OBJECTIVES: To outline and compare the clinical features, preoperative preparation, perioperative management, and outcome of Takotsubo cardiomyopathy (TCM) and dilated cardiomyopathy (DCM) associated with pheochromocytomas and paragangliomas (PPGLs). DESIGN: A retrospective cohort study. SETTING: A single tertiary hospital. PARTICIPANTS: All patients scheduled for elective surgery of PPGL resection with TCM and DCM between March 2005 and June 2023 were enrolled. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: This study enrolled 29 patients: 20 patients were in the TCM group and 9 patients were in the DCM group. The tumor size of the DCM group was bigger and the level of 24-hour urine norepinephrine was higher than those in the TCM group. After the preoperative medication preparation (111 median days) and anti-heart failure treatment (if necessary), the mean preoperative ejection fraction in the TCM group was significantly higher than that in the DCM group (66.8% ± 4.4% v 48.8% ± 7.8%, p<
0.001), both elevated compared to ejection fraction at presentation (p<
0.001). The intraoperative hemodynamic instability score was rather high in PPGL-TCM and PPGL-DCM patients (84.6 points), as well as in the hemodynamic variables section (13.7 points). Patients with DCM were more prone to present hemodynamic disturbances and to require a lower volume of fluids but a higher infusion of vasoactive agents than patients with TCM. The incidence of complications was 6.9% and there was no perioperative mortality. CONCLUSIONS: Following the preoperative medication preparation and anti-heart failure treatment, patients with TCM had better left ventricular recovery before surgery and fewer cardiovascular risks compared to patients with DCM. Optimal perioperative management and individualized anesthetic strategies are essential for this unique patient population.