Morin Melinda J, Hopkins Richard A, Ferguson William S, Ziegler James W
Division of Pediatric Critical Care, Department of Pediatrics, Rhode Island Hospital and Brown University School of Medicine, Providence, Rhode Island 02903, USA.
Pediatrics. 2004 Apr;113(4):e374-6. doi: 10.1542/peds.113.4.e374.
This report describes a new etiology of pediatric syncope. Epilepsy, brain anomalies, infection, electrolyte abnormalities, and trauma are commonly identified etiologies of seizures in the pediatric population. We report here a child with third-degree heart block and right ventricular outflow tract obstruction related to an intracardiac tumor presenting with syncope and seizure-like activity. Echocardiography revealed a large (3 x 8-cm) intracardiac mass filling the right atrium, extending across the tricuspid valve into the right ventricle and crossing the atrial septum into the left atrium, extending into the left ventricular outflow tract. She underwent emergent cardiopulmonary bypass with removal of the majority of the tumor mass, clearing both the left and right ventricular outflow tracts of obstruction and repairing the tricuspid valve. Postoperative cardiac conduction remained blocked and required permanent pacing. The initial serum alpha-fetoprotein level was grossly elevated, and the tumor showed characteristic histopathologic features of a yolk sac tumor. Four years after the completion of her chemotherapy, she remains clinically well, with no evidence of recurrent tumor by echocardiography or radiographic studies, and her alpha-fetoprotein remains in the normal range. The clinical manifestations of tumor infiltration of the heart with complete heart block resulting in loss of consciousness with tonic-clonic movements are detailed. Although rare, cardiac syncope has multiple known causes and should be suspected in any patient with sudden loss of consciousness and pallor. In the pediatric population, cardiac rhythm disturbances are typically the result, rather than the cause, of acute cardiac emergencies. Pediatricians should be aware of depressed cardiac output and dysrhythmias as etiologies of new-onset syncope. Evaluation should include a cardiac assessment with electrocardiogram to exclude a life-threatening arrhythmia as a potential cause.
本报告描述了小儿晕厥的一种新病因。癫痫、脑畸形、感染、电解质异常和创伤是儿科人群中常见的癫痫发作病因。我们在此报告一名患有三度心脏传导阻滞和右心室流出道梗阻的儿童,其与心脏内肿瘤有关,表现为晕厥和癫痫样发作。超声心动图显示一个巨大的(3×8厘米)心脏内肿块,充满右心房,穿过三尖瓣延伸至右心室,并穿过房间隔进入左心房,延伸至左心室流出道。她接受了紧急体外循环,切除了大部分肿瘤肿块,清除了左、右心室流出道的梗阻,并修复了三尖瓣。术后心脏传导仍处于阻滞状态,需要永久起搏。初始血清甲胎蛋白水平显著升高,肿瘤显示出卵黄囊瘤的特征性组织病理学特征。化疗结束四年后,她临床状况良好,超声心动图或影像学检查均未发现肿瘤复发迹象,且甲胎蛋白仍在正常范围内。详细描述了肿瘤浸润心脏导致完全性心脏传导阻滞,进而引起意识丧失伴强直阵挛运动的临床表现。尽管罕见,但心脏性晕厥有多种已知病因,任何突然意识丧失和面色苍白的患者都应怀疑为此病因。在儿科人群中,心律失常通常是急性心脏急症的结果而非原因。儿科医生应意识到心输出量降低和心律失常是新发晕厥的病因。评估应包括通过心电图进行心脏评估,以排除危及生命的心律失常作为潜在病因。