Clinical Study Of 31 Cases With Pheochromocytoma/paraganglioma Related Catecholamine Cardiomyopathy

Background:Pheochromocytoma/paraganglioma is a rare neuroendocrine tumor and it can secrete catecholamines causing a variety of cardiovascular damage,including cardiomyopathy.At present,there are few clinical studies on pheochromocytoma/paraganglioma related catecholamine cardiomyopathy,and little is known about it.Objective:Analyze the clinical characteristics,treatment and prognosis of patients with pheochromocytoma/paraganglioma related catecholamine cardiomyopathy.Methods:The clinical characteristics,treatment and prognosis of all patients diagnosed with pheochromocytoma/paraganglioma complicated with catecholamine cardiomyopathy in our hospital were retrospectively analyzed.Results:A total of 31 patients with definite diagnosis of Pheochromocytoma/Paraganglioma and Catecholamine Cardiomyopathy were finally enrolled in the group.All patients had elevated blood pressure,with a marked increase in blood pressure(systolic>200mmHg and/or diastolic>130mmHg)in 71%patients.Paroxysmal headache,sweating and palpitation were found in 9 cases(29.0%),chest pain in 8 cases(26%),and acute heart failure in 11 cases(36%).Elevation of Troponin I occurred in 52%of the patients,but coronary examination was usually negative.The main electrocardiogram abnormalities were ST-T changes(71%),left ventricular hypertrophy(29%)and prolonged QTc(61%).Echocardiographic abnormalities were observed in all patients,mainly manifested as abnormal ventricular wall motion(74%),left ventricular hypertrophy(61%),decreased left ventricular ejection fraction(45%),enhanced myocardial echogenicity(35%),and left ventricular enlargement(23%).Echocardiography was reviewed in 22 patients 8?210 days after the first echocardiography.,with complete/partial recovery in 18 patients(82%)and no significant change in 4 patients(18%).Echocardiography in 7 cases indicated that the decreased ventricular wall motion and/or LVEF were recovered in a short time,with an average of 27±11(10-42)days,which was consistent with the feature of stress cardiomyopathy.Magnetic resonance imaging showed patchy delayed enhancement in 5 patients.71%of the patients underwent surgical resection of the tumor.Among the 19 patients who were followed up,18 underwent surgical resection of tumor/MIBG treatment,and their symptoms were well controlled without recurrence of cardiovascular events.One patient still had hypertension because of the discontinuation of phenobenzamine without surgery.Conclusion:Catecholamine cardiomyopathy can cause cardiac failure,myocardial ischemia,arrhythmia and other cardiovascular abnormalities and a variety of cardiomyopathy phenotypes.The the left ventricular function can be restored to normal or increased in acute catecholamine cardiomyopathy,while the left ventricular function in the other patients can be totally or partially recovered only after resection of the tumor and following treatment with neuroendocrine inhibitors Catecholamine cardiomyopathy.