pheochromocytoma is a rare disorder and functioning tumor composed of chromaffin cell that secrete catecholamines , affecting one in a million people per year . eighty - five percent are found in the medullae of the adrenal glands , but they may also occur in the extra - adrenal , paraganglia . patients with a pheochromocytoma ' crisis ' have high mortality , mostly from a cardiovascular origin , in spite of aggressive therapy . we present an unusual case of a severe acute catecholamine cardiomyopathy presenting st segment elevation with cardiogenic shock after hemorrhage into a left adrenal tumor . a 43-yr - old man presented with acute severe chest pain , shortness of breath , and dyspnea . three year before , he had an episodic hypertensive crisis with chest discomfort and was diagnosed with pheochromocytoma . he had already severe left ventricular ( lv ) dysfunction ( ejection fraction 27% , end - diastolic / end - systolic dimension 59/48 mm ) due to suspicious catecholamine - induced cardiomyopathy . he was recommended to receive operation for the tumor but refused appropriate treatment without follow - up . he did not smoke and had no other risk factors for coronary artery disease . physical examination revealed an ill , sweaty patient , with a blood pressure of 194/115 mmhg and a heart rate of 117 bpm . initial cardiopulmonary examination revealed elevated jugular venous engorgement , and chest radiography showed signs of pulmonary edema . echocardiography ( ecg ) showed sinus tachycardia , rate of 110 bpm , and significant st depressions in ii , iii , avf , v4 - 6 ( fig . several minutes later , suddenly , he developed loss of consciousness with supraventricular tachycardia on repeated ecg , coinciding with extreme hypotension ( 71/undeterminable mmhg ) . blood pressure did not restored after cardioversion with aggressive intravenous fluid therapy and ecg turned into st elevation with tachycardia in the precordial leads ( fig . cardiac enzyme showed myocardial damage ; creatine kinase ( ck ) 667 iu / l ( normal < 250 ) , ck - mb 34.6 ng / ml ( normal < 5 ) , and myoglobin 1,462 ng / ml ( normal < 110 ) ( fig . 2 ) . echocardiography showed global hypokinesia , especially more hypokinetic in anterior and antero - septal wall in left ventricle ( lv ) . the left ventricular ejection fraction was markedly decreased less than 35% with increased wall thickness ( interventricular septal wall / posterior wall thickness 16/15 mm ) . under the tentative diagnosis of acute myocandial infarction ( ami ) , 3 ) and an intra - aortic balloon pump ( iabp ) was immediately inserted for hemodynamic support . he was admitted to the coronary care unit ( ccu ) , and mechanical ventilation and inotropic agents were started ( 20 g / kg / min dobutamine and 20 g / kg / min dopamine ) . the urinary catecholamine levels were : norepinephrine 3,962 g / day ( normal 30 - 120 ) , epinephrine 3,044 g / day ( normal 2 - 14 ) , and vanilmandelic acid ( vma ) 88.2 mg / day ( normal < 8) . we considered the patient 's presentation as acute decompensation on chronic catecholamine - induced cardiomyopathy . we decided to support the patient with extracorporeal membrane oxygenation ( ecmo ) because hypotension continued despite of fluid therapy , iv inotropics , and iabp . femoral cannulation was done and assess was achieved with a percutaneous , guidewire - based technique . during ecmo , we monitored mean arterial pressure , urine output , blood lactate concentration , and central or venous mixed venous oxygen saturation in order to maintain adequate blood flow and oxygen saturation . to maximized oxygen - carrying capacity , packed red blood cells ( rbcs ) were transfused to achieve a hematocrit above 35% and mean arterial blood pressure was maintained between 60 and 70 mm hg . heparin was administrated continuously to maintain activated clotting time between 150 and 200 sec to prevent blood clotting of the ecmo circuit . on day 3 , all inotropic support could begin to be progressively weaned , with a normal blood pressure and cardiac output . ecmo continued for 3 days maintaining cardiac output ( co ) more than 3.0 l / min . successful weaning was performed according to follow - up echocardiographic data suggestive of improve lv contractility . a repeated echocardiographic examination performed by the same physician 5 days after admission to ccu revealed a vigorous ventricular contraction with normal lv systolic function ( ef 66% , end - diastolic / end - systolic dimension 49/35 mm ) . furthermore , laboratory findings and ecg abnormality had completely resolved . abdominal computerized tomography ( ct ) confirmed the presence of a left suprarenal mass of 4 cm , with a central hypodense region suggestive of necrosis and it was no interval change compared with previous ct ( 3 yr before ) ( fig . 5 ) . after 8 weeks of therapy with alpha and beta blockers ( doxazocin 4 mg bid and carvediol 12.5 mg qd , respectively ) , the patient was sent to elective laparoscopic left adrenalectomy where an about 4.23.53 cm tumor was removed , which was proven to be pheochromocytoma ( fig . a follow - up ecg obtained 3 months after the first one showed complete resolution of the st segment and t inversion ( fig . pheochromocytomas are rare neoplasms of chromaffin tissue derived from the embryonic neural crest and produce their distant effects by secretion of excessive amount of catecholamines ( 1 ) . the classic , clinical presentations of pheochromocytoma , such as headache , sweating attacks , tachycardia , and hypertension are highly sensitive and specific , but not always present ( 2 ) . in addition to the classic symptoms , pheochromocytomas have been rarely presented with acute myocarditis , ami , cardiomyopathy , or hemodynamic collapse ( 3 ) . acute form is associated with an excessive catecholamine surge and prone to present as acute cardiac failure , including acute pulmonary edema or cardiogenic shock ( 3 ) . especially with heart failure , patients may have very gloomy prognosis because of extensive myocardial damage ( 4 ) . chronic