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

Background: Pheochromocytoma and paraganglioma are catecholamine-secreting tumors, rarely presenting with pheochromocytoma multisystem crisis (PMC), a life-threatening endocrine emergency. The severity of the condition includes a refractory cardiogenic shock and may therefore require the use of temporary mechanical circulatory support. The aim of this review is to describe the incidence of pheochromocytoma and paraganglioma crises associated with refractory cardiogenic shock, the physiopathological impact of this condition on the myocardial function, the role of temporary mechanical circulatory support (tMCS) in its management, and the outcomes of this specific population. Methods: For the purpose of this narrative review, a literature search of PubMed was conducted as of 16 November 2024. Medical Subject Headings (MeSH) terms used included extracorporeal circulation", "Impella", "pheochromocytoma", "paraganglioma", and "cardiogenic shock", combined with Boolean "OR" and "AND". Data from case series, retrospective studies, and systematic reviews were considered. Seven studies reporting on 45 patients who developed PMC with cardiogenic shock requiring tMCS were included. Patients were young, with a median age of 43 years (range 25-65) at presentation. Most cases presented with severe hemodynamic instability, blood pressure lability, and rapid progression to severe left ventricular dysfunction. Veno-arterial extracorporeal membrane oxygenation (VA-ECMO) was the most common tMCS used to stabilize patients, initiate specific pheochromocytoma treatments, and, in some cases, provide circulatory support during emergent surgery. The median duration of VA-ECMO support was 4 days (range 1-7) and the reported mean in-hospital survival rate was 93.5%. Following VA-ECMO weaning, survivors showed full recovery of the left ventricular ejection fraction (LVEF). Conclusions: The cardiac dysfunction observed in PMC-associated cardiogenic shock may be severe and life-threatening but appears reversible. tMCS should therefore be considered in eligible cases, as a bridge to recovery, treatment, or surgery. The reported survival rates are impressively high, suggesting possibly a substantial risk of publication bias.