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Hypereosinophilic Syndrome
Seth Kligerman, MD; John D. Grizzard, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        TERMINOLOGY

        • Abbreviations

          • Hypereosinophilic syndrome (HES)
        • Synonyms

          • Cardiac involvement in HES has many synonyms
            • Löffler (or Loeffler) endocarditis
            • Eosinophilic endomyocarditis
            • Nontropical eosinophilic endomyocardial disease
        • Definitions

          • Unexplained hypereosinophilia (> 1,500/μL) of ≥ 6 months duration associated with organ dysfunction due to eosinophilic infiltration
            • Heart is most commonly involved organ (cardiac involvement in up to 60% of cases)

        IMAGING

        • General Features

          • Imaging Recommendations

            • MR Findings

              • CT Findings

                DIFFERENTIAL DIAGNOSIS

                  PATHOLOGY

                  • General Features

                    • Gross Pathologic & Surgical Features

                      CLINICAL ISSUES

                      • Presentation

                        • Demographics

                          • Natural History & Prognosis

                            • Treatment

                              DIAGNOSTIC CHECKLIST

                              • Consider

                                • Image Interpretation Pearls

                                  Selected References

                                  1. Moss AJ et al: A 56-year-old woman with breathlessness. Heart. 103(9):726, 2017
                                  2. Mankad R et al: Hypereosinophilic syndrome: cardiac diagnosis and management. Heart. 102(2):100-6, 2016
                                  3. Perazzolo Marra M et al: Cardiac magnetic resonance features of biopsy-proven endomyocardial diseases. JACC Cardiovasc Imaging. 7(3):309-12, 2014
                                  4. Hoey ET et al: Cardiovascular MRI for assessment of infectious and inflammatory conditions of the heart. AJR Am J Roentgenol. 197(1):103-12, 2011
                                  5. Kleinfeldt T et al: Cardiac manifestation of the hypereosinophilic syndrome: new insights. Clin Res Cardiol. 99(7):419-27, 2010
                                  6. Ogbogu PU et al: Hypereosinophilic syndrome: a multicenter, retrospective analysis of clinical characteristics and response to therapy. J Allergy Clin Immunol. 124(6):1319-25, 2009
                                  7. Chao BH et al: Fatal Loeffler's endocarditis due to hypereosinophilic syndrome. Am J Hematol. 82(10):920-3, 2007
                                  8. Klion AD et al: Approaches to the treatment of hypereosinophilic syndromes: a workshop summary report. J Allergy Clin Immunol. 117(6):1292-302, 2006
                                  9. Cury RC et al: Images in cardiovascular medicine. Visualization of endomyocardial fibrosis by delayed-enhancement magnetic resonance imaging. Circulation. 111(9):e115-7, 2005
                                  10. Salanitri GC: Endomyocardial fibrosis and intracardiac thrombus occurring in idiopathic hypereosinophilic syndrome. AJR Am J Roentgenol. 184(5):1432-3, 2005
                                  11. Bishop GG et al: Hypereosinophilic syndrome and restrictive cardiomyopathy due to apical thrombi. Circulation. 104(2):E3-4, 2001
                                  12. Puvaneswary M et al: Idiopathic hypereosinophilic syndrome: magnetic resonance imaging findings in endomyocardial fibrosis. Australas Radiol. 45(4):524-7, 2001
                                  13. Ommen SR et al: Clinical and echocardiographic features of hypereosinophilic syndromes. Am J Cardiol. 86(1):110-3, 2000
                                  Related Anatomy
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                                  Related Differential Diagnoses
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                                  References
                                  Tables

                                  Tables

                                  KEY FACTS

                                  • Terminology

                                    • Imaging

                                      • Top Differential Diagnoses

                                        TERMINOLOGY

                                        • Abbreviations

                                          • Hypereosinophilic syndrome (HES)
                                        • Synonyms

                                          • Cardiac involvement in HES has many synonyms
                                            • Löffler (or Loeffler) endocarditis
                                            • Eosinophilic endomyocarditis
                                            • Nontropical eosinophilic endomyocardial disease
                                        • Definitions

                                          • Unexplained hypereosinophilia (> 1,500/μL) of ≥ 6 months duration associated with organ dysfunction due to eosinophilic infiltration
                                            • Heart is most commonly involved organ (cardiac involvement in up to 60% of cases)

                                        IMAGING

                                        • General Features

                                          • Imaging Recommendations

                                            • MR Findings

                                              • CT Findings

                                                DIFFERENTIAL DIAGNOSIS

                                                  PATHOLOGY

                                                  • General Features

                                                    • Gross Pathologic & Surgical Features

                                                      CLINICAL ISSUES

                                                      • Presentation

                                                        • Demographics

                                                          • Natural History & Prognosis

                                                            • Treatment

                                                              DIAGNOSTIC CHECKLIST

                                                              • Consider

                                                                • Image Interpretation Pearls

                                                                  Selected References

                                                                  1. Moss AJ et al: A 56-year-old woman with breathlessness. Heart. 103(9):726, 2017
                                                                  2. Mankad R et al: Hypereosinophilic syndrome: cardiac diagnosis and management. Heart. 102(2):100-6, 2016
                                                                  3. Perazzolo Marra M et al: Cardiac magnetic resonance features of biopsy-proven endomyocardial diseases. JACC Cardiovasc Imaging. 7(3):309-12, 2014
                                                                  4. Hoey ET et al: Cardiovascular MRI for assessment of infectious and inflammatory conditions of the heart. AJR Am J Roentgenol. 197(1):103-12, 2011
                                                                  5. Kleinfeldt T et al: Cardiac manifestation of the hypereosinophilic syndrome: new insights. Clin Res Cardiol. 99(7):419-27, 2010
                                                                  6. Ogbogu PU et al: Hypereosinophilic syndrome: a multicenter, retrospective analysis of clinical characteristics and response to therapy. J Allergy Clin Immunol. 124(6):1319-25, 2009
                                                                  7. Chao BH et al: Fatal Loeffler's endocarditis due to hypereosinophilic syndrome. Am J Hematol. 82(10):920-3, 2007
                                                                  8. Klion AD et al: Approaches to the treatment of hypereosinophilic syndromes: a workshop summary report. J Allergy Clin Immunol. 117(6):1292-302, 2006
                                                                  9. Cury RC et al: Images in cardiovascular medicine. Visualization of endomyocardial fibrosis by delayed-enhancement magnetic resonance imaging. Circulation. 111(9):e115-7, 2005
                                                                  10. Salanitri GC: Endomyocardial fibrosis and intracardiac thrombus occurring in idiopathic hypereosinophilic syndrome. AJR Am J Roentgenol. 184(5):1432-3, 2005
                                                                  11. Bishop GG et al: Hypereosinophilic syndrome and restrictive cardiomyopathy due to apical thrombi. Circulation. 104(2):E3-4, 2001
                                                                  12. Puvaneswary M et al: Idiopathic hypereosinophilic syndrome: magnetic resonance imaging findings in endomyocardial fibrosis. Australas Radiol. 45(4):524-7, 2001
                                                                  13. Ommen SR et al: Clinical and echocardiographic features of hypereosinophilic syndromes. Am J Cardiol. 86(1):110-3, 2000