Division of Cardiovascular Diseases, Department of Internal Medicine, Mayo Clinic, Rochester, Minnesota 55905, USA.
J Card Fail. 2012 Feb;18(2):120-5. doi: 10.1016/j.cardfail.2011.10.012. Epub 2011 Dec 3.
Apical ballooning syndrome (ABS) and posterior reversible encephalopathy syndrome (PRES) are recently described, seemingly unrelated, reversible conditions. The precise pathophysiology of these syndromes remains unknown. The aim of this study was to describe the clinical characteristics and outcomes of a unique series of patients with both ABS and PRES.
In a retrospective study of 224 consecutive patients diagnosed with ABS between 2002 and 2010, 6 (2.7%) were also diagnosed with PRES. All were female with a mean age of 63.7 ± 12.5 years. All patients had preceding medical comorbidities and physical stress triggers that precipitated ABS and PRES. Mean peak troponin T levels and left ventricular ejection fraction at presentation were 0.47 ± 0.48 mg/dL and 31.5 ± 8.2%, respectively. Characteristic left ventricular wall motion abnormalities (regional wall motion score index 2.22 ± 0.37) were noted in all patients, and magnetic resonance imaging of the brain was significant for vasogenic edema, predominantly in the posterior circulation. All patients recovered left ventricular (ejection fraction at follow-up 60.2 ± 6.0%) and neurologic function with supportive management. Two patients had recurrence of ABS and 1 of PRES during follow-up.
ABS and PRES can occur simultaneously during an acute illness. Patients with ABS who develop neurologic dysfunction should be evaluated for PRES and vice versa. Because transient sympathetic overactivity and microvascular dysfunction have been observed in both reversible syndromes, we speculate that they may represent the shared pathophysiologic mechanism.
心尖球囊样综合征(ABS)和后部可逆性脑病综合征(PRES)是最近描述的两种看似不相关的、可逆转的疾病。这些综合征的确切病理生理学仍然未知。本研究的目的是描述一组具有 ABS 和 PRES 的独特患者的临床特征和结局。
在 2002 年至 2010 年间连续诊断为 ABS 的 224 例患者的回顾性研究中,有 6 例(2.7%)还被诊断为 PRES。所有患者均为女性,平均年龄为 63.7 ± 12.5 岁。所有患者均有先前的医疗合并症和导致 ABS 和 PRES 的躯体应激触发因素。发病时的肌钙蛋白 T 峰值水平和左心室射血分数分别为 0.47 ± 0.48 mg/dL 和 31.5 ± 8.2%。所有患者均存在特征性的左心室壁运动异常(区域性室壁运动评分指数 2.22 ± 0.37),脑部磁共振成像显示血管源性水肿,主要在后循环。所有患者均通过支持性治疗恢复左心室(随访时射血分数为 60.2 ± 6.0%)和神经功能。2 例患者在随访期间复发 ABS,1 例患者复发 PRES。
ABS 和 PRES 可在急性疾病期间同时发生。出现神经功能障碍的 ABS 患者应评估 PRES,反之亦然。因为在这两种可逆性综合征中都观察到短暂的交感神经过度活跃和微血管功能障碍,我们推测它们可能代表共同的病理生理机制。