摘要
目的分析嗜铬细胞瘤/副神经节瘤(PCC/PGL)相关儿茶酚胺心肌病的临床特征、治疗及预后。方法回顾性分析2021年5月前在北京协和医院诊治的35例PCC/PGL合并儿茶酚胺心肌病病例。结果35例患者中25例为PCC,10例为PGL;所有患者均出现血压升高,出现急性左心衰竭表现15例,阵发性头痛、大汗、心悸三联征11例,胸痛9例;19例心肌钙蛋白I升高;心电图主要异常表现为ST-T改变、左心室肥厚、QTc延长及心律失常;所有患者均出现超声心动图异常改变,主要表现为心室壁运动异常、左心室肥厚、左心室射血分数下降、心肌回声增强、左心室扩大;24例复查超声心动图,20例异常改变完全或部分恢复;8例超声心动图表现符合应激性心肌病改变;6例行心脏磁共振检查,均可见心肌斑片状延迟钆强化。随访23例患者,18例血压和症状控制良好,未再发心血管事件;1例死亡。结论儿茶酚胺心肌病是一种可能逆转的心肌病,可以出现多种心肌病表型,对于出现心力衰竭、心肌缺血、心律失常等心血管异常表现的PCC/PGL患者,应考虑儿茶酚胺心肌病可能。对PCC/PGL相关儿茶酚胺心肌病患者应积极抗心力衰竭等治疗,尽早手术。
Objective To analyze the clinical characteristics,treatment and prognosis of patients with pheochromocytoma(PCC)/paraganglioma(PGL)related catecholamine cardiomyopathy.Methods A total of 35 cases with the diagnosis of PCC/PGL related catecholamine cardiomyopathy admitted to Peking Union Medical College Hospital before May 2021 were retrospectively enrolled.Results Among the 35 cases enrolled,25 were PCC and other 10 were PGL.All the enrolled patients had hypertension,of which 15 cases had acute left heart failure,11 cases had"triad"including paroxysmal headache,perspiration and palpitation,and 9 cases had chest pain.Cardiac troponin I was elevated in 19 cases.The main electrocardiogram abnormalities included ST-T changes,left ventricular hypertrophy,prolonged QTc and arrhythmia.All cases had abnormal echocardiographic changes,which mainly showed abnormal ventricular wall motion,left ventricular hypertrophy,left ventricular ejection fraction decreased,myocardial echo enhancement,left ventricular enlargement.Echocardiography was reviewed in 24 patients with complete/partial recovery in 20 patients.Echocardiographic findings of 8 cases were consistent with stress cardiomyopathy.Patchy myocardial late gadolinium enhancement was found in all 6 cases with cardiac magnetic resonance.We successfully followed up 23 patients,of whom 18 had good symptom control and no recurrent cardiovascular events.Conclusion PCC/PGL realated catecholamine cardiomyopathy is a reversible cardiomyopathy,which can present a variety of phenotypes.The possibility of catecholamine cardiomyopathy should be considered for PCC/PGL with cardiovascular abnormalities such as heart failure,myocardial ischemia and arrhythmia.Patients with PCC/PGL related catecholamine cardiomyopathy should be actively treated with anti-heart failure in order to undergo early surgical removal of PCC/PGL.
作者
莫小凤
童安莉
林雪
方理刚
Mo Xiaofeng;Tong Anli;Lin Xue;Fang Ligang(Department of Cardiology,State Key Laboratory of Complex Severe and Rare Diseases,Peking Union Medical College Hospital,Chinese Academy of Medical Science and Peking Union Medical College,Beijing 100730,China;Department of Endocrinology,Key Laboratory of Endocrine,Ministry of Health,Peking Union Medical College Hospital,Chinese Academy of Medical Science and Peking Union Medical College,Beijing 100730,China;Department of Cardiology,Shenzhen Luohu People's Hospital,Shenzhen 518005,China)
基金
中央高水平医院临床科研业务经费(2022-PUMCH-B-098)。
