Pruetz Jay D, Votava-Smith Jodie K, Chmait Hikmat R, Korst Lisa M, Llanes Arlyn, Chmait Ramen H
Division of Pediatric Cardiology, Children's Hospital Los Angeles, University of Southern California, Los Angeles, California, USA.
Department of Obstetrics & Gynecology, Keck School of Medicine, University of Southern California, Los Angeles, California, USA.
J Ultrasound Med. 2017 Aug;36(8):1595-1605. doi: 10.7863/ultra.16.08038. Epub 2017 Apr 3.
To evaluate risk for congenital heart disease (CHD) in recipient twins with circular shunt physiology (CSP).
This prospective study enrolled twin-twin transfusion syndrome (TTTS) cases from 2006 to 2015. Fetal echocardiography (FE) was performed before laser surgery when cardiac involvement was suspected. Diagnosis of recipient twin CSP required tricuspid and pulmonary regurgitation, right ventricular dysfunction, and flow reversal in the ductus arteriosus. Outcomes were assessed at 30 days after birth.
Of the 496 TTTS pregnancies, 20 (4%) met the criteria for CSP. Among those born alive, who had documented cardiac outcomes (n = 457), patients with CSP were more likely to have CHD, specifically right ventricular outflow tract obstruction (5 of 18 [27.8%] versus 22 of 439 [5.0%], odd ratio [OR] 7.29, 95% confidence interval [CI] 2.05-24.72, P = .0025). Of the recipient twins with preoperative FE (n = 259, 52%) who were born alive and had documented cardiac outcomes (n = 242), those with CSP were still more likely to have right ventricular outflow tract obstruction (5 of 18 [27.8%] versus 14 of 224 [6.3%], OR 5.77, CI 1.54-20.92, P = .0077). With both analyses, twins with CSP had higher Quintero stage, but similar patient characteristics and 30-day mortality compared with those without CSP. Subgroup analyses of the CSP cohort identified no differences in preoperative characteristics or FE findings predictive of CHD.
Recipient twins with preoperative CSP were at increased risk for postnatal right ventricular outflow tract obstruction, but appeared to have comparable survival after fetal laser surgery despite these dramatic pathophysiological prenatal findings. Preoperative FE in TTTS remains important for prediction of postnatal CHD.
评估患有循环分流生理（CSP）的双胎受血儿患先天性心脏病（CHD）的风险。
这项前瞻性研究纳入了2006年至2015年的双胎输血综合征（TTTS）病例。当怀疑有心脏受累时，在激光手术前进行胎儿超声心动图（FE）检查。双胎受血儿CSP的诊断需要三尖瓣和肺动脉反流、右心室功能障碍以及动脉导管血流逆转。在出生后30天评估结局。
在496例TTTS妊娠中，20例（4%）符合CSP标准。在存活且有记录的心脏结局的婴儿中（n = 457），CSP患者更易患CHD，特别是右心室流出道梗阻（18例中有5例[27.8%]，而439例中有22例[5.0%]，比值比[OR] 7.29，95%置信区间[CI] 2.05 - 24.72，P = 0.0025）。在术前进行FE检查的存活且有记录的心脏结局的双胎受血儿中（n = 259，52%）（n = 242），CSP患儿仍更易患右心室流出道梗阻（18例中有5例[27.8%]，而224例中有14例[6.3%]，OR 5.77，CI 1.54 - 20.92，P = 0.0077）。两项分析均显示，与无CSP的双胎相比，CSP双胎的Quintero分期更高，但患者特征和30天死亡率相似。CSP队列的亚组分析未发现术前特征或FE检查结果在预测CHD方面存在差异。
术前患有CSP的双胎受血儿出生后发生右心室流出道梗阻的风险增加，但尽管产前有这些显著的病理生理表现，胎儿激光手术后其生存率似乎相当。TTTS术前FE对于预测出生后CHD仍然很重要。
