Advertisement

Clinical Research in Cardiology

, Volume 99, Issue 7, pp 419–427 | Cite as

Cardiac manifestation of the hypereosinophilic syndrome: new insights

  • T. Kleinfeldt
  • C. A. Nienaber
  • S. Kische
  • I. Akin
  • R. G. Turan
  • T. Körber
  • H. Schneider
  • H. Ince
Review

Abstract

Cardiac manifestation is the major cause of morbidity in patients with hypereosinophilic syndrome (HES). Clinical features range from heart failure to arterial embolism, which are caused by thickening of the endocardium and mural left ventricular thrombosis. Modern magnetic resonance imaging and echocardiography are able to detect fibrosis, eosinophilic infiltrate and thrombi to stage the fibrotic evolution of the disease. Treatment of HES involves standard medication for heart failure, anticoagulant therapy, immunosuppressive therapy and potentially surgical resection. The outcome of HES depends on both the progression of endocardial fibrosis and associated complications and the 5-year mortality is estimated at 30%.

Keywords

Hypereosinophilic syndrome MRI Endocardiac fibrosis Immunosuppression Cardiomyopathy Gene Thrombus Echocardiography Biopsy Therapy 

Notes

Acknowledgments

The authors wish to acknowledge Princess Ogbogu, M.D., Douglas R. Rosing, M.D., and McDonald K. Horne, III, M.D. Allergy and Immunology, National Institute of Allergy and Infectious Diseases, National Institutes of Health. Written informed consent was obtained from the patient for publication of the accompanying images (Figs. 3a, b; 4a, b). A copy of the written consent is available for review by the Editor-in-Chief of this journal. The authors wish also to acknowledge Korczyk D et al. for Fig. 1a, b and Yeon Joo Jeong for Fig. 5a–c.

Conflict of interest statement

None.

