Pheochromocytoma Multisystem Crisis Requiring Temporary Mechanical Circulatory Support: A Narrative Review

Area of Science:

• Endocrinology
• Cardiology
• Critical Care Medicine

Background:

• Pheochromocytoma and paraganglioma are rare catecholamine-secreting tumors.
• Pheochromocytoma multisystem crisis (PMC) is a life-threatening endocrine emergency, often presenting with refractory cardiogenic shock.
• Severe cardiogenic shock in PMC may necessitate temporary mechanical circulatory support (tMCS).

Purpose of the Study:

• To review the incidence of pheochromocytoma and paraganglioma crises associated with refractory cardiogenic shock.
• To describe the physiopathological impact on myocardial function.
• To evaluate the role and outcomes of tMCS in managing PMC-associated cardiogenic shock.

Main Methods:

• A narrative literature review of PubMed was conducted up to November 16, 2024.
• Search terms included 'extracorporeal circulation', 'Impella', 'pheochromocytoma', 'paraganglioma', and 'cardiogenic shock'.
• Seven studies involving 45 patients with PMC and cardiogenic shock requiring tMCS were analyzed.

Main Results:

• Patients were typically young (median age 43) and presented with severe hemodynamic instability and left ventricular dysfunction.
• Veno-arterial extracorporeal membrane oxygenation (VA-ECMO) was the primary tMCS used, with a median support duration of 4 days.
• The in-hospital survival rate was 93.5%, with survivors showing full recovery of left ventricular ejection fraction (LVEF) post-weaning.

Conclusions:

• Cardiac dysfunction in PMC-associated cardiogenic shock is severe but reversible.
• tMCS is a crucial bridge to recovery, treatment, or surgery for eligible PMC patients.
• High survival rates suggest potential publication bias, warranting further investigation.