Pheochromocytoma-induced cardiogenic shock: A multicentre analysis of clinical profiles, management and outcomes

Highlights

• Pheochromocytoma-induced cardiogenic shock presents a fairly good mid-term prognosis with rapid improvement of left ventricle ejection fraction.

• It should be suspected in case of Takotsubo-like myocardial injury progressing to cardiogenic shock or severe cyclic blood pressure fluctuation and rapid hemodynamic deterioration.

• The main challenge is to stabilize the patient, with medical therapy or with temporary-mechanical circulatory support in most severe cases.

Abstract

Objective

There is still uncertainty about the management of patients with pheochromocytoma-induced cardiogenic shock (PICS). This study aims to investigate the clinical presentation, management, and outcome of patients with PICS.

Methods

We collected, retrospectively, the data of 18 patients without previously known pheochromocytoma admitted to 8 European hospitals with a diagnosis of PICS.

Results

Among the 18 patients with a median age of 50 years (Q1-Q3: 40–61), 50% were men. The main clinical features at presentation were pulmonary congestion (83%) and cyclic fluctuation of hypertension peaks and hypotension (72%). Echocardiography showed a median left ventricular ejection fraction (LVEF) of 25% (Q1-Q3: 15–33.5) with an atypical- Takotsubo (TTS) pattern in 50%. Inotropes/vasopressors were started in all patients and temporary mechanical circulatory support (t-MCS) was required in 11 (61%) patients. All patients underwent surgical removal of the pheochromocytoma; 4 patients (22%) were operated on while under t-MCS. The median LVEF was estimated at 55% at discharge. Only one patient required heart transplantation (5.5%), and all patients were alive at a median follow-up of 679 days.

Conclusions

PICS should be suspected in case of a CS with severe cyclic blood pressure fluctuation and rapid hemodynamic deterioration, associated with increased inflammatory markers or in case of TTS progressing to CS, particularly if an atypical TTS echocardiographic pattern is revealed.

T-MCS should be considered in the most severe cases. The main challenge is to stabilize the patient, with medical therapy or with t-MCS, since it remains a reversible cause of CS with a low mortality rate.

Introduction

Pheochromocytoma is a rare catecholamine-secreting neuroendocrine tumor arising from chromaffin cells in the adrenal medulla with an age-standardized incidence rate of 0.46 per 100,000 person-years [1]. Due to its highly variable clinical picture and location in the body it may be confused with a number of other diseases, that's why it is often referred to as the “great imitator” [2]. The diagnosis can be established through the presentation of classic symptoms (persistent or paroxysmal hypertension, palpitations, headaches, diaphoresis, tremors, and/or anxiety), the detection of incidentaloma on routine imaging workup, like point-of-care ultrasound (POCUS) or abdominal computed tomography (CT) in the emergency department, routine biochemical screening, or a pheochromocytoma crisis [ [3], [4], [5]]. Cardiogenic shock (CS), as an expression of a pheochromocytoma crisis, can occur in about 2% of patients [6]. Chronic and acute catecholamine secretion can lead to structural myocardial damage which is the leading cause of morbidity and mortality in this population [7]. Pheochromocytoma crisis can occur spontaneously, because of tumor hemorrhage and/or necrosis leading to an abrupt release of catecholamine into the bloodstream, or can be precipitated by trauma or specific drugs (for instance beta-adrenergic blockers, tricyclic antidepressants, corticosteroids, metoclopramide) [8].

Patients with no history of pheochromocytoma presenting with CS and evidence of severe left ventricular (LV) systolic dysfunction may pose a significant challenge with regards to the diagnosis of pheochromocytoma-induced CS (PICS) [9].

There is still uncertainty over the management of PICS [10]. Literature is scarce and is only limited to case reports, data-limited retrospective studies and literature reviews [[11], [12], [13], [14]]. Our work aims to bring new insights into the diagnosis of PICS with regard to clinical presentations, hemodynamic profiles, echocardiographic findings, management, and outcome. We seek at raising awareness around this pathology while suggesting features that should prompt a search for pheochromocytoma in the presence of CS [8,9].