伴儿茶酚胺心肌病的嗜铬细胞瘤／副神经节瘤的围手术期处理经验

Perioperative treatment of patients with pheochromocytoma/paraganglioma combined catecholamine cardiomyopathy

目的 总结伴儿茶酚胺心肌病的嗜铬细胞瘤/副神经节瘤（PHEO/PGL）患者的心功能变化、诊断及围手术期治疗经验。方法 回顾性分析2008年1月至2018年3月收治的15例PHEO/PGL伴儿茶酚胺心肌病患者的临床资料，男8例，女7例。年龄13-64岁，平均32.8岁。左侧PHEO 4例，右侧PHEO 4例，双侧PHEO 3例（其中VHL病2例），颈静脉体PGL 1例，左腹膜后PGL 2例，右腹膜后PGL 1例。肿瘤直径2.3-7.2 cm。14例术前确诊为PHEO/PGL伴儿茶酚胺心肌病，有头痛、心悸、大汗等典型PHEO/PGL临床表现；24 h尿儿茶酚胺检查示肾上腺素平均24.87 μg/24 h，去甲肾上腺素平均551.70μg/24 h，多巴胺平均395.41μg/24 h；7例奥曲肽检查及131I-间碘苄胍显像均阳性，6例奥曲肽检查阳性，1例肾上腺髓质全身131I-间碘苄胍显像阳性。增强CT检查示腹膜后肾上腺区或肾上腺外明显不均匀强化肿物，内部可见缺血坏死区，部分瘤体血运极丰富，可见肿瘤周边呈蔓状分布的不规则血管网。超声心动图检查示儿茶酚胺心肌病发作时左室射血分数（LVEF）为（42.0±7.1）%（32%-54%），其中轻度异常（LVEF 45%-54%）5例，中度异常（LVEF 30%-44%）9例，伴左心室肥厚、心肌回声增强、左心扩大、心肌运动减弱等儿茶酚胺心肌病表现。9例严重心功能不全患者伴急性左心衰，表现为肺水肿、咳大量粉红色泡沫痰。此14例予规律α受体阻滞剂、β受体阻滞剂及钙离子拮抗剂治疗1-3个月，怀疑肺部感染者加用敏感抗生素，并予强心、利尿等治疗，待心功能显著改善后手术。1例为隐匿型PHEO，缺乏典型临床表现及影像学改变，24 h尿儿茶酚胺检查及奥曲肽显像均无异常；术前超声心动图检查示心功能正常，LVEF为73%；术前诊断为右肾上腺无功能腺瘤，未行药物准备。本组15例，行腹腔镜手术13例，其中1例中转开放手术；开放手术2例（均为PGL患者）。结果 本组15例手术均完整切除肿瘤，1例隐匿型PHEO术中触碰瘤体时出现血压剧烈波动，血压190/130-80/50 mmHg（1 mmHg＝0.133 kPa），心率150次/分，术后于ICU病房出现儿茶酚胺心肌病表现，超声心动图检查示LVEF为37%，左心扩大且心肌普遍收缩不良；纤维支气管镜检查示双肺粉红色泡沫痰；胸部X线片检查示双侧肺水肿、左侧为著。确诊为儿茶酚胺心肌病，经治疗后心功能恢复。14例术前确诊病例，药物治疗后LVEF为（55.9±7.6）%，与治疗前比较差异有统计学意义（P＝0.041）；术后LVEF为（66.1±8.5）%，与药物治疗后比较差异有统计学意义（P＝0.013）。本组15例术后随访1个月-10年，均无复发。结论 PHEO/PGL伴儿茶酚胺心肌病患者如诊断明确，需行充分药物准备，发生心功能异常需积极对症治疗，心功能异常可在肿瘤切除后有效改善。隐匿型PHEO围手术期风险大，对于疑似隐匿型PHEO需积极药物准备后再行手术治疗。

