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.

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.

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.
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).
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.

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- (<10 years), mid- (10-20 years), and long-term (>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 <4 months. We conducted a thorough literature review to identify Mustard patients that received systemic RV VAD as a bridge to a successful heart transplantation. In this article, we summarize the outcomes and focus on pulmonary hypertension reversibility following VAD implant.

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BACKGROUND: 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.
METHODS: 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.

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.
METHODS: 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.