We report a case of general anesthesia for the removal of pheochromocytoma in a patient complicated with severe hypertrophic obstructive cardiomyopathy. A 65-year-old woman complained of fatigability with hypertension and diabetes mellitus. She was diagnosed as an extra-adrenal peri-aortic pheochromocytoma with severe hypertrophic obstructive cardiomyopathy. The left ventricular outflow gradient (LVOG) was 199 mmHg and the serum noradrenaline level was 13,567 pg x ml(-1) (100-450). As a preoperative management, atenolol, verapamil and disopyramide were given to decrease LVOG. Then doxazosin was given to control hypertension and to increase the circulating blood volume without deteriorating the outflow tract obstruction. LVOG decreased to 50 mmHg preoperatively. Anesthesia was given with propofol, fentanyl, remifentanil and isoflurane with a continuous infusion of diltiazem. The circulating blood volume was maintained with adequate volume loading assessed by the measurement of the left ventricular end-diastolic diameter and LVOG with transesophageal echocardiography. After the removal of the tumor, continuous infusion of noradrenaline was given to maintain the blood pressure. She was extubated in the ICU. LVOG decreased to 20 mmHg with stable hemodynamics on the second postoperative day. She was discharged from the ICU without any adverse cardiac events during the perioperative period.

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