Department of Obstetrics and Fetal Medicine, Leiden University Medical Center, Leiden, Netherlands.
Department of Obstetrics and Gynecology, Center for Fetal Medicine, Karolinska University Hospital, Stockholm, Sweden.
Prenat Diagn. 2021 May;41(6):754-765. doi: 10.1002/pd.5907. Epub 2021 Feb 26.
The limited number of large fetal cohort studies on common arterial trunk (CAT) impedes prenatal counseling at midgestation. This study evaluates the prognosis of CAT from a fetal perspective.
Fetuses with a prenatally diagnosed CAT were extracted from the PRECOR registry (2002-2016). We evaluated fetal and postnatal survival and the presence of additional morbidity at last follow-up. Literature databases were searches systematically for additional cases.
Thirty-eight cases with a prenatal diagnosis of CAT were identified in our registry, of which 18/38 (47%) opted for pregnancy termination (TOP). Two cases resulted in spontaneous intrauterine demise (10%, 2/20), six cases demised postnatally (33%, 6/18), leaving 60% (12/20) alive, after exclusion of TOP, at a mean age of six (range: 2-10 years). Additional morbidity was found in 42% (5/12) of survivors, including 22q11.2 deletion syndrome, Adams-Oliver syndrome and intestinal atresia, whereas 8% (1/12) had developmental delay. The remaining 30% (6/12) of survivors appeared isolated with normal development. All of whom six required replacement of the initial right ventricle to pulmonary artery conduit. Additionally, we reviewed 197 literature cases on short-term outcome.
The risk of fetal and neonatal demise, as well as significant morbidity amongst survivors, should be included in prenatal counseling for CAT.
有限数量的关于共同动脉干(CAT)的大型胎儿队列研究阻碍了妊娠中期的产前咨询。本研究从胎儿角度评估 CAT 的预后。
从 PRECOR 登记处(2002-2016 年)提取出产前诊断为 CAT 的胎儿。我们评估了胎儿和围生期的存活率以及最后一次随访时是否存在其他发病率。系统地搜索文献数据库以获取其他病例。
在我们的登记处发现了 38 例产前诊断为 CAT 的病例,其中 18/38(47%)选择终止妊娠(TOP)。2 例自发宫内死亡(10%,2/20),6 例围生期死亡(33%,6/18),在排除 TOP 后,60%(12/20)存活,平均年龄为 6 岁(范围:2-10 岁)。在幸存者中发现了 42%(5/12)的额外发病率,包括 22q11.2 缺失综合征、Adams-Oliver 综合征和肠闭锁,而 8%(1/12)有发育迟缓。其余 30%(6/12)的幸存者表现孤立,发育正常。他们均需要更换最初的右心室至肺动脉导管。此外,我们还回顾了 197 篇关于短期预后的文献病例。
在 CAT 的产前咨询中,应包括胎儿和新生儿死亡的风险,以及幸存者中存在的严重发病率。