Perioperative treatment of patients with pheochromocytoma/paraganglioma combined catecholamine cardiomyopathy / 中华泌尿外科杂志

ABSTRACT

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 (131 I-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 nonfunctional 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.