170 Pheochromocytoma Crisis Presenting as Fulminant Cardiopulmonary Failure: A Case Report Chun-Wen Chiu 1, Cheng-Hsiung Chen 2 Fulminant cardiopulmonary failure in a patient with pheochromocytoma is a challenge in the emergency department and intensive care unit. We present a case in a 39-year-old woman with previously undiagnosed pheochromocytoma, who developed acute pulmonary edema after morning exercise. Her condition rapidly deteriorated into cardiac arrest. Cardiopulmonary resuscitation with extracorporeal membrane oxygenation successfully rescued the patient when an intra-aortic balloon pump failed to provide cardiopulmonary support for the pheochromocytoma crisis. Key words: pheochromocytoma crisis, fulminant cardiopulmonary failure, cardiopulmonary resuscitation (CPR), extracorporeal membrane oxygenation (ECMO), cardiac arrest Introduction Pheochromocytoma is a rare neuroendocrine tumor with variable clinical manifestations. T h e t y p i c a l p r e s e n t a t i o n s a r e h e a d a c h e, diaphoresis, palpitations, and hypertension. Critical cardiopulmonary complications from pheochromocytoma occur because of excess s e c r e t i o n o f c a t e c h o l a m i n e s. F u l m i n a n t cardiopulmonary failure with cardiac arrest c a u s e d b y p h e o c h r o m o c y t o m a i s a r a r e presentation with a low survival rate. A previous report showed that cardiopulmonary resuscitation (CPR) with extracorporeal membrane oxygenation (ECMO) can provide adequate cardiopulmonary support and improve the survival rate. 1 We present a patient in pheochromocytoma crisis with cardiac arrest who was successfully rescued with ECMO. Case Report A 39-year-old woman without a history of hypertension or heart disease presented to the emergency department with palpitations during morning exercise. On arrival, she was ambulant and had a temperature of 36.8, heart rate of 112 beats/min, respiratory rate of 22 breaths/min, and blood pressure of 120/80 mmhg and her Glasgow Coma Score was E4M6V5. Her electrocardiogram (EKG) showed sinus tachycardia. Chest radiography showed no infiltration or cardiomegaly. The cardiac enzyme values were as follows: creatine kinase 74 u/l; MB fraction 1.7 ng/ml; and troponin-i 0.08 ng/ml. The patient suddenly developed tachypnea with diaphoresis one hour later. Her heart rate increased to 156 beats/min, her respiratory rate increased to 32 breaths/min, and her blood pressure decreased to 82/42 mmhg. A subsequent Received: June 2, 2011 Accepted for publication: September 19, 2011 From the 1 Department of Emergency Medicine, 2 Division of Chest Medicine, Division of Critical Care Medicine Changhua Christian Hospital Address reprint requests and correspondence: Dr. Cheng-Hsiung Chen Division of Chest Medicine, Division of Critical Care Medicine 135 Nanxiao Street, Changhua City, Changhua County 500, Taiwan (R.O.C.) Tel: (04)7238595 ext 7101 Fax: (04)7228289 E-mail: 77112@cch.org.tw
Pheochromocytoma crisis 171 chest radiograph showed bilateral increased lung markings and pulmonary edema. The EKG revealed sinus tachycardia. Arterial blood gas analysis revealed a ph of 7.50, PaCO 2 of 44.7 mmhg, PaO 2 of 45.8 mmhg, bicarbonate of 22.4 mmol/l, base excess of -3.6 mmol/l, and O 2 saturation of 76.8%. The severe hypoxia required mechanical ventilation. Frothy pink sputum was noted, and acute pulmonary edema developed. The patient s blood pressure fluctuated between 198/100 mmhg and 82/46 mmhg. A pheochromocytoma crisis was suspected. Computed tomography (CT) showed a left suprarenal 4.0 4.2 cm tumor. (Fig. 1) A diagnosis of pheochromocytoma was made, and the patient was transferred to the intensive care unit for further treatment of her fulminant cardiopulmonary failure. An intra-aortic balloon pump (IABP) was inserted, and the patient required inotropic and vasopressor support for fulminant cardiopulmonary failure. The follow-up cardiac enzymes were elevated as follows: creatine kinase 1148 u/l; MB fraction 106.1 ng/ml; and troponin-i 34.19 ng/ml. Pulmonary artery catheterization demonstrated a cardiac index of 1.46 L/min/m 2, a systemic vascular resistance of 2712 dynes/sec/cm 5, a pulmonary artery wedge pressure of 14 mmhg, a central venous pressure of 11 mmhg, and a pulmonary artery pressure of 24/17 mmhg. She had a sudden onset of pulseless ventricular tachycardia, followed by cardiac arrest. Immediate defibrillation and standard CPR were performed. The patient received advanced cardiopulmonary life support which involved minimal interruption of chest compressions, ventilation, intravenous a d m i n i s t r a t i o n o f e p i n e p h r i n e 1 m g 7, monophasic shock of 360 J 3, and intravenous administration of amiodarone 300 mg 1 and 150 mg 2. CPR was done for approximately 24 minutes, followed by a return of spontaneous circulation. Her unstable hemodynamic status was refractory to the IABP, as well as inotropic agents and vasopressors (dopamine 20 µg/kg/min and dobutamine 5 µg/kg/min). Echocardiography demonstrated severe, diffuse hypokinesia of the left ventricle with a left ventricular ejection fraction (LVEF) of 10%. The patient required more aggressive mechanical cardiopulmonary life support. Veno-arterial extracorporeal membrane oxygenation (ECMO) cannulas were inserted in her right femoral vessels. A size 19 Fr cannula was implanted on the venous side, and a size 17 Fr catheter on the arterial side. Heparin was Fig. 1 Abdominal computed tomography showing a 4.0 4.2 cm mass in the left adrenal gland
