Cardiogenic shock in phaeochromocytoma multisystem crisis: a case report

Abstract

Background: Phaeochromocytoma multisystem crisis (PMC) is characterized by labile blood pressures (extremes of hypo- and/or hypertension) and multiorgan failure as a result of catecholamine excess. Initial stabilization requires pharmacological and/or mechanical circulatory support, followed by the institution of antihypertensives to correct the underlying pathophysiology.

Case summary: A previously well 40-year-old male developed a sudden onset of breathlessness. On presentation, he was in shock with multiorgan failure. He required intubation, mechanical ventilation, dual inotropic support, and renal replacement therapy. Bedside echocardiogram showed a severely impaired left ventricular ejection fraction (LVEF) of 25%. Coronary angiography revealed normal coronary arteries. In view of raised inflammatory markers and transaminitis, a computed tomography abdomen/pelvis was performed. An incidental left adrenal mass was found. Further work-ups revealed raised plasma metanephrine and normetanephrine, 24-h urine epinephrine, and norepinephrine. A cardiac magnetic resonance (CMR) showed myocardial inflammation and reverse Takotsubo pattern of regional wall motion abnormality (RWMA). The diagnosis of cardiogenic shock and stress cardiomyopathy secondary to PMC was made. He was subsequently initiated on α- and β-blockers and goal-directed medical therapy for heart failure. A ⁶⁸Ga-DOTATATE scan showed avid tracer uptake of the left phaeochromocytoma. An interval CMR 3 weeks from presentation showed near normalization of the LVEF and RWMA. He underwent a successful laparoscopic left adrenalectomy and was antihypertensive-free since.

Discussion: The clinical suspicion for PMC as the cause of cardiogenic shock requires astute clinical judgement, while the management requires an understanding of the underlying pathophysiology that calls for multidisciplinary inputs.

Keywords: Case report; Catecholamines; Phaeochromocytoma; Phaeochromocytoma multisystem crisis.

Graphical abstract

Figure 1

(Left) Initial electrocardiogram showing sinus tachycardia and no ischaemic changes. (Right) Chest X-ray on presentation, showing pulmonary congestion.

Figure 2

Cyclical arterial blood pressure spikes, at times reaching arterial blood pressure >130/80 mmHg despite severely impaired systolic function and poor cardiac output.

Figure 3

Cardiac magnetic resonance imaging. (A and B) Systolic frames of cine images showing akinesia of the mid-segments, i.e. mid-anterior, septal, lateral, and inferior (chevron arrows), and normokinesia of the basal and apical segments. (C) Mid-wall Late Gadolinium enhancement at the mid-anterior, anteroseptal, anterolateral, and inferior (chevron arrows) segments. (D and E) Parametric mapping of the corresponding slice, showing increased global native T1 (D) and T2 (E) relaxation times, implying the presence of oedema.

Figure 4

68Ga-68 DOTATATE scan showing avid tracer uptake by the left phaeochromocytoma (indicated by the arrows).

Figure 5

(A) Gross surgical specimen. Phaeochromocytoma (round, fleshy mass, asterisk) with adjacent normal adrenal structures and surrounding fat. (B) Phaeochromocytoma (well-circumscribed, cream-coloured mass, asterisk) arising from the medulla. The normal adrenal cortex appears dark yellow and the normal medulla appears brown. (C) Low power H&E specimen. Nodular mass (purple) involving the medulla of the adrenal gland. A small amount of normal adrenal gland is present (bright pink). (D) High-power H&E specimen. The tumour has a Zellballen (German for ball of cells) pattern, large and ovoid nuclei with nucleoli, and abundant granular cytoplasm. (E) Tumour expressing neuroendocrine markers, i.e. synaptophysin and chromogranin positive. Note that the normal adrenal cortex in the right upper corner is stained negative.