Martínez-García Alfonso, Michel-Macías Carolina, Cordero-González Guadalupe, Escamilla-Sánchez Karla I, Aguinaga-Ríos Mónica, Coronado-Zarco Alejandra, Cardona-Pérez Jorge A
1Fetal and Pediatric Cardiology, Instituto Nacional de Perinatología Isidro Espinosa de los Reyes,Mexico City,Mexico.
2Neonatal Intensive Care Unit,Instituto Nacional de Perinatología Isidro Espinosa de los Reyes,Mexico City,Mexico.
Cardiol Young. 2018 Jul;28(7):903-909. doi: 10.1017/S1047951118000598. Epub 2018 May 15.
IntroductionIntracardiac rhabdomyomas can cause severe ventricular dysfunction and outflow tract obstruction.Case reportA term newborn infant with antenatal diagnosis of giant left ventricle rhabdomyoma presented with cardiac failure and duct-dependent systemic circulation after birth. She was treated successfully with everolimus, showing decrease in tumour size and improvement in left ventricular ejection fraction.DiscussionTumour regression rate was 0.32 cm2/day and improved to 0.80 cm2/day with the use of everolimus. Herein we report a newborn with inoperable giant left ventricular cardiac rhabdomyoma and significant regression of the tumour. To our knowledge, this is the largest left ventricular rhabdomyoma reported. A review of the literature was undertaken for comparison.
Everolimus has proven to be efficacious in size reduction of cardiac rhabdomyomas in cases when surgical resection is not possible.
引言
心脏横纹肌瘤可导致严重的心室功能障碍和流出道梗阻。
病例报告
一名足月新生儿，产前诊断为巨大左心室横纹肌瘤，出生后出现心力衰竭和依赖动脉导管的体循环。她接受依维莫司治疗成功，肿瘤大小减小，左心室射血分数改善。
讨论
肿瘤消退率为每天0.32平方厘米，使用依维莫司后提高到每天0.80平方厘米。在此，我们报告一名患有无法手术的巨大左心室心脏横纹肌瘤且肿瘤显著消退的新生儿。据我们所知，这是报告的最大的左心室横纹肌瘤。为作比较进行了文献综述。
已证明依维莫司在无法进行手术切除的情况下对减小心脏横纹肌瘤的大小有效。
