Faiola Stefano, Mandalari Maria, Coco Chiara, Casati Daniela, Laoreti Arianna, Mannarino Savina, Corti Carla, Consonni Dario, Cetin Irene, Lanna Mariano
Fetal Therapy Unit 'Umberto Nicolini', Buzzi Children's Hospital, 20154 Milan, Italy.
Department of Woman, Mother and Neonate, Buzzi Children's Hospital, 20154 Milan, Italy.
J Clin Med. 2023 Jul 17;12(14):4734. doi: 10.3390/jcm12144734.
Right ventricular outflow tract anomalies (RVOTAs), such as pulmonary stenosis (PS), pulmonary atresia (PA), and pulmonary insufficiency (PI), are typical cardiac anomalies in monochorionic twins, and they are complicated by twin-to-twin transfusion syndrome (TTTS). The aim of this study was to conduct a long-term postnatal cardiological evaluation of prenatal RVOTAs in monochorionic diamniotic twin pregnancies complicated by TTTS and treated with fetoscopic laser surgery (FLS) and to analyze possible prenatal predictors of congenital heart disease (CHD). Prenatal RVOTAs were retrospectively retrieved from all TTTS cases treated with FLS in our unit between 2009 and 2019. Twenty-eight prenatal cases of RVOTAs (16 PI, 10 PS, 2 PA) were observed out of 335 cases of TTTS. Four cases did not reach the postnatal period. CHD was present in 17 of the remaining 24 cases (70.8%), with 10 being severe (58.8%; 10/17); nine cases of PS required balloon valvuloplasty, and one case required biventricular non-compaction cardiomyopathy. The risk of major CHD increased with prenatal evidence of PS and decreased with the gestational age at the time of TTTS and with the prenatal normalization of blood flow across the pulmonary valve. Despite treatment with FLS, the majority of monochorionic diamniotic twin pregnancies complicated by TTTS with prenatal RVOTAs had CHD at long-term follow-up.
右心室流出道异常(RVOTA),如肺动脉狭窄(PS)、肺动脉闭锁(PA)和肺动脉瓣关闭不全(PI),是单绒毛膜双胎典型的心脏异常,并且常合并双胎输血综合征(TTTS)。本研究的目的是对合并TTTS且接受胎儿镜激光手术(FLS)治疗的单绒毛膜双羊膜囊双胎妊娠中的产前RVOTA进行长期产后心脏评估,并分析先天性心脏病(CHD)可能的产前预测因素。回顾性检索了2009年至2019年在本单位接受FLS治疗的所有TTTS病例中的产前RVOTA。在335例TTTS病例中观察到28例产前RVOTA病例(16例PI、10例PS、2例PA)。4例未达到产后阶段。其余24例中有17例存在CHD(70.8%),其中10例病情严重(58.8%;10/17);9例PS患者需要球囊瓣膜成形术,1例需要双心室心肌致密化不全治疗。严重CHD的风险随着产前PS证据的出现而增加,随着TTTS发生时的孕周以及肺动脉瓣血流产前恢复正常而降低。尽管接受了FLS治疗,但大多数合并产前RVOTA的TTTS单绒毛膜双羊膜囊双胎妊娠在长期随访中仍患有CHD。