完全大血管転位症

BACKGROUND: The arterial switch operation (ASO) is standard treatment for D-transposition of the great arteries, and neo-aortic dilation is common afterward. Its behavior during pregnancy remains uncertain, especially the risk of dissection. CASE: We describe a 30-year-old woman (G6P0A5) with neo-aortic dilation (44 mm) during her first pregnancy, which remained stable on beta blockers until an uncomplicated caesarean delivery. In her subsequent pregnancy, an initial measurement of 47 mm raised concern. Expert review highlighted the challenges of accurately measuring asymmetric neo-aortas post-ASO and confirmed that the neo-aorta was stable (43 mm). The pregnancy continued without complications under beta-blocker therapy. DISCUSSION: No neo-aortic dissections have been reported after ASO, and progression above 2 mm was reported only in a few (2/131). Preconception evaluation, standardized measurement techniques, and trimester-based surveillance are essential. Mode of delivery should be individualized; caesarean delivery may be reasonable when dilation exceeds 45 mm.

BACKGROUND: Reentrant atrial tachycardias (ATs) are frequent after atrial switch for dextro-transposition of the great arteries and are associated with adverse prognosis. Catheter ablation is first-line therapy, yet long-term outcomes and predictors of recurrence remain poorly defined. Our aim was to characterize induced AT circuits, describe acute and long-term catheter ablation outcomes, and identify recurrence predictors in dextro-transposition of the great arteries. METHODS: Single-center ambispective cohort of consecutive adults with dextro-transposition of the great arteries undergoing a first AT ablation (2003-2025). Arrhythmias were mechanically induced with decremental pacing or extra stimuli. Acute success was defined as elimination of all clinical and reproducibly inducible tachycardias (cycle length >200 ms), with bidirectional cavotricuspid isthmus block when applicable. Recurrence was defined as any documented AT >30 seconds. RESULTS: Thirty-three patients underwent 46 procedures. During the index procedure, 66 ATs were induced; 51 (83%) were characterized: 32 (63%) were cavotricuspid isthmus-dependent and 11 (22%) incisional. Cavotricuspid isthmus block was achieved in 84%; acute noninducibility in 73%. Median follow-up was 7.0 years (interquartile range, 2.5-10.4). Overall, 15 patients (45.5%) recurred; 1-year freedom was 75%. Recurrence was strongly associated with failed or uncertain acute efficacy, severely impaired systemic right ventricular fractional shortening ≤22%, prolonged PR interval (220 ms), and greater number of inducible ATs (all P<0.05). In multivariable modelling, these 4 variables yielded an optimism-corrected C-index of 0.85. Eleven (33%) underwent redo ablation; 8 achieved acute noninducibility, and only 3 recurred thereafter. CONCLUSIONS: In dextro-transposition of the great arteries postatrial switch, cavotricuspid isthmus-dependent flutter is most common, but scar-related circuits are frequent. Catheter ablation is safe and acutely effective, but long-term recurrence remains substantial. Failed acute efficacy, severely impaired systemic right ventricle contractility, prolonged PR interval, and multiple inducible ATs identify patients at highest risk and may guide follow-up and referral for early repeat ablation.

Dextro-transposition of the great arteries (d-TGA) is a congenital heart disease (CHD) characterized by parallel circulations, in which survival depends on intercirculatory mixing. CHD as a whole is a recognized risk factor for necrotizing enterocolitis (NEC), which carries high morbidity and mortality. Managing perforated NEC in a neonate with uncorrected complex d-TGA presents a complex challenge, particularly in centers lacking specialized pediatric cardiac surgery facilities. We report the successful emergency management of a 28-day-old preterm neonate with uncorrected complex d-TGA, a large ventricular septal defect, and severe subpulmonary obstruction requiring urgent surgery. Due to vital urgency and the impossibility of transfer to a specialized center with pediatric cardiac surgery, local care was optimized through remote expert consultation and an intensive multidisciplinary briefing. Anesthesia was tailored to maintain systemic vascular resistance and preserve pressure-driven intercirculatory mixing. The team relied on multimodal noninvasive monitoring, integrating cerebral near-infrared spectroscopy (NIRS) and continuous noninvasive hemoglobin (SpHb). Cerebral NIRS complemented conventional monitoring, detecting a drop in regional oxygen saturation (rSO2) and prompting a proactive transfusion strategy that stabilized the infant intraoperatively. Following a successful left hemicolectomy, the infant was later transferred to a specialized cardiac center for further management of d-TGA. Although surgery in such critical neonates is ideally performed in specialized centers, this case demonstrates how leveraging multidisciplinary teamwork, advanced monitoring, and physiological understanding can bridge the gap between gold standards and the practical realities of life-threatening neonatal emergencies in nonspecialized facilities.

BackgroundTransposition of the great arteries (TGA) is a congenital heart disease characterized by ventriculoarterial discordance. Corrective surgeries can provide long-term palliation, but many patients ultimately develop systemic right ventricular failure and end-stage heart failure. Heart transplantation (HT) is an established treatment in adults with congenital heart disease, but data specifically focused on adults with TGA undergoing HT remains limited, with only small single-center series reported to date. The aim of this study was to describe perioperative complexity, immunologic burden, and posttransplant outcomes in adults with TGA undergoing HT.MethodsIn this retrospective study, 9 consecutive adult TGA patients received HT between January 2019 and December 2025. Pre-listing assessment by a multidisciplinary team and operative and postoperative details were recorded. The primary endpoint was overall survival; secondary endpoints included need for mechanical circulatory support (MCS) and acute rejection.ResultsMedian recipient age was 45 (43-52) years; 55.6% (5/9) were women. Median waiting time was 98.0 (62.2-140.5) days. Median ischemia and cardiopulmonary bypass times were 240 (195-272) and 191 (145-220) minutes, respectively. One of 9 patients (11.1%) required postoperative MCS. Median follow-up time was 4.0 (0.8-5.3) years, with a survival rate of 88.9% (8/9) during the follow-up period and 1 in-hospital death. Four of 9 patients (44.4%) developed acute rejection, and all were successfully managed.ConclusionsHeart transplantation is a feasible and life-extending strategy for patients with TGA who develop heart failure despite prior palliative interventions. Multidisciplinary assessment and surgical expertise are crucial for optimizing outcomes. This study highlights the challenges of HT in this disease.