Unexpected findings during the anesthetic management of a patient with a cardiac paraganglioma.

ARDIAC PARAGANGLIOMA is a rare and mostly benign neoplasm, arising from neuroendocrine cells, similar to pheochromocytoma in the adrenal glands. Endocrinologically active tumors may cause hypertension, arrhythmias, and other cardiac symptoms and mostly arise from the left atrium.1 Significant hemodynamic derangement may be encountered during anesthetic management and surgical manipulation. Paraganglioma without catecholamine secretion usually presents with symptoms of superior vena cava obstruction, pericardial effusion, or tamponade. 2 Some unexpected findings in a patient who underwent resection of a catecholamine-secreting cardiac paraganglioma are presented. CASE REPORT An otherwise healthy 67-year-old woman, with no prior relevant medical history, presented to the emergency room with a sudden onset of episodic tachyarrhythmias as well as multiple episodes of chest, arm, and jaw pain. The electrocardiogram showed T-wave inversions in leads V1 to V3 and Q waves in leads III and aVF. There were no symptoms of congestive heart failure. Coronary angiography revealed abnormal branches from the left anterior descending coronary artery and the conus of the right coronary artery (RCA) feeding a vascular mass medial to the right atrial appendage and anterior to the aorta, overlying the origin of the right coronary artery. Transthoracic echocardiography showed an extracardiac mass (4 6 4 cm) adjacent to the right atrium. The mass was also observed on a thoracic computed tomography (CT) scan (Fig 1). A small pericardial effusion was observed at the time. Transvascular biopsy of the mass, accessed through the right internal jugular vein, revealed pathology consistent with paraganglioma. Serum catecholamine levels of dopamine (345 pg/mL) and norepinephrine (526 pg/mL) were mildly elevated, with normal epinephrine (10 pg/mL) levels. Preoperative treatment with phenoxybenzamine, 10 mg orally twice a day, with close outpatient blood pressure monitoring was initiated. The patient was scheduled for surgical resection 2.5 months after the biopsy with stable blood pressure and no additional cardiac symptoms. Additional treatment with -blocking agents was not initiated because of the patient’s relatively low resting heart rate (62 beats/min). After arrival in the operating room, a preinduction arterial catheter was placed, and blood samples were taken for catecholamine levels (Table 1). The patient’s blood pressure was 140/75 mmHg, and her heart rate was 65 beats/min. Anesthesia was induced with fentanyl, 8 g/kg, etomidate, 20 mg, and rocuronium, 1 mg/kg intravenously, without causing any hemodynamic derangement. During laryngoscopy, the patient developed profound bradycardia progressing to asystole, lasting 8 seconds (by continuous recording), which spontaneously resolved once laryngoscopy was completed. No cardiopulmonary resuscitation or pharmacologic interventions were required. Anesthesia was maintained with sevoflurane, fentanyl, and rocuronium. After central venous catheter placement into the right internal jugular vein, the recorded initial central venous pressure (CVP) was 22 mmHg. A transesophageal echocardiography (TEE) probe was placed revealing a significant amount of pericardial effusion (Fig 2). A richly vascularized mass could be observed in the upper anterior region of the right ventricle (Fig 3). All valves appeared to be competent, myocardial contractility was preserved, and regional wall motion abnormalities were not observed. After median sternotomy and opening of the pericardium, 350 mL of clear pericardial fluid were evacuated; CVP immediately decreased to 15 mmHg. The tumor was visible anterior to the aorta and had a distinct vascular blush appearance. With the use of cardiopulmonary bypass and cardioplegic arrest, the tumor could be completely resected without injury to the RCA. Electrocautery and suture ligation of feeding vessels were used. There were no signs of additional tumor presence in the heart. The pericardial fluid was sent for cytology. After successful weaning from cardiopulmonary bypass, blood samples were taken for repeat assessment of catecholamine levels (Table 1). The patient was hemodynamically stable after resection of the mass and did not require any vasoactive medications to support her blood pressure. The postoperative course was uncomplicated, and she was discharged on postoperative day 4. Pathology of the mass confirmed the diagnosis of paraganglioma with an intact capsule. The cytology of the pericardial fluid was negative.