Objective To discuss the diagnosis, perioperative treatment, cardiac function changing of pheochromocytoma/paraganglioma （PHEO/PGL）patients with catecholamine cardiomyopathy.Methods Fifteen PHEO/PGL patients with catecholamine cardiomyopathy were included in our hospital from Jan 2008 to Mar 2018. There were 8 males and 7 females with an average age of 32.8 years, ranging 13-64 years old.4 cases were found left PHEO. 4 cases were found right PHEO and 3 cases were bilateral PHEO, including 2 cases of VHL. One case was jugular PGL. 2 cases were lift PGL and 1 case was right PGL. The tumors diameter ranged from 2.3 to 7.2 cm. 14 patients were diagnosed as PHEO/PGL with catecholamine cardiomyopathy with typical clinical manifestations of PHEO/PGL, such as headache, palpitation and perspiration. Their 24 hours of urine catecholamines showed an average of 24.87 μg/24 h in epinephrine and 551.70 μg/24 h in norepinephrine. Their average value of dopamine was 395.41 μg/24 h. Among 7 cases, the octreotide scan and Iodine-131-meta-iodobenzylguanidine（131I-MIBG）s scan were positive in 6 and 1 case, respectively. Enhanced CT showed significantly heterogeneous enhancement tumors in adrenal or retroperitoneal area. Ischemia and necrosis area were found inside tumors. The blood flow of some tumors were extremely rich and irregular vascular network with a racemose distribution around the tumors could be observed. Echocardiography showed that the left ventricular ejection fraction （LVEF） ranged from 32% to 54%, mean （42.0±7.1） % during catecholamine cardiomyopathy. 5 cases were mild abnormal, which the LVEF ranged from 45% to 54%. 9 cases were moderate abnormal, which the LVEF ranged from 30% to 44%.Their catecholamine cardiomyopathy performance including left ventricular hypertrophy, myocardial echo enhancement and left ventricular enlargement.9 cases combined with acute left ventricular failure manifested as pulmonary edema and pink foamy phlegm. Sensitive antibiotics were given to suspected pulmonary infection patients. 14 cases accepted regular alpha blockers, beta blockers and calcium antagonists treatment for 1 to 3 months and underwent surgery after a significant improvement in cardiac function.1 non-functional PHEO patient was lack of typical clinical and imaging changing. Both of her 24 hours of urine catecholamines and octreotide scan were negative. Echocardiography showed that LVEF was 73% before operation. She was misdiagnosed as an adrenal non-functional adenoma. All patients underwent surgical treatment, including 13 laparoscopic surgery.One case switched to open surgery. 2 PGL patients all accepted open surgery.Rusults All cases accepted complete resection of the tumors. 1 case of non-functional PHEO had severe fluctuations in blood pressure during operation. Her BP were 190/130 to 80/50 mmHg （1 mmHg=0.133 kPa） and heart rate raised to 150 bpm. Catecholamine cardiomyopathy appeared in ICU ward after operation. Echocardiography showed that LVEF was 37%. The left ventricular enlargement and myocardial systolic function decreased. Fibrous bronchoscopy showed pink foamy phlegm in both sides of lungs. Chest X ray showed bilateral pulmonary edema which predominant in left side. The diagnosis was catecholamine cardiomyopathy of this patient. The LVEF was （55.9±7.6）% after treatment in 14 PHEO/PGL patients with catecholamine cardiomyopathy, and there was statistically significant difference between before and after treatment （P=0.041）. The LVEF was （66.1±8.5）% postoperation, and there was statistically significant difference between postoperation and after treatment （P=0.013）. The non-functional PHEO case occurred severe catecholamin cardiomyopathy after operation and cardiac function recovered after treatment. 15 cases were followed up from 1 months to 10 years without recurrence.Conclusions PHEO/PGL patients with catecholamine cardiomyopathy should have adequate medication. The abnormal cardiac function of patients would be reversed after surgical treatment. Cardiac function failure would be recovered after active treatment. Non-functional pheochromocytoma patients were extremely dangerous. Adequate medication should be given to any suspected non-functional pheochromocytoma patients.