Lillitos Peter, Moriarty Grace, Witter Thomas, Austin Conal, Miller Owen, Sharland Gurleen K, Simpson John M, Zidere Vita, Vigneswaran Trisha V
Department of Congenital Heart Disease, Evelina London Children's Hospital, Guy's & St Thomas' NHS Trust, Westminster Bridge Road, London, SE1 7EH, UK.
School of Biomedical Engineering & Imaging Sciences, King's College London, London, UK.
Pediatr Cardiol. 2025 Jul 15. doi: 10.1007/s00246-025-03939-w.
Outcomes following the arterial switch operation (ASO) are well documented. Survival and long-term morbidity for fetuses diagnosed with dextro-transposition of the great arteries (d-TGA) are less reported. We aimed to document survival, reinterventions and morbidity for prenatally diagnosed d-TGA to inform prenatal counseling. Fetuses with d-TGA with or without ventricular septal defect (VSD) diagnosed between 1995 and 2022 at our institution were reviewed. Outcomes were compared to postnatally diagnosed patients undergoing intervention during the same era. Two hundred and seven fetuses were diagnosed resulting in 201 livebirths, 2 intrauterine demise, and 4 pregnancy terminations. There were 137 (68.2%) with isolated d-TGA and 64 (31.8%) with d-TGA-VSD. Median birthweight 3.21 kg (IQR 2.94-3.5 kg), and median birth gestation 38 weeks (IQR 38-39). Preoperative balloon atrial septostomy (BAS) was performed in 126/201(62.7%). No patients died prior to BAS. Three died before ASO following BAS. ASO was performed in 198/201(98.5%) with 45/198(22.7%) having concomitant VSD closure. Thirty-day survival for ASO was 95.5%. Survival over the study period was 186/201(92.5%). During the same period, 91 infants were referred for surgery with postnatal diagnosis, and 90 underwent ASO. There was no significant difference in 30-day or long-term survival following ASO according to timing of diagnosis. Three prenatally diagnosed patients undergoing the ASO were lost to follow up. There was no significant difference in reintervention rates (prenatal: 18/195 (9.2%); postnatal: 12/86 (13.9%) p = 0.24). Morbidity in prenatally diagnosed patients included myocardial dysfunction 3/195(1.5%), pulmonary hypertension 1/195(0.5%), supraventricular tachycardia 3/195(1.5%), neurological morbidity 9/195(4.6%), and autism 11/195(5.6%), and none were statistically different to the postnatal group. Survival following prenatal diagnosis of d-TGA is good. In the prenatally diagnosed cohort there were no deaths prior to BAS. Three died after BAS, with one of these deaths attributable to newborn hypoxia between BAS and ASO. Following ASO, most patients survive into third decade following prenatal diagnosis. Reintervention occurred in 9.2%. Neurological and behavioral morbidity affected approximately 1 in 20 patients. Outcomes for those with prenatal diagnosis of d-TGA are comparable with the patients that undergo ASO following postnatal diagnosis, but this excludes patients with a postnatal diangosis who did not survive to cardiac intervention. This data will be helpful for prenatal counseling.
动脉调转术(ASO)后的结果已有充分记录。对于诊断为大动脉右位转位(d-TGA)的胎儿,其生存率和长期发病率的报道较少。我们旨在记录产前诊断为d-TGA的胎儿的生存率、再次干预情况和发病率,为产前咨询提供依据。对1995年至2022年在我们机构诊断为d-TGA伴或不伴室间隔缺损(VSD)的胎儿进行了回顾。将结果与同一时期接受干预的产后诊断患者进行比较。共诊断出207例胎儿,其中201例活产,2例宫内死亡,4例终止妊娠。137例(68.2%)为单纯d-TGA,64例(31.8%)为d-TGA-VSD。中位出生体重3.21 kg(四分位间距2.94 - 3.5 kg),中位出生孕周38周(四分位间距38 - 39周)。126/201例(62.7%)在术前进行了球囊房间隔造口术(BAS)。在进行BAS之前没有患者死亡。3例在BAS后ASO前死亡。198/201例(98.5%)进行了ASO,其中45/198例(22.7%)同时进行了VSD修补。ASO术后30天生存率为95.5%。研究期间的生存率为186/201例(92.5%)。同一时期,91例产后诊断的婴儿被转诊进行手术,90例接受了ASO。根据诊断时间,ASO术后30天或长期生存率无显著差异。3例产前诊断并接受ASO的患者失访。再次干预率无显著差异(产前:18/195例(9.2%);产后:12/86例(13.9%),p = 0.24)。产前诊断患者的并发症包括心肌功能障碍3/195例(1.5%)、肺动脉高压1/195例(0.5%)、室上性心动过速3/195例(1.5%)、神经并发症9/195例(4.6%)和自闭症11/195例(5.6%),与产后组相比均无统计学差异。产前诊断为d-TGA后的生存率良好。在产前诊断队列中,在BAS之前没有死亡病例。3例在BAS后死亡,其中1例死亡归因于BAS和ASO之间的新生儿缺氧。ASO术后,大多数患者在产前诊断后的第三个十年存活。再次干预发生率为9.2%。神经和行为并发症影响约二十分之一的患者。产前诊断为d-TGA的患者的结果与产后诊断后接受ASO的患者相当,但这排除了未存活至心脏干预的产后诊断患者。这些数据将有助于产前咨询。