Oberhoffer R, Cook A C, Lang D, Sharland G, Allan L D, Fagg N L, Anderson R H
Sektion Kinderkardiologie, Universitaets-KinderKlinik, Ulm, Germany.
Br Heart J. 1992 Dec;68(6):580-5. doi: 10.1136/hrt.68.12.580.
To assess the degree of agreement between the fetal echocardiographic and postmortem examination of hearts from fetuses with severe malformations of the tricuspid valve.
A retrospective study to analyse echocardiographic recordings and make comparisons with postmortem findings.
Tertiary referral centre for fetal echocardiography. Institute for cardiac morphology.
19 cases shown to have severe malformation of the tricuspid valve by fetal echocardiography that died in the prenatal or neonatal period.
Correlations between morphology and measurements made at echocardiography and necropsy.
The echocardiographic diagnosis was Ebstein's malformation in seven and tricuspid valvar dysplasia in 12 fetuses. These findings were confirmed in six and eight cases at necropsy. In one false positive diagnosis of Ebstein's malformation, necropsy showed dysplasia of the leaflets of the tricuspid valve without displacement. In four cases with the echocardiographic diagnosis of valvar dysplasia, necropsy showed displacement, the hallmark of Ebstein's malformation. Associated malformations that are known to worsen prognosis were predicted correctly by echocardiography. Taking the mean duration of four weeks between echocardiographic and postmortem investigations, both methods showed cardiomegaly causing lung hypoplasia, right atrial dilatation, and relative hypoplasia of the pulmonary trunk, morphometric factors that may be responsible for the poor outcome. Mostly good agreement existed between the echocardiographic and postmortem measurements if cases with an interval of more than eight weeks between the measurements were excluded.
Fetal echocardiography was proved to be a reliable technique in differentiating the variants of tricuspid valvar disease, in diagnosing associated cardiac lesions, and in predicting quantitative factors that can define the subsequent outcome.
评估胎儿超声心动图检查与患有严重三尖瓣畸形胎儿心脏尸检结果之间的一致性程度。
一项回顾性研究，分析超声心动图记录并与尸检结果进行比较。
胎儿超声心动图三级转诊中心。心脏形态学研究所。
19例经胎儿超声心动图显示患有严重三尖瓣畸形且在产前或新生儿期死亡的病例。
超声心动图检查与尸检时形态学及测量结果之间的相关性。
超声心动图诊断为埃布斯坦畸形的有7例胎儿，三尖瓣发育异常的有12例胎儿。尸检在6例和8例中证实了这些发现。在1例假阳性的埃布斯坦畸形诊断中，尸检显示三尖瓣叶发育异常但无移位。在4例超声心动图诊断为瓣膜发育异常的病例中，尸检显示有移位，这是埃布斯坦畸形的特征。已知会使预后恶化的相关畸形通过超声心动图被正确预测。以超声心动图检查与尸检之间平均四周的间隔时间计算，两种方法均显示心脏肿大导致肺发育不全、右心房扩张以及肺动脉相对发育不全，这些形态学因素可能是导致不良结局的原因。如果排除测量间隔超过八周的病例，超声心动图测量与尸检测量结果大多具有良好的一致性。
胎儿超声心动图被证明是一种可靠的技术，可用于区分三尖瓣疾病的变体、诊断相关心脏病变以及预测可确定后续结局的定量因素。
