早发型双胎输血综合征受血儿双胞胎心肌病的流行率和进展。

Prevalence and progression of recipient-twin cardiomyopathy in early-stage twin-twin transfusion syndrome.

机构信息

The Fetal Care Center of Cincinnati, The Heart Institute, Cincinnati Children's Hospital, Cincinnati, OH, USA.

出版信息

Ultrasound Obstet Gynecol. 2012 Jan;39(1):63-8. doi: 10.1002/uog.10117.

DOI:10.1002/uog.10117

PMID:21998013

Abstract

OBJECTIVE

The management of twin-twin transfusion syndrome (TTTS) in its early stages (Quintero Stages I and II) is controversial. We describe the prevalence, severity, incidence and rate of progression of recipient-twin cardiomyopathy in Stages I and II TTTS.

METHODS

Among 451 cases of TTTS evaluated between 2004 and 2009, 123 (27.3%) cases of Stages I and II were reviewed. Echocardiography was used to 'upstage' cases based on the presence or absence of mild (IIIA), moderate (IIIB), or severe (IIIC) recipient cardiomyopathy. Progression was defined by worsening in the degree of recipient-twin cardiomyopathy from initial presentation or failure to respond to amnioreduction. Outcome data included progression of recipient-twin cardiomyopathy, treatment and survival to birth. Data were compared by the chi-square, Fisher's exact test or t-test as appropriate.

RESULTS

Seventy-seven of 123 (62.6%) cases were Quintero Stage I and 46/123 (37.4%) Quintero Stage II. Eighty (65.0%) were upstaged to Cincinnati Stage IIIA (n = 25), IIIB (n = 23) or IIIC (n = 32). Management included observation in 11 (8.9%), amnioreduction in 26 (21.1%), amnioreduction followed by selective fetoscopic laser photocoagulation (SFLP) in 43 (35.0%) and primary SFLP in 43 (35.0%). Of 80 cases managed by observation or amnioreduction initially, 43 (53.8%) progressed within a mean duration of 1.4 ± 1.5 weeks. The incidence of progression increased significantly as degree of recipient-twin cardiomyopathy at presentation worsened: Stage I, 9/27 (33.3%); Stage II, 8/15 (53.3%); Stage IIIA, 8/16 (50.0%); Stage IIIB, 10/10 (100%); and Stage IIIC, 8/12 (66.7%) (χ(2) = 14, P < 0.01). Overall fetal survival was 205 out of 244 (84.0%). Fetal survival with observation only was 81.8% (18/22), with amnioreduction only it was 92.3% (48/52), with initial observation or amnioreduction followed by SFLP it was 86.9% (73/84) and with primary SFLP it was 76.7% (66/86).

CONCLUSION

Echocardiography demonstrates a high incidence of recipient-twin cardiomyopathy in early-stage TTTS. The more advanced the recipient-twin cardiomyopathy is, the more likely is progression to occur during observation or following amnioreduction.

摘要

目的

双胞胎输血综合征（TTTS）早期（Quintero 1 期和 2 期）的处理存在争议。我们描述了 1 期和 2 期 TTTS 中受血儿心肌病的患病率、严重程度、发生率和进展率。

方法

在 2004 年至 2009 年间评估的 451 例 TTTS 中，回顾了 123 例（27.3%）1 期和 2 期病例。采用超声心动图根据轻度（IIIA）、中度（IIIB）或重度（IIIC）受血儿心肌病的存在或不存在对病例进行“升级”。进展定义为受血儿心肌病从初始表现恶化或羊膜减少术治疗无反应。结局数据包括受血儿心肌病的进展、治疗和存活至分娩。采用卡方检验、Fisher 确切概率法或 t 检验比较数据。

结果

123 例中有 77 例（62.6%）为 Quintero 1 期，46 例（37.4%）为 Quintero 2 期。80 例（65.0%）升级为辛辛那提 3A 期（n=25）、3B 期（n=23）或 3C 期（n=32）。治疗包括观察（n=11，8.9%）、羊膜减少术（n=26，21.1%）、羊膜减少术后选择性胎儿镜激光凝固术（n=43，35.0%）和原发性胎儿镜激光凝固术（n=43，35.0%）。在最初接受观察或羊膜减少术治疗的 80 例中，43 例（53.8%）在平均 1.4±1.5 周内进展。受血儿心肌病在就诊时的严重程度越严重，进展的发生率显著增加：1 期，9/27（33.3%）；2 期，8/15（53.3%）；3A 期，8/16（50.0%）；3B 期，10/10（100%）；3C 期，8/12（66.7%）（χ²=14，P<0.01）。总胎儿存活率为 244 例中的 205 例（84.0%）。仅观察的胎儿存活率为 81.8%（18/22），仅羊膜减少术为 92.3%（48/52），初始观察或羊膜减少术后联合胎儿镜激光凝固术为 86.9%（73/84），原发性胎儿镜激光凝固术为 76.7%（66/86）。