Phaeochromocytoma can cause a myriad of cardiovascular 1issues including cardiogenic shock which is associated with high mortality. Early diagnosis and prompt treatment are critical but often delayed because of the diagnostic challenge. We present two cases of adrenergic cardiomyopathy associated with cardiogenic shock as the initial presentation of phaeochromocytoma.
Case 1:A 39-year-old female with a history of hypertension, anxiety and palpitations presented with severe hypertension, headache and rapid deterioration into cardiogenic shock. An echocardiogram demonstrated severely impaired left ventricular function with basal akinesis. Inotrope therapy worsened oscillatory periods of tachycardia, hyper- and hypotension. Extracorporeal membrane oxygenation (ECMO) was required for circulatory support. Because of the possibility of a catecholamine associated cardiomyopathy, a bedside abdominal ultrasonography was performed and showed an 8 cm diameter left adrenal mass. Plasma metanephrines were elevated (give data). A 68-Gallium DOTATATE positron emission tomography (PET) scan showed a large left adrenal pheochromocytoma which was successfully removed after a month of preparation with phenozybenzamine.
Case 2:A 46-year-old female presented with cardiogenic shock and multi-organ failure. An echocardiogram showed regional cardiac wall motion abnormality consistent with Takotsubo’s She was haemodynamically unstable with markedly varying vasopressor requirements and fever. ECMO was commenced. Bedside ultrasonography incidentally detected a 4.3 cm diameter left adrenal mass. Raised plasma metanephrines660pmol/L (<500)and MIBG Nuclear Imaging indicated this was a avid left adrenal phaeochromocytoma. After phenoxybenzamine preparation left adrenalectomy was performed. Histopathology confirmed phaeochromocytoma .
Initially a diagnostic dilemma, in both cases the identification of an adrenal mass was delayed. Alpha- and subsequent beta-blockade therapy markedly improved left ventricular function. Excision of the tumour was safely done in a non-emergency situation. Both patients are now well, and normotensive.
In patients with severe cardiomyopathy with unstable haemodynamic status, acute catecholamine cardiomyopathyassociated with phaemochromocytoma should be considered in the differential diagnosis and adrenal imaging performed.