Dextro-transposition of the great arteries in one twin: case reports and literature review

Background: 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. Case Description: 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. Conclusions: 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. Keywords: Dextro-transposition of the great arteries; atrial switch operation; monochorionic-diamniotic twin pregnancy; dichorionic diamniotic twin pregnancy; one twin