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Comprehensive review of evaluation and management of cardiac paragangliomas
  1. Sri Harsha Tella1,
  2. Abhishek Jha2,
  3. David Taïeb3,
  4. Keith A Horvath4,
  5. Karel Pacak2
  1. 1Division of Endocrinology and Metabolism, Department of Internal Medicine, University of South Carolina, Columbia, South Carolina, USA
  2. 2Section on Medical Neuroendocrinology, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, Maryland, USA
  3. 3Biophysics and Nuclear Medicine, La Timone University Hospital, European Center for Research in Medical Imaging, Aix-Marseille University, Marseille, France
  4. 4Clinical Transformation, Association of American Medical Colleges, Washington, District of Columbia, USA
  1. Correspondence to Dr Karel Pacak, Section on Medical Neuroendocrinology, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, Maryland, USA; karel{at}mail.nih.gov

Abstract

Cardiac paraganglioma (PGL) is a rare neuroendocrine tumour causing significant morbidity primarily due to norepinephrine secretion potentially causing severe hypertension, palpitations, lethal tachyarrhythmias, stroke and syncope. Cardiologists are faced with two clinical scenarios. The first is the elevated norepinephrine, whose actions must be properly counteracted by adrenoceptor blockade to avoid catastrophic consequences. The second is to evaluate the precise location of a cardiac PGL and its spread since compression of cardiovascular structures may result in ischaemia, angina, non-noradrenergic-induced arrhythmia, cardiac dysfunction or failure. Thus, appropriate assessment of elevated norepinephrine by its metabolite normetanephrine is a gold biochemical standard at present. Furthermore, dedicated cardiac CT, MRI and transthoracic echocardiogram are necessary for the precise anatomic information of cardiac PGL. Moreover, a cardiologist needs to be aware of advanced functional imaging using 68Ga-DOTA(0)-Tyr(3)-octreotide positron emission tomography/CT, which offers the best cardiac PGL-specific diagnostic accuracy and helps to stage and rule out metastasis, determining the next therapeutic strategies. Patients should also undergo genetic testing, especially for mutations in genes encoding succinate dehydrogenase enzyme subunits that are most commonly present as a genetic cause of these tumours. Curative surgical resection after appropriate α-adrenoceptor and β-adrenoceptor blockade in norepinephrine-secreting tumours is the primary therapeutic strategy. Therefore, appropriate and up-to-date knowledge about early diagnosis and management of cardiac PGLs is paramount for optimal outcomes in patients where a cardiologist is an essential team member of a multidisciplinary team in its management.

  • cardiac imaging and diagnostics
  • cardiac CT imaging
  • positron emission tomographic (PET) imaging
  • genetics
  • systemic review
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Footnotes

  • SHT and AJ are joint first authors.

  • Contributions All the authors contributed to this manuscript in terms of researching the material, drafting sections, figures, tables, editing, critical revision and final approval of the manuscript. The authors' contribution to the work was done as part of authors' official duties as NIH employee and is a work of the US Government. Therefore, copyright may not be established in the USA, 17 U.S.C. § 105. If publisher intends to disseminate the work outside of the USA, publisher may secure copyright to the extent authorised under the domestic laws of the relevant country, subject to a paid-up, non-exclusive, irrevocable worldwide licence to the USA in such copyrighted work to reproduce, prepare derivative works, distribute copies to the public and perform publicly and display publicly the work, and to permit others to do so.

  • Funding This study was funded by the National Institutes of Health (grant number Z1AHD008735) awarded to KP. This work was supported by the Intramural Research Program of the National Institutes of Health, Eunice Kennedy Shriver National Institute of Child Health and Human Development.

  • Competing interests None declared.

  • Patient consent for publication Obtained.

  • Provenance and peer review Not commissioned; externally peer reviewed.

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