forms are manifested as dilated cardiomyopathy or hypertrophic cardiomyopathy with or without outflow tract obstruction , leading to congestive heart failure ( 3 , 5 ) . when it occurs , ecg can show st elevation or , more commonly , non - st elevation mi . it is not unusual for the patient with pheochromocytoma - related mi to present with acute , severe symptoms leading rapidly to death and diagnosis made at autopsy . most of patients in these cases had no significant coronary atherosclerosis in contrast to classic mi ( 7 ) . high levels of catecholamine released by the tumor can increase myocardium metabolism and cause coronary vasospasm resulting in angina , mi , and cardiomyopathy , which could be irreversible if left untreated ( 8 - 10 ) . in patients with pheochromocytoma , however , ventricular tachycardia , including " torsades de pointes " , or rarely ventricular fibrillation has also been described ( 12 ) . the ischemia - appearing ecg changes make the diagnosis of mi confounding ( 13 ) . ecg abnormalities like this may result from stimulation of the myocardium by excessively high catecholamines levels , are transient , and usually turn to normal after the removal of the tumor , which suggests a toxic myocarditis rather than true transmural infarction ( 14 , 15 ) . ecg in this patient showed st depression and st elevation , mimicking ami , so that it was difficult to differentiate between obstructive and nonobstructive coronary lesion . we found normal coronary arteries on angiogram and increased lv wall thickness ( interventricular septal wall / posterior wall , 16/15 mm ) on echocardiography , which suggested catecholamine - induced cardiomyopathy associated with pheochromocytoma . first , acute spontaneous hemorrhage into the tumor led to a massive release of catecholamines , which was proven on pathology . patients with a pheochromocytoma crisis caused by spontaneous tumor necrosis or hemorrhage are exceedingly rare but their presentation can be very severe ( 4 ) . second , the clinical course of acute pulmonary edema and subsequent cardiogenic shock can be explained by the severe diffuse myocardial impairment present . third reason for severe hypotension might be the severely reduced blood volume . from the recovery of cardiac function from severe it was the indirect evidences that the heart had exposed to long - standing high blood pressure . hemorrhagic necrosis of the tumor led to a massive release of catecholamines , which resulted in the patient 's initial hypertension and subsequent cardiogenic shock ( 16 ) . there is no reliable , controlled data regarding the treatment of adrenergic shock so far . , early detection and supportive care should be combined with treatment of underlying disease to prevent the development of irreversible multiorgan failure . preoperative preparation with volume replacement and alpha - adrenergic blockades ( usually prazocin or phenoxylbenzamine ) have accounted for the most significant reductions in perioperative mortality ( 17 ) . several preliminary clinical reports have suggested that extracorporeal circulatory resuscitation can aid in the resuscitation of patients with cardiopulmonary arrest regardless of causes ( 18 , 19 ) . we found only one report of successful combination of iabp and ecmo in management of acute myocarditis ( 20 ) . at present , iabp , ecmo , and ventricular assist device ( vad ) are available as extracorporeal support systems . iabp is convenient to be applied but can only provide limited additional co , which usually is not adequate for all critical situations . vad implantation is expensive and time - consuming , and not always available for some patients group . besides , it also requires cardiopulmonary bypass for vad setup and removal ( 19 ) . in case of iabp failure supporting the patient it can be started within 30 min at bedside and can take over the functions of both ventricles and lungs . furthermore , it can support the failing heart for long term as needed and is easy to be removed at bedside in the intensive care unit . it also allows several hours or days to try weaning ecmo if the bridging tube is added to the circuit . it is reasonable to choose ecmo to treat cardiomyopathy with shock that can usually improve within 1 or 2 weeks ( 19 ) . catastrophic shock can be completely reversible without leading to further damage after its recovery from the acute phase , if ecmo can be provided in time and long enough for the recovery of myocardium . we conclude that a catecholamine - induced cardiomyopathy in patients with a pheochromocytoma seems to be completely reversible and potentially cured by timely adequate treatment . thus , if acute cardiogenic shock in patients with a pheochromocytoma is unresponsive despite intensive medical treatment , immediate mechanical support using iabp and pcps ( percutaneous cardiopulmonary support ) should be considered as a very useful therapeutic choice alongside the classic therapy of - and -adrenergic antagonist . laparoscopic adrenalectomy has become the procedure of choice for most patients , with open adrenalectomy generally reserved for large tumors or those most likely to be malignant ( 21 ) .
pheochromocytoma is a rare disorder and functioning tumor composed of chromaffin cells that secrete catecholamines . 
 patients with a pheochromocytoma ' crisis ' have a high mortality in spite of aggressive therapy . 
 we present a case with a severe acute catecholamine cardiomyopathy presenting st segment elevation with cardiogenic shock after hemorrhage into a left suprarenal tumor . 
 intra - aortic balloon pump ( iabp ) support , combined with inotropic therapy , was performed . however , the patient deteriorated rapidly and was unresponsive to a full dose of inotropics and iabp . 
 we decided to apply extracorporeal membrane oxygenation ( ecmo ) device for the patient . 
 his clinical state began to improve 3 days after ecmo . 
 after achieving hemodynamic stabilization , he underwent successful laparoscopic left adrenalectomy . 
 he needed no further cardiac medication after discharge .