Orr William B, Chokshi Riti, Groh Georgeann, Silva Jennifer N Avari, Van Hare George F, Dalal Aarti S
Department of Pediatrics, Division of Pediatric, Division of Cardiology, Washington University School of Medicine, St. Louis, MO, USA.
Saint Louis Children's Hospital, St. Louis, MO, USA.
SAGE Open Med Case Rep. 2021 Feb 20;9:2050313X21994037. doi: 10.1177/2050313X21994037. eCollection 2021.
We describe a unique presentation of a pheochromocytoma in a normotensive teenager, who presented with symptoms of headache, neck pain, and palpitations. Holter and event monitor tracings revealed intermittent junctional rhythm causing electromechanical dyssynchrony between atrial and ventricular contraction resulting in reported symptoms. Exercise stress testing helped correlate symptomatic junctional rhythm events to episodic hypertension which led to the eventual diagnosis of pheochromocytoma. The exercise test provided insight into the physiologic coupling that the sympathetic and parasympathetic autonomic nervous systems have on the cardiovascular system during exercise and exaggerated hypertension. The patient was found to have MEN2A and partial adrenalectomy resulted in complete resolution of symptoms and arrhythmia. This unusual presentation illustrates the benefit of a comprehensive clinical evaluation, which led to the eventual diagnosis.
我们描述了一名血压正常的青少年嗜铬细胞瘤的独特表现，该患者出现头痛、颈部疼痛和心悸症状。动态心电图和事件监测仪记录显示间歇性交界性心律，导致心房和心室收缩之间的机电不同步，从而引发所报告的症状。运动应激试验有助于将有症状的交界性心律事件与发作性高血压相关联，最终导致嗜铬细胞瘤的诊断。运动试验深入了解了交感和副交感自主神经系统在运动和高血压加剧期间对心血管系统的生理耦合作用。发现该患者患有MEN2A，部分肾上腺切除术使症状和心律失常完全缓解。这种不寻常的表现说明了全面临床评估的益处，最终促成了诊断。
