背景:右室流出道梗阻(RVOTO)是双胎输血综合征(TTTS)患者中最常见的先天性心脏病,尤其是在受者双胎中。在这项回顾性研究中,我们评估了发病率,预后,产后管理,在台湾的一个中心,在胎儿镜激光光凝(FLP)后26周前诊断的重度TTTS病例中,受者双胞胎的围产期结局和RVOTO危险因素。
方法:使用胎儿或产后超声心动图诊断RVOTO。评估的胎儿结局是围产期生存率,新生儿脑影像异常率,分娩时的胎龄,出生体重。
结果:共187例严重TTTS病例;14例(7.49%)患有RVOTO受体双胎(12例肺动脉狭窄,2例肺动脉闭锁)。在这14个案例中,3(21.4%)显示FLP后流出道阻塞的改善,11例(78.6%)导致围产期存活。在11名幸存者中,5例(45.5%)接受经导管球囊瓣膜成形术缓解RVOTO。围产期存活率,分娩时的胎龄,新生儿脑影像异常率,受者双胞胎有和没有RVOTO的组之间的出生体重没有显著差异.一般来说,受者双胞胎在较年轻的胎龄时接受了RVOTOFLP(以周为单位;19.3±2.4vs.20.7±2.6,p=0.048),并且在QuinteroIV期的病例百分比较高(50.0%vs.12.1%,p<0.001)比接受者双胞胎没有RVOTO的那些。使用逻辑回归,我们发现,在重度TTTS病例中,胎龄较小的FLP(p=0.046,比值比=0.779)和QuinteroIV期的TTTS(p=0.001,比值比=7.206)是受者双胎在FLP后发生RVOTO的危险因素.
结论:在这项研究中,受者双胞胎有与没有RVOTO的重度TTTS病例的FLP围产期结局具有可比性,这可能是由于分娩时的胎龄相似以及高Quintero期(III期和IV期)的强烈影响。
BACKGROUND: Right ventricular outflow tract obstruction (RVOTO) is the most frequently encountered congenital heart disease in patients with twin -twin transfusion syndrome (TTTS) and is especially prevalent in the recipient twin. In this retrospective study, we evaluated the incidence, prognosis, postnatal management, and perinatal outcomes of and risk factors for RVOTO in the recipient twin in severe TTTS cases which diagnosed before 26 weeks after fetoscopic laser photocoagulation (FLP) at a single center in Taiwan.
METHODS: RVOTO was diagnosed using fetal or postnatal echocardiography. The fetal outcomes evaluated were perinatal survival rate, neonatal brain image anomalies rate, gestational age at delivery, and birth weight.
RESULTS: Total 187 severe TTTS cases were included; 14 (7.49%) had a recipient twin with RVOTO (12 cases of pulmonary stenosis and 2 of pulmonary atresia). Of these 14 cases, 3 (21.4%) demonstrated improvements in outflow obstruction after FLP, and 11 (78.6%) resulted in perinatal survival. Of the 11 survivors, 5 (45.5%) received transcatheter balloon valvuloplasty to alleviate the RVOTO. The perinatal survival rate, gestational age at delivery, neonatal brain image anomaly rate, and birth weights did not significantly differ between the groups in which the recipient twin had versus did not have RVOTO. Generally, the recipient twin had RVOTO received FLP at a younger gestational age (in weeks; 19.3 ± 2.4 vs. 20.7 ± 2.6, p = 0.048) and had a higher percentage of cases at Quintero stage IV (50.0% vs. 12.1%, p < 0.001) than those in which the recipient twin did not have with RVOTO. Using logistic regression, we discovered that FLP at a younger gestational age (p = 0.046, odds ratio = 0.779) and TTTS at Quintero stage IV (p = 0.001, odds ratio = 7.206) were risk factors for the recipient twin developing RVOTO after FLP in severe TTTS cases.
CONCLUSIONS: The post-FLP perinatal outcomes of cases of severe TTTS in which the recipient twin had versus did not have RVOTO were comparable in this study, which may have been due to the similar gestational ages at delivery and strong influence of high Quintero stages (stages III and IV).