anatomical repair of congenitally corrected transposition of the great arteries (CCTGA) in selected patients. METHODS Between 2004 and 2011, eight patients underwent hemi-Mustard/BCPA with the Rastelli operation (n = 6) or ASO (n = 2). The median age was 2.9 (range: 1.2–9.1) years. Positional anomalies were present in 75% of the patients. Both patients with ASO had dysplastic and insufficient tricuspid valves. In the Rastelli group, four patients had previously received shunts followed by BCPA in one patient. In the ASO group, both patients underwent pulmonary artery banding initially. RESULTS There was one in-hospital death and no late mortality. Two patients received a pacemaker. One patient from the Rastelli group required conduit change 6 years later. At the mean follow-up of 4.5 years, six and one patients are in NYHA classes I and II, respectively; six patients showed good biventricular function, while one had LV dysfunction. Systemic venous obstruction and sinus node dysfunction were not observed, and BCPA was functioning well in all patients. CONCLUSIONS Hemi-Mustard/BCPA is useful in anatomical repair of CCTGA in selected patients. When compared with the classic atrial switch operation, it is technically easier which makes it especially helpful in atrio-apical discordance; it unloads an RV with limited size or function, and avoids complications related to the upper limb of the classic atrial switch procedure. Mid-term results of this approach are favourable. Further follow-up is needed to prove long-term benefits. [ABSTRACT FROM AUTHOR]

With advances in surgical techniques and perioperative care, the survival rate of patients with congenital heart disease continues to increase. As such, patients with palliated or corrected congenital heart disease may present for major surgical procedures. Although alternative techniques are now available for the anatomic repair of patients with transposition of the great arteries (TGA), an atrial level baffle (Mustard or Senning procedure) was previously performed. As these patients age, long-term adverse effects may occur including myocardial dysfunction, rhythm disturbances requiring pacemaker placement, pulmonary hypertension, and baffle obstruction. We present a case report of a 14 year old girl with TGA, who underwent a Senning procedure during infancy and now presented for posterior spinal fusion in the treatment of kyphoscoliosis. The perioperative care of such patients is discussed. [ABSTRACT FROM AUTHOR]

With advances in surgical techniques and perioperative care, the survival rate of patients with congenital heart disease continues to increase. As such, patients with palliated or corrected congenital heart disease may present for major surgical procedures. Although alternative techniques are now available for the anatomic repair of patients with transposition of the great arteries (TGA), an atrial level baffle Mustard or Senning procedure) was previously performed. As these patients age, long-term adverse effects may occur including myocardial dysfunction, rhythm disturbances requiring pacemaker placement, pulmonary hypertension, and baffle obstruction. The current manuscript reviews the anatomy of TGA, outlines the past and current surgical approaches, and discusses the perioperative concerns of such patients. [ABSTRACT FROM AUTHOR]

Objectives: To evaluate the relation of echocardiographic parameters of diastolic function, exercise capacity (expressed as peakVO2) and NT-proBNP in patients with transposition of the great arteries (TGA) and Mustard procedure. Methods: Diastolic function was determined by measuring tricuspid flow velocities (Ea/Aa ratio), isovolumic relaxation time (IVRT), and deceleration time (DT). E/Ea ratios were calculated. For assessment of systolic function, CMR was applied. Results: E/A (r = 0.07, p = 0.66), E/Ea medial (r = 0.03, p = 0.84), E/Ea lateral (r = -0.01, p = 0.92), IVRT (r = -0.13, p = 0.44), and DT (r = -0.05, p = 0.76) were not correlated with peakVO2. NT-proBNP showed a significant correlation with IVRT (r = 0.44, p = 0.004) and Ea/Aa medial (r = -0.34, p = 0.025). No correlation was found between RV systolic function and peakVO2 (r = 0.07, p = 0.63). Conclusions: Exercise capacity in patients with TGA and Mustard procedure is not related to echocardiographic parameters of diastolic function. NT-proBNP is associated with selected echocardiographic parameters of diastolic function. Copyright © 2010 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]

