ECMO联合肾上腺切除救治妊娠期嗜铬细胞瘤并儿茶酚胺心肌病一例并文献回顾

ECMO assisted adrenal excision for pheochromocytoma catecholamine cardiomyopathy during pregnancy:a case report and literature review

嗜铬细胞瘤是一组罕见的能够产生儿茶酚胺的肿瘤,临床主要以高血压、头痛、大汗、心悸为主,心源性休克很少为嗜铬细胞瘤的首发表现。笔者所在医院近期收治1例以心源性休克为首发临床表现的孕31+3周患者,初诊考虑为急性心肌炎并行静脉动脉体外膜肺氧合(VA-ECMO)治疗,心功能快速得到改善,但在ECMO撤机的过程中再度发生心源性休克,再次启动VA-ECMO辅助治疗。查腹部CT及24 h尿、血浆甲氧基肾上腺素类物质(MNs)明确诊断为嗜铬细胞瘤并儿茶酚胺心肌病,二次ECMO期间出现离心泵头血栓,在ECMO保护下紧急联合外科完成了肾上腺嗜铬细胞瘤切除,并于当日成功撤离ECMO,术后9 d产妇顺利出院。笔者总结本例产妇救治经过,并对相关病例报道进行回顾,为嗜铬细胞瘤并儿茶酚胺心肌病的临床救治提供参考。

Pheochromocytoma is a group of rare tumors produce catecholamines.Clinical manifestations are mainly hypertension,headache,sweating,and palpitation.Cardiogenic shock rarely appears as the first manifestation of pheochromocytoma.A pregnant patient of 31+3 weeks was admitted to our hospital recently with cardiogenic shock as the first clinical manifestation.Initial diagnosis was considered as acute myocarditis and veno-arterial extracorporeal membrane oxygenation(VA-ECMO)was started.Her cardiac function rapidly improved,however,cardiogenic shock occurred again during ECMO withdrawal,and VA-ECMO was started again.Though abdominal CT examination and 24 hour urine and plasma methoxyepinephrine substances(MNs),diagnosis of pheochromocytoma with catecholamine cardiomyopathy was confirmed.With ECMO assistance,adrenal pheochromocytoma was removed with surgery.The patient was successfully evacuated from ECMO on the surgery day,discharged successfully after 9 days.The authors summarized the treatments in this case and reviewed related case reports,which could provide reference to treat pheochromocytoma catecholamine cardiomyopathy.