Hu Qing, Deng Chunyan, Zhu Qi, Yang Xiaoyan, Liu Hongyan, Liao Hua, Wang Xiaodong, Yu Haiyan
Department of Obstetrics and Gynecology, West China Second University Hospital, Sichuan University, Chengdu, China.
Laboratory of Birth Defects and Related Diseases of Women and Children (Sichuan University), Ministry of Education, Chengdu, China.
Transl Pediatr. 2022 Apr;11(4):601-609. doi: 10.21037/tp-21-569.
Dextro-transposition of the great arteries (D-TGA) is a rare congenital heart disease, as it affects only 0.02-0.05% of live births. It is the second most common cyanotic heart disease following Tetralogy of Fallot. It has a male predominance. Fetal echocardiography is an optimal method for prenatal diagnosis of fetal D-TGA. In twin pregnancies, fetal D-TGA in one twin is very rare, especially in monochorionic-diamniotic twin pregnancies. Herein, we report a case of D-TGA in one twin in two dichorionic-diamniotic twin pregnancies and one monochorionic-diamniotic twin pregnancy from January 2018 to June 2021.
One twin with D-TGA was diagnosed by fetal echocardiography in the second trimester, and the co-twin was normal in all three cases. A multidisciplinary team provided extensive counseling regarding the D-TGA twin and the co-twin, and adequate perinatal management was provided. In cases 1, 2, and 3, the mothers underwent cesarean sections at 37 weeks + 2 days, 34 weeks + 5 days, and 36 weeks + 1 day, respectively. In case 1, which involved a female D-TGA neonate with birth weight 2,410 g, an emergent atrial septostomy was performed at 20 h after birth, and the neonate underwent atrial switch operation (ASO) 24 days after birth. In case 2, involving a male D-TGA neonate with a birth weight of 2,380 g, ASO was performed 24 days after birth. In case 3, involving a female D-TGA neonate with birth weight 2,240 g, ASO was performed 19 days after birth and delayed sternal closure was performed 4 days later. All six infants showed normal development during follow-up.
Early antenatal diagnosis of D-TGA in one fetus of a twin pregnancy is significantly important. A multidisciplinary team should carry individual evaluation and integrated management of the D-TGA twin and co-twin during the pregnancy and perinatal period. After birth, delayed ductus arteriosus closure in the D-TGA twins should be performed when necessary and individualized timings for arterial switch operation should be considered.
大动脉右位转位（D-TGA）是一种罕见的先天性心脏病，仅影响0.02-0.05%的活产儿。它是仅次于法洛四联症的第二常见的青紫型心脏病。该病男性居多。胎儿超声心动图是产前诊断胎儿D-TGA的最佳方法。在双胎妊娠中，一个胎儿患D-TGA非常罕见，尤其是在单绒毛膜双羊膜囊双胎妊娠中。在此，我们报告2018年1月至2021年6月期间，两例双绒毛膜双羊膜囊双胎妊娠和一例单绒毛膜双羊膜囊双胎妊娠中一个胎儿患D-TGA的病例。
三例中均在孕中期通过胎儿超声心动图诊断出一个胎儿患D-TGA，另一胎儿均正常。多学科团队为患D-TGA的胎儿及其同胞提供了广泛的咨询，并给予了充分的围产期管理。病例1、2和3中，母亲分别于孕37周+2天、34周+5天和36周+1天行剖宫产。病例1中，一名出生体重2410g的患D-TGA的女婴在出生后20小时紧急行房间隔造口术，并于出生后24天接受心房调转术（ASO）。病例2中，一名出生体重2380g的患D-TGA的男婴在出生后24天接受ASO。病例3中，一名出生体重2240g的患D-TGA的女婴在出生后19天接受ASO，4天后延迟关闭胸骨。所有六名婴儿在随访期间均发育正常。
双胎妊娠中一个胎儿的D-TGA的早期产前诊断非常重要。多学科团队应在孕期和围产期对患D-TGA的胎儿及其同胞进行个体化评估和综合管理。出生后，必要时应对患D-TGA的双胎进行动脉导管延迟关闭，并应考虑个体化的动脉调转手术时机。
