Management of an acute catecholamine-induced cardiomyopathy and circulatory collapse: a multidisciplinary approach

A phaeochromocytoma (PC) is a rare, catecholamine-secreting neuroendocrine tumour arising from the adrenal medulla. Presenting symptoms of this rare tumour are highly variable but life-threatening multiorgan dysfunction can occur secondary to catecholamine-induced hypertension or hypotension and subsequent cardiovascular collapse. High levels of circulating catecholamines can induce an acute stress cardiomyopathy, also known as Takotsubo cardiomyopathy. Recent studies have focused on early diagnosis and estimation of the prevalence of acute stress cardiomyopathy in patients with PC, but very little is reported about management of these complex cases. Here, we report the case of a 38-year-old lady who presented with an acute Takotsubo or stress cardiomyopathy and catecholamine crisis, caused by an occult left-sided 5 cm PC. The initial presenting crisis manifested with symptoms of severe headache and abdominal pain, triggered by a respiratory tract infection. On admission to hospital, the patient rapidly deteriorated, developing respiratory failure, cardiogenic shock and subsequent cardiovascular collapse due to further exacerbation of the catecholamine crisis caused by a combination of opiates and intravenous corticosteroid. An echocardiogram revealed left ventricular apical hypokinesia and ballooning, with an estimated left ventricular ejection fraction of 10–15%. Herein, we outline the early stabilisation period, preoperative optimisation and intraoperative management, providing anecdotal guidance for the management of this rare life-threatening complication of PC. Learning points: A diagnosis of phaeochromocytoma should be considered in patients presenting with acute cardiomyopathy or cardiogenic shock without a clear ischaemic or valvular aetiology. Catecholamine crisis is a life-threatening medical emergency that requires cross-disciplinary expertise and management to ensure the best clinical outcome. After initial resuscitation, treatment of acute catecholamine-induced stress cardiomyopathy requires careful introduction of alpha-blockade followed by beta-blockade if necessary to manage β-receptor-mediated tachycardia. Prolonged α-adrenergic receptor stimulation by high levels of circulating catecholamines precipitates arterial vasoconstriction and intravascular volume contraction, which can further exacerbate hypotension. Invasive pressure monitoring can aid management of intravascular volume in these complex patients. Background Phaeochromocytoma (PC) typically presents with symptoms of catecholamine excess including headache, palpitations, paroxysmal hypertension, anxiety and excessive sweating. However, the severity of these symptoms is variable, owing, in part, to the heterogenous genetic background of these tumours, and increasingly PC tumours are diagnosed incidentally following cross-sectional imaging (1). A catecholamine crisis is defined as acute and severe haemodynamic instability and collapse (2) and is a rare, but dreaded, complication of PC. A recent multicentre retrospective review by Resiter et al. reported that 11% of the patients with PC presented with a catecholamine crisis (3) and a recent review reported a pooled mortality rate of 15% (4). Acute stress cardiomyopathy is one of the most commonly reported complications of a catecholamine crisis (3, 4, 5) and is defined as an acute-reversible regional ventricular wall motion abnormality with a characteristic circular pattern, incongruent with the coronary artery supply distribution (6, 7). Convergent evidence over the past fifteen years suggests that this distinct form of cardiomyopathy is caused by hyperstimulation of the cardiac sympathetic nerve terminals, due to physiological production of catecholamines during severe stress or autonomous pathological secretion from a PC (6). Chest pain is the most common presenting feature of acute stress cardiomyopathy and patients with an underlying PC may report symptoms related to excess catecholamine secretion (8). Acute stress cardiomyopathy due to PC typically presents at a younger age, is more commonly associated with an apical regional wall motion abnormality (8) and is associated with a lower mean left ventricular ejection fraction compared with acute stress cardiomyopathy due to other causes (8), as evident in this case. Early cautious treatment with alpha-blockade remains the mainstay of treatment for a catecholamine crisis including acute stress cardiomyopathy (4), with the aim of reducing peripheral vasoconstriction and preventing arrhythmia provocation. Indeed, a review of the published literature has revealed a correlation between alpha-blockade administration and improved survival in patients with catecholamine crises (4). However, as refractory hypotension is a common feature of a catecholamine crisis and acute stress cardiomyopathy, early fluid resuscitation, invasive pressure monitoring and cross-speciality input from cardiology, endocrinology and intensive care are crucial to enable sustained treatment with alpha-blockade therapy.