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

Article Article scientifique Revue de la littérature Pheochromocytoma Multisystem Crisis Requiring Temporary Mechanical Circulatory Support : A Narrative Review eng 2025-03-12 author Giraud Raphaël 0000-0001-5956-8643 639599 Département d'anesthésiologie, pharmacologie et soins intensifs (APSIC) author Glauser Amandine 648962 author Looyens Carole Marie 417580 author Della Badia Chiara 892965 author Jolou Jalal 649382 author Cikirikcioglu Mustafa 0000-0003-1143-347X 135609 Département de chirurgie (CHIRU) author Gariani Karim 542849 Département de médecine (DEMED) author Bendjelid Karim 0000-0002-7921-7207 527948 Département d'anesthésiologie, pharmacologie et soins intensifs (APSIC) author Assouline Benjamin 595385 1907 10.3390/jcm14061907 40142715 2077-0383 https://www.mdpi.com/2077-0383/14/6/1907 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. VA-ECMO Cardiogenic shock Pheochromocytoma multisystem crisis T-MCS Dewey 616 Dewey 617 63 Département d'anesthésiologie, pharmacologie, soins intensifs et urgences 229 57 Département de chirurgie 222 56 Département de médecine 221 false 570 Groupe de recherche en hémodynamique 913 Journal of clinical medicine 14 6 e7a1124d-93eb-414b-a2f4-2bd1feac5564 Article (Published version) Public Article_scientifique_from_crossref.pdf 599740 application/pdf CC BY-4.0 2294212cd250c25cf8539c68a02fb8c5063a285e911c0d9b141e6b52d5d69d0d openAccess Faculté de médecine / Section de médecine clinique / Département d'anesthésiologie, pharmacologie, soins intensifs et urgences Faculté de médecine / Section de médecine clinique / Département de chirurgie Faculté de médecine / Section de médecine clinique / Département de médecine

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Pheochromocytoma Multisystem Crisis Requiring Temporary Mechanical Circulatory Support : A Narrative Review	Journal of clinical medicine			2025	77	462

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Glauser, Amandine