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      Two cases of cardiomyopathy associated with phaeochromocytoma successfully managed with veno-arterial extracorporeal membrane oxygenation (V-A ECMO)

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          Abstract

          Summary

          Phaeochromocytoma, a rare neuroendocrine tumour of chromaffin cell origin, is characterised by catecholamine excess. Clinical presentation ranges from asymptomatic disease to life-threatening multiorgan dysfunction. Catecholamine-induced cardiomyopathy is a dreaded complication with high lethality. While there is lack of evidence-based guidelines for use of veno-arterial extracorporeal membrane oxygenation (V-A ECMO) in the management of this condition, limited to case reports and small case series, V-A ECMO has been reported as ‘bridge to recovery’ therapy, providing circulatory support in the initial period of stabilisation prior to surgery. We report on two patients presenting with catecholamine-induced cardiomyopathy and circulatory collapse who were successfully treated with V-A ECMO for 5 and 6 days, respectively, providing initial haemodynamic support. After stabilisation and introduction of alpha-blockade, both cases had favourable outcomes, with successful laparoscopic adrenalectomies on days 62 and 83 of admission, respectively. Our case reports provide further support for the use of V-A ECMO in the treatment of such gravely ill patients.

          Learning points
          • Phaeochromocytoma should be considered in the diagnosis of patients presenting with acute cardiomyopathy.

          • Management of catecholamine-induced cardiomyopathy is complex and requires multidisciplinary specialist input.

          • Pre-operative management of phaeochromocytoma involves alpha-blockade; however, haemodynamic instability in the setting of cardiogenic shock can preclude alpha-blockade use.

          • Veno-arterial extracorporeal membrane oxygenation is a life-saving intervention which may be considered in cases of acute catecholamine-induced cardiomyopathy and cardiogenic shock in order to provide the required haemodynamic support in the initial phase of treatment, enabling the administration of traditional pharmacological agents, including alpha-blockade.

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          Most cited references11

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          Pheochromocytoma and paraganglioma: an endocrine society clinical practice guideline.

          The aim was to formulate clinical practice guidelines for pheochromocytoma and paraganglioma (PPGL).
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            Pheochromocytoma crisis is not a surgical emergency.

            Pheochromocytoma crisis is a feared and potentially lethal complication of pheochromocytoma. We sought to determine the best treatment strategy for pheochromocytoma crisis patients and hypothesized that emergency resection is not indicated. Retrospective cohort study (1993-2011); literature review (1944-2011). Tertiary referral center. There were 137 pheochromocytoma patients from our center and 97 pheochromocytoma crisis patients who underwent adrenalectomy from the literature. Medical management of pheochromocytoma crisis; adrenalectomy. Perioperative complications, conversion, and mortality. In our database, 25 patients (18%) presented with crisis. After medical stabilization and α-blockade, 15 patients were discharged and readmitted for elective surgery and 10 patients were operated on urgently during the same hospitalization. None underwent emergency surgery. Postoperatively, patients who underwent elective surgery had shorter hospital stays (1.7 vs 5.7 d, P = 0.001) and fewer postoperative complications (1 of 15 [7%] vs 5 of 10 [50%], P = 0.045) and were less often admitted to the intensive care unit (1 of 15 [7%] vs 5 of 10 [50%], P = 0.045) in comparison with urgently operated patients. There was no mortality. Review of the literature (n = 97) showed that crisis patients who underwent elective or urgent surgery vs emergency surgery had less intraoperative (13 of 31 [42%] vs 20 of 25 [80%], P < 0.001) and postoperative complications (15 of 45 [33%] vs 15 of 21 [71%], P = 0.047) and a lower mortality (0 of 64 vs 6 of 33 [18%], P = 0.002). Management of patients presenting with pheochromocytoma crisis should include initial stabilization of the acute crisis followed by sufficient α-blockade before surgery. Emergency resection of pheochromocytoma is associated with high surgical morbidity and mortality.
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              Pheochromocytoma Characteristics and Behavior Differ Depending on Method of Discovery

                Author and article information

                Journal
                Endocrinol Diabetes Metab Case Rep
                Endocrinol Diabetes Metab Case Rep
                EDM
                Endocrinology, Diabetes & Metabolism Case Reports
                Bioscientifica Ltd (Bristol )
                2052-0573
                16 March 2023
                2023
                : 2023
                : 2
                : 22-0392
                Affiliations
                [1 ]Department of Endocrinology , Mater Misericordiae University Hospital, Dublin, Ireland
                [2 ]Department of Surgery , Mater Misericordiae University Hospital, Dublin, Ireland
                [3 ]Department of Critical Care Medicine , Mater Misericordiae University Hospital, Dublin, Ireland
                [4 ]School of Medicine , University College Dublin, Dublin, Ireland
                Author notes
                Correspondence should be addressed to D Fennell; Email: davidfennell@ 123456mater.ie
                Author information
                http://orcid.org/0000-0001-6066-8318
                http://orcid.org/0000-0003-0193-8976
                http://orcid.org/0000-0002-9597-9471
                Article
                EDM220392
                10.1530/EDM-22-0392
                10241236
                37073853
                1b5af5b7-d858-4ace-b5aa-1956f110aa5e
                © the author(s)

                This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License..

                History
                : 27 October 2022
                : 16 March 2023
                Categories
                Adult
                Female
                Hispanic or Latino - Central American or South American
                White
                Ireland
                Heart
                Adrenal
                Insight into Disease Pathogenesis or Mechanism of Therapy
                Insight into Disease Pathogenesis or Mechanism of Therapy

                adult,female,hispanic or latino - central american or south american,white,ireland,heart,adrenal,insight into disease pathogenesis or mechanism of therapy,april,2023

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