Publication of patient data was performed in accordance with the ethical guidelines of the institution. A 37-year-old white woman with no medical history was admitted to the emergency department of a referral institution for severe left- sided chest pain and fever. The patient was severely ill with profound fatigue and rigors. Her initial vital signs included a temperature of 38?C, a blood pressure of 80/60 mm Hg, and tachycardia to 130 beats per minute. There was no evidence of ischemia on electrocardiogram, and chest x-ray revealed a left upper lobe consolidation. Laboratories revealed a leukocytosis of 26290/mm³, C-reactive protein of 126 mg/L, lactate of 4.5 mmol/L, and a negative ? human chorionic gonadotropin. Fluid resuscitation and antiobiotic therapy were started for a presumed pneumonia. Further workup included a computed tomography of the chest, abdomen, and pelvis, which revealed diffuse pulmonary edema and a 7-cm diameter hypervascular lesion of the left adrenal gland Fig. 1. The patient was transferred to our region referral center for further intensive care unit management. The patient became progressively hypoxic and hypotensive, requiring mechanical ventilation and inotropic support. Echocardiography revealed a severe global hypokinesia with a left ventricular ejection fraction of 15%. Despite maximal ionotropic support, adequate perfusion could not be achieved and arterio-venous (A-V) extracorporeal membrane oxygenation was started.

Elevated urinary metanephrine and normetanephrine (73 750 nmol per 24 hours and 330 630 nmol per 24 hours) confirmed the suspicion of pheochromocytoma and acute catecholamine-induced cardiac failure. A magnetic resonance imaging confirmed the presence of an isolated large left adrenal mass without evidence of a secondary or ectopic pheochromocytoma. Medical therapy was immedi- ately started with calcium blockers (nicardipine chlorhydrate 50 mg daily) to reverse the catecholamine-induced cardiac failure. The left ventricular ejection fraction improved to 65%, and ECMO was stopped after 11 days. An open left adrenalectomy was performed 10 days after the discontin- uation of ECMO. The postoperative course was uneventful, and the patient was discharged on the fourth postoperative day. At 1-year follow-up, the patient is in good health with a normal cardiac function.

The life-threatening Cardiovascular complications of pheochromocytomas are due to the effects of high-level secreted catecholamines [2]. Different factors have been

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1017.e2 Case Report

Pheochromocytomas are rare but can present with dramatic Cardiovascular collapse. The diagnosis should be suspected early, especially in young patients. With Timely diagnosis and medical therapy, followed by surgical resection, the Cardiovascular effects can be reversed; and the condition, cured [14]. Young patients with catecholamine- induced cardiac failure refractory to medical therapy are ideal candidates for short-term ECMO support as the underlying cause is imminently reversible.

Fig. 1 Computed tomographic scan showing a 7-cm diameter hypervascular lesion of the left adrenal gland.

retained to explain catecholamine-induced cardiotoxicity: first, the coronary vasoconstriction due to the hyperstimula- tion of ?-adrenergic receptors [11]; second, the direct toxicity expressed by oxidative products of the catechol- amines [12]; and third, the hypocalcemia induced by the sequestration of the calcium in the sarcoplasma of myocar- diocytes [13]. delayed treatment over the first 24 hours of symptoms in cases of cardiac failure with pulmonary edema carries a poor prognosis [9].

Extracorporeal membrane oxygenation is ideally suited to the treatment of Reversible causes of cardiopulmonary failure, and younger patients have generally superior outcomes [17]. Although pheochromocytoma-induced car- diac failure refractory to medical management is an excellent indication for ECMO, only 3 cases have been reported in the literature with ECMO as the sole cardiovascular support modality [10].

The diagnosis of pheochromocytoma is easily made with either urinary or plasma metanephrines. Once diagnosed, accurate localization is critical to surgical planning as 10% of pheochromocytomas can be ectopic and another 10% can be multiple. Computed tomography and magnetic resonance imaging have a sensitivity of 77% to 98% and 90% to 100%, respectively, and a specificity of 29% to 92% and 50% to 90%, respectively [15]. Scintigraphy with the functional ligand (123)I-MIBG has a sensitivity of 83% to 100% and a specificity of 95% to 100% and is used to localize biochemically proven tumors to complement conventional Imaging techniques [16].

There is no consensus about the timing of pheochromo- cytoma resection after a cardiac failure as initial presentation, but results of surgery within 2 months of the episode are reported to be excellent [18]. After the cardiovascular recovery, the use of appropriate preOperative treatment to block the effects of secreted catecholamines is an important step to prepare the surgery as well as the accurate preoperative imaging and planning.

Ghassam Sojod MD Michele Diana MD James Wall MD

Jacopo D’Agostino MD Didier Mutter MD, PhD Jacques Marescaux MD

Department of General, Digestive and Endocrine Surgery IRCAD/EITS, University Hospital, Strasbourg 67091, France E-mail address: [email protected]

doi:10.1016/j.ajem.2011.05.006

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