172 administered and titrated to maintain an activated clotting time around 200 sec to prevent blood clotting in the ECMO circuit. The patient s hemodynamic status became stable 14 hours later, and the hemodynamic calculations showed a pulmonary artery pressure of 21/17 mmhg, a pulmonary wedge pressure of 8 mmhg, and a central venous pressure of 8 mmhg. Serial echocardiography revealed a gradual recovery of the heart contractility (LVEF increased from 10 to 65% in five days), and the patient s clinical condition recovered. The ECMO and IABP were successfully weaned off on day five, and the patient was successfully extubated on day six. The 24-hour urine catecholamine levels were elevated. (Table 1) Under high suspicion of pheochromocytoma, the patient was started on oral phenoxybenzamine 20 mg twice daily and subsequent bisoprolol 2.5 mg twice daily. After 40 days of treatment with α- and β-blockers, the patient was stable enough to tolerate surgical intervention. She underwent a laparoscopic left adrenalectomy with tumor excision. The pathology confirmed the diagnosis of pheochromocytoma. She was discharged six days postoperatively and was followed up in the outpatient department. Discussion The early diagnosis of pheochromocytoma and treatment of its life-threatening cardiopulmonary Table 1 24-hour urine catecholamine levels Levels Normal Values Noradrenaline (μg/day) 292 <97 Adrenaline (μg/day) 877 <27 Dopamine (μg/day) 49 <500 VMA* (mg/day) 73.8 1.0-7.5 VMA: vanillyl mandelic acid complications is a challenge in the emergency department and intensive care unit. In our case, we had a high suspicion of pheochromocytoma because of the flutuations in the blood pressure and palpitations. Pheochromocytoma was confirmed by computed tomography and 24-hour urine catecholamine levels. This case demonstrates fulminant cardiopulmonary failure secondary to pheochromocytoma with pulseless ventricular tachycardia and cardiac arrest. The pathogenesis of catecholamine-i n d u c e d cardiomyopathy has been reported (2). Excess catecholamines may cause severe, transient, but reversible cardiogenic shock (3). The unusual cardiovascular complications of pheochromocytoma include sudden death, arrhythmias, acute myocardial infarction and acute pulmonary edema. Acute pulmonary edema caused by pheochromocytoma is a rare presentation with a high motality rate. In one report, five of six patients died of pulmonary edema within 24 hours of the onset of symptoms (2). These critical patients need mechanical life support. Thiagarajan et al. reported that the use of ECMO-CPR was associated with survival in 27% of adults with cardiac arrest facing imminent mortality (1). Initially, our patient was implanted with an intra-aortic balloon pump, and dobutamine and dopamine infusions were administered for fulminant cardiopulmonary failure. However, her condition continued to deteriorate, and sudden onset of pulseless ventricular tachycardia developed with cardiac arrest. Obviously, the IABP, inotropic agents and vasopressors were inadequate. Cardiopulmonary resuscitation with extracorporeal membrane oxygenation and defibrillation with amiodarone was administered, and the hemodynamic status stabilized. We had to decide between emergency resection and pretreatment with an α-adrenergic blockade.
Pheochromocytoma crisis 173 Bos et al. reported that emergency surgery for pheochromocytoma does not have to be structurally avoided and may be considered under life-threatening circumstances (4). May et al. reported that a patient who had emergency surgery for pheochromocytoma still had severe complications from massive catecholamine excess, including shock, cardiomyopathy, and adult respiratory distress syndrome (5). In this case, we decided to pretreat with α blockers. Once the diagnosis of pheochromocytoma was made, α blockers were administered, and when adequate α blockade was achieved, β blockers could be initiated for preoperative management. In conclusion, early diagnosis and treatment were necessary for this patient. Fulminant cardiopulmonary failure without response to conventional treatment may cause a high mortality if there is no aggressive mechanical cardiopulmonary life support system. ECMO successfully provided cardiopulmonary life support until recovery from a critical status secondary to a pheochromocytoma crisis. This was successful CPR with ECMO in a patient with a pheochromocytoma crisis. References 1. Thiagarajan RR, Brogan TV, Scheurer MA, Laussen PC, Rycus PT, Bratton SL. Extracorporeal membrane oxygenation to support cardiopulmonary resuscitation in adults. Ann Thorac Surg 2009;87:778-85. 2. Sardesai SH, Mourant AJ, Sivathandon Y, Farrow R, Gibbons DO. Phaeochromocytoma and catecholamine induced cardiomyopathy presenting as heart failure. Br Heart J 1990;63:234-7. 3. Wittstein IS, Thiemann DR, Lima JA, et al. Neurohumoral features of myocardial stunning due to sudden emotional stress. N Engl J Med 2005;352:539-48. 4. Bos JC, Toorians AW, van Mourik JC, van Schijndel RJ. Emergency resection of an extraadrenal phaeochromocytoma: wrong or right? A case report and a review of literature. Neth J Med 2003;61:258-65. 5. May EE, Beal AL, Beilman GJ. Traumatic hemorrhage of occult pheochromocytoma: a case report and review of the literature. Am Surg 2000;66:720-4.
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