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      Cardiogenic Shock and Guillain–Barré Syndrome as the First Manifestations of Pheochromocytoma

      case-report

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          Abstract

          Febrile congestive heart failure is a rare first manifestation of pheochromocytoma. Herein, the case of a 31-year-old female with febrile congestive heart failure and subsequent cardiogenic shock is presented. After intensive care unit (ICU) admission and further evaluating the right adrenal mass observed in abdominal ultrasonography, the diagnosis of pheochromocytoma was confirmed. Then, she was scheduled for the right adrenalectomy. Before surgery, she complained of acute-onset progressive muscle weakness in the lower limbs, followed by the upper limbs. After further investigation, she was diagnosed with Guillain–Barré syndrome and treated with intravenous immunoglobulin (IVIG). She recovered well after the right adrenalectomy, and during the subsequent 18 months, the follow-up did not reveal any complications, and left ventricular function recovered to normal.

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          Most cited references21

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          Phaeochromocytoma.

          Phaeochromocytomas are rare neuroendocrine tumours with a highly variable clinical presentation but most commonly presenting with episodes of headaches, sweating, palpitations, and hypertension. The serious and potentially lethal cardiovascular complications of these tumours are due to the potent effects of secreted catecholamines. Biochemical testing for phaeochromocytoma is indicated not only in symptomatic patients, but also in patients with adrenal incidentalomas or identified genetic predispositions (eg, multiple endocrine neoplasia type 2, von Hippel-Lindau syndrome, neurofibromatosis type 1, and mutations of the succinate dehydrogenase genes). Imaging techniques such as CT or MRI and functional ligands such as (123)I-MIBG are used to localise biochemically proven tumours. After the use of appropriate preoperative treatment to block the effects of secreted catecholamines, laparoscopic tumour removal is the preferred procedure. If removal of phaeochromocytoma is timely, prognosis is excellent. However, prognosis is poor in patients with metastases, which especially occur in patients with large, extra-adrenal tumours.
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            Pheochromocytoma: current approaches and future directions.

            Pheochromocytomas are rare catecholamine-secreting tumors that arise from chromaffin tissue within the adrenal medulla and extra-adrenal sites. Because of the excess secretion of hormones, these tumors often cause debilitating symptoms and a poor quality of life. While medical management plays a significant role in the treatment of pheochromocytoma patients, surgical excision remains the only cure. Improved medical management and surgical techniques and an increased understanding of hereditary disease have improved the outcome of pheochromocytoma patients with benign disease; however, the outcome of patients with malignant disease remains poor. In this review, we discuss the presentation, diagnosis, management, and future directions in the management of this disease.
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              Acute catecholamine cardiomyopathy in patients with phaeochromocytoma or functional paraganglioma.

              Phaeochromocytomas and paragangliomas (PPGL) can cause acute catecholamine cardiomyopathy (ACC). We assessed the prevalence of ACC and compared the presentation of cases with and without ACC in a large series of PPGL. Single centre retrospective study. Hypertension Unit, University Hospital, Paris. 140 consecutive patients with PPGL, referred from January 2003 to September 2012. Left ventricular ejection fraction (LVEF), perioperative mortality. Fifteen patients (11%) had suffered an ACC, occurring in 14 cases before the diagnosis of PPGL. Precipitating factors were identified in 11 cases. Twelve patients presented with acute pulmonary oedema, including 10 with cardiogenic shock, requiring life support in eight cases. Seven patients (five with pulmonary oedema) presented with acute chest pain and cardiac dysfunction. Electrocardiographic abnormalities were present in 14 cases: ST segment elevation or pathological Q waves, ST segment depression, and/or diffuse T wave inversion. Six patients displayed classical (apical ballooning) or inverted (basal/mid ventricular stunning) takotsubo-like cardiomyopathy. Coronary arteries were always normal on angiography. In patients with ACC, median LVEF rose from 30% (IQR 23-33%) during ACC to 71% (50-72%) before surgery (n=11, p<0.001). Median LVEF before PPGL surgery was 65% (51-72%) and 65% (60-70%) in patients with and without a history of ACC, respectively (not significant). PPGL may present as ACC in 11% of cases, excluding patients dying from undiagnosed tumours. Left ventricular dysfunction is usually reversible before surgery. PPGL should be suspected in patients with acute heart failure without evidence of valvular or coronary artery disease.
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                Author and article information

                Contributors
                Journal
                Case Rep Endocrinol
                Case Rep Endocrinol
                CRIE
                Case Reports in Endocrinology
                Hindawi
                2090-6501
                2090-651X
                2021
                3 June 2021
                : 2021
                : 6691095
                Affiliations
                1Isfahan Endocrine and Metabolism Research Center, Isfahan University of Medical Sciences, Isfahan, Iran
                2Heart Failure Research Center, Isfahan Cardiovascular Research Institute, Isfahan University of Medical Sciences, Isfahan, Iran
                Author notes

                Academic Editor: Osamu Isozaki

                Author information
                https://orcid.org/0000-0002-7999-2393
                https://orcid.org/0000-0003-4524-6744
                Article
                10.1155/2021/6691095
                8192183
                7d75e0d7-7f0f-4f0f-aab9-625f04ca2074
                Copyright © 2021 Maryam Heidarpour et al.

                This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                : 9 November 2020
                : 5 May 2021
                : 25 May 2021
                Categories
                Case Report

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