Hofbeck M, Deeg K H, Singer H, vd Emde J, Rein J
Kardiologische Abteilung, Universität Erlangen-Nürnberg.
Z Kardiol. 1989 Sep;78(9):607-10.
Rhabdomyomas are benign cardiac tumors often occurring as multiple lesions. We describe an infant with multiple cardiac rhabdomyomas causing severe right-ventricular and left-ventricular outflow tract obstructions. The intracavitary parts of the rhabdomyomas were successfully resected when the patient was 5 weeks old. Echocardiography showed regression in size of the remaining intramural tumors. Since there is only a low growth potential in cardiac rhabdomyomas, surgical management is indicated only in patients with hemodynamic obstruction or significant arrhythmias. Surgery should be primarily directed to relieve hemodynamic obstruction. If complete resection is not possible without damage to vital structures, resection should be restricted to the intracavitary parts of the tumor.
横纹肌瘤是一种常见的良性心脏肿瘤,常呈多发性病变。我们报道了一名患有多发性心脏横纹肌瘤的婴儿,该肿瘤导致严重的右心室和左心室流出道梗阻。患儿5周大时成功切除了瘤体的腔内部分。超声心动图显示,残留的壁内肿瘤体积缩小。由于心脏横纹肌瘤的生长潜能较低,仅在有血流动力学梗阻或严重心律失常的患者中考虑手术治疗。手术应主要针对解除血流动力学梗阻。如果在不损伤重要结构的情况下无法完全切除肿瘤,则应将切除范围限制在肿瘤的腔内部分。