Endocrine Abstracts

Introduction: Catecholamine excess causes profound vasoconstriction, resulting in reduced intravascular volume. Pheochromocytoma crisis can be further complicated with acute stress (Takotsubo-like) cardiomyopathy, attributed to the toxic catecholamine effect on the myocardium, and cardiogenic shock leading to tissue ischemia.

Aim: To present a rare case of pheochromocytoma crisis, complicated with fulminant cardiogenic shock, which led to life-threatening end-organ damage.

Case report: A 34-year-old woman presented with a recent history of paroxysmal hypertension, palpitations and concomitant sweating. Echocardiogram and abdominal ultrasound had revealed a normal left ventricular ejection fraction (LVEF>55%) and a 7 cm mass in the right adrenal gland. Subsequently, she was admitted to the Endocrinology department, where hormonal workup showed elevated 24-h urine fractionated metanephrines and normetanephrines [18 266 μg/24 h (NR<800 μg/24 h) and 12 609 μg/24 h (NR<444 μg/24 h) respectively]. However, during the initial workup, the patient’s condition deteriorated dramatically over a few hours, with no obvious physical, emotional or pharmacological triggering factor, following two episodes of hypertensive peaks (BP~200/120 mmHg) with symptoms of acute chest and abdominal pain, excessive weakness, sweating, pallor and wide fluctuations of blood pressure. Urgent electrocardiogram showed ST-segment depression in V4-lead and transthoracic echocardiography revealed a reduction of LVEF to ~15% and a Takotsubo-pattern image of diffuse hypokinesia with apical ballooning. Phenoxybenzamine treatment was initiated immediately. Active haemorrhage or eruption of the adrenal tumour was excluded with emergency abdominal CT scan. The patient was transferred to the Cardiologic Intensive Care Unit, where her condition continued to deteriorate with abruptly elevated levels of cardiac enzymes and worsening tachycardia (160 bpm) non-responding to phenoxybenzamine and esmolol. Coronary artery angiography was normal. Despite attempts of cautious volume expansion the patient rapidly developed refractory cardiogenic shock, incipient multiple organ failure and hyperlactatemia (9 mmol/l) leading to intubation and initiation of central VA-ECMO support. Cardiac function was progressively restored and VA-ECMO was removed on day 12, with an LVEF>50%. Treatment with phenoxybenzamine was continued and right adrenalectomy was performed 18 days later. However, the patient’s course had been complicated further with severe central nervous system damage, rhabdomyolysis and lower limb amputation due to thromboembolic events and hypoperfusion, with subsequent fatal hospital infections after a long-term ICU hospitalization.

Conclusion: Pheochromocytoma crisis can fulminantly develop into Takotsubo-like cardiomyopathy and refractory cardiogenic shock with fatal complications. In such perplexing cases, central VA-ECMO might be one of the few life-saving measures to be considered until myocardial function recovers allowing further management and surgical intervention.