Lillie Frank Abercrombie Section of Cardiology, Department of Pediatrics, Texas Children's Hospital, Baylor College of Medicine, Houston, TX, USA.
Section of Hematology/Oncology, Vascular Anomalies Center, Department of Pediatrics, Texas Children's Hospital, Baylor College of Medicine, Houston, TX, USA.
J Neonatal Perinatal Med. 2021;14(1):143-148. doi: 10.3233/NPM-190330.
Cardiac rhabdomyomas can be prenatally diagnosed in patients with tuberous sclerosis complex. Many neonates require no intervention in early life other than close monitoring for regression of tumor over the period of months to years. In rare instances, cardiac rhabdomyomas can result in obstruction to blood flow or decreased ventricular function.
We describe the case of a neonate who was prenatally diagnosed with multiple large cardiac rhabdomyomas, one of which caused clinically significant obstruction to prograde blood flow across the tricuspid valve in the newborn period. To address the disturbance to prograde pulmonary blood flow, the patient underwent successful ductal stent placement in the neonatal period. A troponin elevation was noted shortly after birth, but no evidence of coronary compression or involvement was demonstrated by coronary angiography. The patient has subsequently been treated with sirolimus over a period of three months, with noted regression in tumors and improvement in tricuspid valve inflow.
A brief review of the literature regarding the diagnosis, treatment, and management of neonatal patients with cardiac rhabdomyomas is presented. A combined percutaneous and medical management approach may be of benefit in future cases of rhabdomyomas causing obstruction to pulmonary blood flow.
结节性硬化症患者的心脏横纹肌瘤可在产前诊断。许多新生儿在生命早期无需干预，只需密切监测数月至数年内肿瘤的消退情况。在极少数情况下，心脏横纹肌瘤可导致血流阻塞或心室功能降低。
我们描述了一例新生儿，其在产前被诊断为多个大型心脏横纹肌瘤，其中一个导致三尖瓣前向血流在新生儿期出现临床显著阻塞。为了解决前向肺血流的障碍，患者在新生儿期成功进行了动脉导管支架置入术。出生后不久发现肌钙蛋白升高，但冠状动脉造影未显示冠状动脉受压或受累的证据。此后，该患者接受了为期三个月的西罗莫司治疗，肿瘤明显消退，三尖瓣流入道改善。
本文简要回顾了新生儿心脏横纹肌瘤的诊断、治疗和管理方面的文献。对于因肺血流阻塞而导致的横纹肌瘤病例，经皮介入和药物联合治疗方法可能具有一定的应用价值。
