Solomon Julia E, Stock John H, Richardson Randy R, Silverman Norman H
Division of Maternal Fetal Medicine, Fetal Diagnostic Center, St. Joseph's Hospital and Medical Center-Dignity Health, Phoenix, Arizona.
AJP Rep. 2013 Oct;3(2):97-102. doi: 10.1055/s-0033-1344004. Epub 2013 May 21.
We report the accurate prenatal diagnosis at 22 weeks gestation of right atrial isomerism in association with tricuspid atresia. Several distinctive sonographic features of isomerism of the right atrial appendages were present in this fetus: complex cardiac abnormality, ventriculoarterial discordance, juxtaposition of the aorta and the inferior vena cava to the right side, pulmonary atresia, and anomalous pulmonary venous return to the morphological right atrium. Tricuspid atresia, which is an extremely rare lesion within heterotaxy spectrum disorders, was present. Postnatal investigations confirmed all prenatally diagnosed abnormalities, with additional findings of pulmonary atresia with discontinuous pulmonary arteries and bilateral arterial ducts, asplenia, and bilateral eparterial bronchi. To our knowledge, tricuspid atresia in the setting of isomerism of the right atrial appendages has not previously been diagnosed or reported prenatally. Because of the complexity of cardiac lesions that may be present in cases of atrial isomerism, these disorders should be considered even if sonographic findings are uncommon or atypical.
我们报告了一例妊娠22周时准确产前诊断为右心房异构合并三尖瓣闭锁的病例。该胎儿存在右心耳异构的几个独特超声特征：复杂心脏异常、心室动脉不一致、主动脉和下腔静脉并列于右侧、肺动脉闭锁以及异常肺静脉回流至形态学右心房。还存在三尖瓣闭锁，这在心脏异构谱系障碍中是一种极其罕见的病变。产后检查证实了所有产前诊断的异常情况，另外还发现了肺动脉闭锁伴肺动脉中断和双侧动脉导管、无脾以及双侧动脉上支气管。据我们所知，此前尚未有产前诊断或报道过右心耳异构情况下的三尖瓣闭锁。由于心房异构病例中可能存在的心脏病变复杂，即使超声检查结果不常见或不典型，也应考虑这些疾病。
