Department of Obstetrics and Gynecology, Division of Maternal-Fetal Medicine, Mount Sinai Hospital, University of Toronto, Toronto, Canada.
Prenat Diagn. 2010 Oct;30(10):941-9. doi: 10.1002/pd.2590.
To determine the natural history and outcome of fetal cardiac tumors.
This was a retrospective cohort study of all prenatally detected cases of cardiac tumors at a tertiary cardiac care center.
Forty fetuses were identified to have one or several cardiac tumors in association with fetal hydrops (18%), ventricular obstruction (30%) and/or arrhythmia (13%). Of 33 cases with rhabdomyoma, three patients elected to terminate the pregnancy, four offspring died at birth and 26 (79%) survived. On follow-up, 95% of all live-born cases with rhabdomyomas were free of cardiac symptoms but 88% had tuberous sclerosis. All three fetuses with teratoma presented with hydrops and none of them survived. In contrast, all three fetuses with cardiac fibroma are alive and have a biventricular physiology. One fetus with a large atrial hemangioendothelioma died in early infancy. Fetal or neonatal death was associated with an earlier cardiac anomaly diagnosis, earlier delivery, larger tumor size and fetal hydrops at presentation.
The outcome of fetal cardiac tumors was predicted by the etiology and size of the cardiac mass and the presence of hydrops. Although most cardiac rhabdomyomas have a relatively benign perinatal course, the long-term prognosis is determined by the neurological manifestations associated with tuberous sclerosis.
确定胎儿心脏肿瘤的自然病史和结局。
这是一项在一家三级心脏护理中心对所有产前发现的心脏肿瘤病例进行的回顾性队列研究。
40 例胎儿被诊断为存在一个或多个心脏肿瘤，并伴有胎儿水肿（18%）、心室阻塞（30%）和/或心律失常（13%）。在 33 例横纹肌瘤病例中，有 3 例患者选择终止妊娠，4 例新生儿死亡，26 例（79%）存活。随访时，所有存活的横纹肌瘤患儿 95%无心脏症状，但 88%有结节性硬化症。所有 3 例患有畸胎瘤的胎儿均出现水肿，且均未存活。相比之下，所有 3 例患有心脏纤维瘤的胎儿均存活且具有双心室功能。1 例大型心房血管内皮细胞瘤胎儿在婴儿早期死亡。胎儿或新生儿死亡与心脏畸形的早期诊断、更早分娩、更大的肿瘤大小和产前水肿有关。
心脏肿瘤的病因、肿瘤大小和是否存在水肿可预测胎儿心脏肿瘤的结局。虽然大多数胎儿横纹肌瘤在围产期具有相对良性的病程，但长期预后取决于与结节性硬化症相关的神经系统表现。
