目的:有症状的新生儿和Ebstein异常(EA)的婴儿需要复杂的管理。美国胸外科协会委托了一组专家,以提供有关该主题的框架,重点是风险分层和管理。
方法:EA临床先天性实践标准委员会是一个由具有EA专业知识的外科医生和心脏病学家组成的多国和多学科小组。PubMed中的引文搜索,Embase,Scopus,WebofScience使用与EA相关的关键词进行。搜索仅限于英语和2000年或以后,并产生了455个结果,其中71例与新生儿和婴儿有关。使用改进的德尔菲法开发了具有建议类别和证据水平的专家共识声明,要求80%的成员投票,对每项声明至少有75%的同意。
结果:使用EA评估胎儿时,那些有严重心脏肥大的人,在导管水平逆行或双向分流,肺动脉瓣闭锁,圆形分流器,左心功能不全,或胎儿积水应被认为是宫内死亡和产后发病率和死亡率的高风险。患有EA和严重心脏肥大的新生儿,早产(<32周),宫内生长受限,肺动脉瓣闭锁,圆形分流器,左心功能不全,或心源性休克应被视为发病率和死亡率的高风险。具有圆形分流的血流动力学不稳定的新生儿应紧急中断圆形分流。难治性心源性休克的新生儿可以通过Starnes程序减轻。在Starnes手术后,可以评估儿童的后期双心室修复。可以监测没有EA高风险特征的新生儿的PDA自发关闭。血流动力学稳定的新生儿,有严重的肺反流,有正常RVSP的圆形分流的风险,应尝试对PDA进行医学封闭。应在患有功能性肺动脉闭锁和正常RVSP(>20-25mmHg)的新生儿中进行PDA闭合的医学试验。血流动力学稳定的新生儿,无肺返流,但顺行肺血流不足,可考虑使用PDA支架或全身性肺动脉分流术。
结论:对于Ebstein异常的新生儿和婴儿,危险分层是必不可少的。姑息舒适护理可能是合理的新生儿与相关的危险因素,可能包括早产,遗传综合征,其他主要的医疗合并症,心室功能障碍,或者败血症.患有圆形分流的不稳定新生儿应紧急中断圆形分流。不稳定的新生儿最常见于Starnes手术。稳定的新生儿应进行导管闭合。肺血流不足的稳定新生儿可能有导管支架置入术或全身至肺动脉分流术。Starnes姑息治疗后的后续手术包括单心室姑息治疗或双心室修复策略。
OBJECTIVE: Symptomatic neonates and infants with Ebstein anomaly (EA) require complex management. A group of experts was commissioned by the American Association for Thoracic Surgery to provide a framework on this topic focusing on risk stratification and management.
METHODS: The EA Clinical Congenital Practice Standards Committee is a multinational and multidisciplinary group of surgeons and cardiologists with expertise in EA. A citation search in PubMed, Embase, Scopus, and Web of Science was performed using key words related to EA. The search was restricted to the English language and the year 2000 or later and yielded 455 results, of which 71 were related to neonates and infants. Expert
consensus statements with class of recommendation and level of evidence were developed using a modified Delphi method, requiring 80% of members votes with at least 75% agreement on each statement.
RESULTS: When evaluating fetuses with EA, those with severe cardiomegaly, retrograde or bidirectional shunt at the ductal level, pulmonary valve atresia, circular shunt, left ventricular dysfunction, or fetal hydrops should be considered high risk for intrauterine demise and postnatal morbidity and mortality. Neonates with EA and severe cardiomegaly, prematurity (<32 weeks), intrauterine growth restriction, pulmonary valve atresia, circular shunt, left ventricular dysfunction, or cardiogenic shock should be considered high risk for morbidity and mortality. Hemodynamically unstable neonates with a circular shunt should have emergent interruption of the circular shunt. Neonates in refractory cardiogenic shock may be palliated with the Starnes procedure. Children may be assessed for later biventricular repair after the Starnes procedure. Neonates without high-risk features of EA may be monitored for spontaneous closure of the patent ductus arteriosus (PDA). Hemodynamically stable neonates with significant pulmonary regurgitation at risk for circular shunt with normal right ventricular systolic pressure should have an attempt at medical closure of the PDA. A medical trial of PDA closure in neonates with functional pulmonary atresia and normal right ventricular systolic pressure (>20-25 mm Hg) should be performed. Neonates who are hemodynamically stable without pulmonary regurgitation but inadequate antegrade pulmonary blood flow may be considered for a PDA stent or systemic to pulmonary artery shunt.
CONCLUSIONS: Risk stratification is essential in neonates and infants with EA. Palliative comfort care may be reasonable in neonates with associated risk factors that may include prematurity, genetic syndromes, other major medical comorbidities, ventricular dysfunction, or sepsis. Neonates who are unstable with a circular shunt should have emergent interruption of the circular shunt. Neonates who are unstable are most commonly palliated with the Starnes procedure. Neonates who are stable should undergo ductal closure. Neonates who are stable with inadequate pulmonary flow may have ductal stenting or a systemic-to-pulmonary artery shunt. Subsequent procedures after Starnes palliation include either single-ventricle palliation or biventricular repair strategies.