Cardiology - Case Reports Open access

Severe heart failure and intracardiac thrombosis: going beyond the appearance for diagnosis and treatments

Authors

  • Andrea Segreti – articles in this journal Corresponding author Email https://orcid.org/0000-0003-2631-8642 Research Unit of Cardiovascular Science, Department of Medicine and Surgery, Università Campus Bio-Medico di Roma; Cardiology Unit, Fondazione Policlinico Universitario Campus Bio-Medico, Rome; Department of Movement, Human and Health Sciences, University of Rome “Foro Italico”, Italy.
  • Sara Mastroberardino – articles in this journal Research Unit of Cardiovascular Science, Department of Medicine and Surgery, Università Campus Bio-Medico di Roma; Cardiology Unit, Fondazione Policlinico Universitario Campus Bio-Medico, Rome, Italy.
  • Lorenzo Frau – articles in this journal Research Unit of Cardiovascular Science, Department of Medicine and Surgery, Università Campus Bio-Medico di Roma; Cardiology Unit, Fondazione Policlinico Universitario Campus Bio-Medico, Rome, Italy.
  • Alessandro Appetecchia – articles in this journal Research Unit of Cardiovascular Science, Department of Medicine and Surgery, Università Campus Bio-Medico di Roma; Cardiology Unit, Fondazione Policlinico Universitario Campus Bio-Medico, Rome, Italy.
  • Luca D’Antonio – articles in this journal Research Unit of Cardiovascular Science, Department of Medicine and Surgery, Università Campus Bio-Medico di Roma; Cardiology Unit, Fondazione Policlinico Universitario Campus Bio-Medico, Rome, Italy.
  • Danilo Ricciardi – articles in this journal Research Unit of Cardiovascular Science, Department of Medicine and Surgery, Università Campus Bio-Medico di Roma; Cardiology Unit, Fondazione Policlinico Universitario Campus Bio-Medico, Rome, Italy.
  • Gian Paolo Ussia – articles in this journal Research Unit of Cardiovascular Science, Department of Medicine and Surgery, Università Campus Bio-Medico di Roma; Cardiology Unit, Fondazione Policlinico Universitario Campus Bio-Medico, Rome, Italy.
  • Francesco Grigioni – articles in this journal Research Unit of Cardiovascular Science, Department of Medicine and Surgery, Università Campus Bio-Medico di Roma; Cardiology Unit, Fondazione Policlinico Universitario Campus Bio-Medico, Rome, Italy.
Published
2 May 2024
Section
Cardiology - Case Reports

Abstract

We describe the case of a 45-year-old female affected by asthma and nasal polyposis who presented to the emergency department because of worsening dyspnea and paresthesia of the left lower limb. Comprehensive instrumental examinations revealed the presence of severe left ventricle dysfunction, intracardiac thrombus, deep vein thrombosis, pulmonary embolism, lung infiltrates, polyserositis, and neurological involvement. Finally, the patient was diagnosed with eosinophilic granulomatosis with polyangiitis (EGPA), formerly Churg-Strauss syndrome, a rare vasculitis of small-medium blood vessels with several organ involvements. Treatment with anticoagulants, corticosteroids, and cyclophosphamide led to a significant clinical improvement. However, a subcutaneous cardiac defibrillator was implanted because of the persistence of severe left ventricular dysfunction and the high cardiovascular risk phenotype. Indeed, several cardiac manifestations may occur in EGPA, particularly in patients with anti-neutrophil cytoplasmic antibody-negative disease. Therefore, clinicians should have high clinical suspicion because cardiac involvement in EGPA results in a poor prognosis if not diagnosed and adequately treated.

References

  1. Churg J, Strauss L. Allergic granulomatosis, allergic angiitis, and periarteritis nodosa. Am J Pathol 1951;27:277-301.
  2. White J, Dubey S. Eosinophilic granulomatosis with polyangiitis: a review. Autoimmun Rev 2023;22:103219. DOI: https://doi.org/10.1016/j.autrev.2022.103219
  3. Grayson PC, Ponte C, Suppiah R, et al. 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology classification criteria for eosinophilic granulomatosis with polyangiitis. Ann Rheum Dis 2022;81:309-14. DOI: https://doi.org/10.1136/annrheumdis-2021-221794
  4. Zeppenfeld K, Tfelt-Hansen J, de Riva M, et al. 2022 ESC Guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death. Eur Heart J 2022;43:3997-4126. DOI: https://doi.org/10.1093/eurheartj/ehac699
  5. Emmi G, Bettiol A, Gelain E, et al. Evidence-based guideline for the diagnosis and management of eosinophilic granulomatosis with polyangiitis. Nat Rev Rheumatol 2023;19:378-93. DOI: https://doi.org/10.1038/s41584-023-00958-w
  6. Neumann T, Manger B, Schmid M, et al. Cardiac involvement in Churg-Strauss syndrome: impact of endomyocarditis. Medicine (Baltimore) 2009;88(4):236-43. DOI: https://doi.org/10.1097/MD.0b013e3181af35a5
  7. Guillevin L, Cohen P, Gayraud M, et al. Churg-Strauss syndrome. Clinical study and long-term follow-up of 96 patients. Medicine (Baltimore) 1999;78:26-37. DOI: https://doi.org/10.1097/00005792-199901000-00003
  8. Garcia-Vives E, Rodriguez-Palomares JF, Harty L, et al. Heart disease in eosinophilic granulomatosis with polyangiitis (EGPA) patients: a screening approach proposal. Rheumatology (Oxford) 2021;60:4538-47. DOI: https://doi.org/10.1093/rheumatology/keab027
  9. Maino A, Rossio R, Cugno M, et al. Hypereosinophilic syndrome, Churg-Strauss syndrome and parasitic diseases: possible links between eosinophilia and thrombosis. Curr Vasc Pharmacol 2012;10:670-5. DOI: https://doi.org/10.2174/157016112801784594
  10. Bond M, Fagni F, Moretti M, et al. At the heart of eosinophilic granulomatosis with polyangiitis: into cardiac and vascular involvement. Curr Rheumatol Rep 2022;24:337-51. DOI: https://doi.org/10.1007/s11926-022-01087-1
  11. Jachiet M, Samson M, Cottin V, et al. Anti‐IgE monoclonal antibody (omalizumab) in refractory and relapsing eosinophilic granulomatosis with polyangiitis (Churg‐Strauss): data on seventeen patients. Arthritis Rheumatol 2016;68:2274-82. DOI: https://doi.org/10.1002/art.39663
  12. Faverio P, Bonaiti G, Bini F, et al. Mepolizumab as the first targeted treatment for eosinophilic granulomatosis with polyangiitis: a review of current evidence and potential place in therapy. Ther Clin Risk Manag 2018:2385-96. DOI: https://doi.org/10.2147/TCRM.S159949
  13. Mohammad A, Hot A, Arndt F, et al. Rituximab for the treatment of eosinophilic granulomatosis with polyangiitis (Churg–Strauss). Ann Rheum Dis 2016;75:396-401. DOI: https://doi.org/10.1136/annrheumdis-2014-206095
  14. Cools J, DeAngelo DJ, Gotlib J, et al. A tyrosine kinase created by fusion of the PDGFRA and FIP1L1 genes as a therapeutic target of imatinib in idiopathic hypereosinophilic syndrome. N Engl J Med 2003;348:1201-14. DOI: https://doi.org/10.1056/NEJMoa025217

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