Conditions in which the right ventricle serves as the systemic pumping chamber are frequently complicated by the development of right ventricular failure and tricuspid valve regurgitation. The right ventricle is the systemic ventricle in conditions of ventriculoarterial discordance with atrioventricular concordance (transposition of the great arteries) or with atrioventricular discordance (congenitally corrected transposition of the great arteries). Concerns regarding actual or potential systemic right ventricular failure in these cases may lead to surgical evaluation and treatment designed to reestablish the left ventricle as the systemic pump. In cases where the left ventricle has prolonged exposure to low pressures in the pulmonary circulation, the left ventricle must be “retrained” to assume a systemic pressure load. Anatomic repair, with or without a preparatory period of left ventricular retraining, is a consideration for three clinically relevant scenarios: (1) patients with transposition of the great arteries after an atrial level switch (Senning or Mustard procedure), (2) patients with congenitally corrected transposition who are unoperated or who have undergone physiologic (“classic”) repair, and (3) unoperated patients with transposition who present after the neonatal period. [Copyright &y& Elsevier]

This case report discusses a young man with d-transposition of the great arteries who suffered three myocardial infarctions late after his Mustard procedure. The etiology was found to be a coronary embolism, with a mural thrombus in the systemic ventricle as the probable nidus. The risk factors of coronary embolism are discussed, and it is concluded that Mustard patients are at risk for coronary embolization and myocardial infarction, which can be a cause of sudden death. [ABSTRACT FROM AUTHOR]

We report an adult patient with transposition of the great arteries status post-Mustard procedure in whom three-dimensional transesophageal echocardiography demonstrated intraatrial baffle obstruction. The baffle could be visualized in both long-axis and “en face” short-axis views. (ECHOCARDIOGRAPHY, Volume 20, July 2003). [ABSTRACT FROM AUTHOR]

Background-While volume derived global indices of right ventricle (RV) function are frequently abnormal after the Mustard procedure, the mechanism for these abnormalities is poorly understood. RV muscle fibres are predominantly arranged longitudinally and thus indices derived in the long axis may better describe RV function. Methods-20 survivors of the Mustard operation were studied (age 7.8-37.3 years, median 14.2 years). Long axis recordings from the apical four chamber view were obtained with the M mode cursor positioned through the lateral angle of the tricuspid valve annulus. M mode traces were recorded on paper and later digitised to derive total atrioventricular ring excursion, peak lengthening rate, and peak shortening rate. These data were averaged and compared with control data for the normal RV and left ventricle (LV). Results-RV total atrioventricular ring excursion was lower than that for the RV (p < 0.0001) or LV (p < 0.005) of controls. Peak lengthening rate was lower than the normal RV (p < 0.0001) and LV (p < 0.000 1) rates. Furthermore, peak shortening rate was less than that of normal RV (p < 0.000 1) and normal LV (p <0.005) controls. Conclusion-Systemic RV long axis function is notably reduced compared with that of either the normal subpulmonary RV or the systemic LV. This presumably reflects the response of the predominantly longitudinally arranged myocardial fibres to increased afterload. However, such measurements may provide a more sensitive marker for progressive changes in global function during long term follow up. [ABSTRACT FROM AUTHOR]

Ablation of intra-atrial reentrant tachycardia following Mustard or Senning procedures has low success rates. The Biosense Carto® system was used to map intra-atrial reentry in a 22-year-old woman who had undergone a Mustard procedure. A line of block was created connecting a Mustard baffle suture line to the tricuspid valve annulus, which terminated the arrhythmia and prevented its reinitiation. Multisite electoanatomic mapping was invaluable in defining atrial anatomy and the intra-atrial reentrant pathway, and in creating a contiguous line of block. This mapping may improve ablation success rates in patients following the Mustard or Senning repair. [ABSTRACT FROM AUTHOR]