References

  1. 1.
    Klion AD, Bochner BS, Gleich GJ et al (2006) Approaches to the treatment of hypereosinophilic syndromes: a workshop summary report. J Allergy Clin Immunol 117:1292–1302CrossRefPubMedGoogle Scholar
  2. 2.
    Corssmit EP, Trip MD, Durrer JD (1999) Loffler’s endomyocarditis in the idiopathic hypereosinophilic syndrome. Cardiology 91:272–276CrossRefPubMedGoogle Scholar
  3. 3.
    Parrillo JE (1990) Heart disease and the eosinophil. N Engl J Med 323:1560–1561PubMedGoogle Scholar
  4. 4.
    Chusid MJ, Dale DC, West BC, Wolff SM (1975) The hypereosinophilic syndrome: analysis of fourteen cases with review of the literature. Medicine (Baltimore) 54:1–27CrossRefGoogle Scholar
  5. 5.
    Weller PF, Bubley GJ (1994) The idiopathic hypereosinophilic syndrome. Blood 83:2759–2779PubMedGoogle Scholar
  6. 6.
    Chang HW, Leong KH, Koh DR, Lee SH (1999) Clonality of isolated eosinophils in the hypereosinophilic syndrome. Blood 93:1651–1657PubMedGoogle Scholar
  7. 7.
    Löffler W. Endocarditis parietalis fibroplastica mit Bluteosinophilie. Schweiz Med Wochenschr 1936, 65:817–820 and Klinische Wochenschrift, Berlin, 1935, 14:297–299Google Scholar
  8. 8.
    Fletcher S, Bain B (2007) Diagnosis and treatment of hypereosinophilic syndromes, current opinion in hematology. Curr Opin Hematol 14(1):37–42CrossRefPubMedGoogle Scholar
  9. 9.
    Baxter EJ, Hochhaus A, Bolufer P et al (2002) The t(4;22)(q12;q11) in atypical chronic myeloid leukaemia fuses BCR to PDGFRA. Hum Mol Genet 11:1391–1397CrossRefPubMedGoogle Scholar
  10. 10.
    Score J, Curtis C, Waghorn K et al (2006) Identification of a novel imatinib responsive KIF5B–PDGFRA fusion gene following screening for PDGFRA overexpression in patients with hypereosinophilia. Leukemia 20:827–832CrossRefPubMedGoogle Scholar
  11. 11.
    Walz C, Curtis C, Schnittger S et al (2006) Transient response to imatinib in a chronic eosinophilic leukaemia associated with ins(9;4)(q33;q12q25) and a CDK5RAP2–PDGFRA fusion gene. Genes Chromosomes Cancer 45(10):950–956CrossRefPubMedGoogle Scholar
  12. 12.
    Young JD, Peterson CG, Venge P, Cohn ZA (1986) Mechanism of membrane damage mediated by human eosinophil cationic protein. Nature 321(6070):613–616CrossRefPubMedGoogle Scholar
  13. 13.
    Tai PC, Ackerman SJ, Spry CJ, Dunnette S, Olsen EG, Gleich GJ (1987) Deposits of eosinophil granule proteins in cardiac tissues of patients with eosinophilic endomyocardial disease. Lancet 1(8534):643–647CrossRefPubMedGoogle Scholar
  14. 14.
    Ruggeri ZM (2006) Platelet interactions with vessel wall components during thrombogenesis. Blood Cells Mol Dis 36(2):145–147CrossRefPubMedGoogle Scholar
  15. 15.
    Rohrbach MS, Wheatley CL, Slifman NR, Gleich GJ (1990) Activation of platelets by eosinophil granule proteins. J Exp Med 172(4):1271–1274CrossRefPubMedGoogle Scholar
  16. 16.
    Ogbogu P, Rosing DR, Horne MD (2007) Cardiovascular manifestation of hypereosinophilic syndromes. Immunol Allergy Clin North Am 27(3):457–475CrossRefPubMedGoogle Scholar
  17. 17.
    Roufosse F, Cogan E, Goldman M (2004) Recent advances in pathogenesis and management of hypereosinophilic syndromes. Allergy 59:673–689CrossRefPubMedGoogle Scholar
  18. 18.
    Parrillo JE, Borer JS, Henry WL, Wolff SM, Fauci AS (1979) The cardiovascular manifestations of the hypereosinophilic syndrome. Prospective study of 26 patients, with review of the literature. Am J Med 67(4):572–582CrossRefPubMedGoogle Scholar
  19. 19.
    Roufosse F, Goldman M, Cogan E (2006) Hypereosinophilic syndrome: lymphoproliferative and myeloproliferative variants. Semin Respir Crit Care Med 27:158–170CrossRefPubMedGoogle Scholar
  20. 20.
    Jaffe ES, Harris NL, Stein H et al (2001) World Health Organization classification of tumours: pathology and genetics of tumours of haematopoietic and lymphoid tissues. IARSPress, LyonGoogle Scholar
  21. 21.
    Cohen J, Goodwin J, Spry JF et al (1980) Arrhythmias in patients with hypereosinophilia: a comparison of patients with and without Loffler’s endomyocardial disease. Postgrad Med J 56:828–832CrossRefPubMedGoogle Scholar
  22. 22.
    Oh JK, Seward JB, Tajik AJ (1999) The echo manual, 2nd edn edn. Lippincott-Raven, PhiladelphiaGoogle Scholar
  23. 23.
    Braunwald E (2005) The cardiomyopathies. In: Zipes D et al (eds) Braunwald’s heart disease, 7th edn. Saunders, PhiladelphiaGoogle Scholar
  24. 24.
    Shah R, Ananthasubramaniam K (2006) Evaluation of cardiac involvement in hypereosinophilic syndrome: complementary roles of transthoracic, transesophageal, and contrast echocardiography. Echocardiography 23(8):689–691CrossRefPubMedGoogle Scholar
  25. 25.
    Jeserich M, Konsantinides S, Pavlik G, Bode C, Geibel A (2009) Non-invasive imaging in the diagnosis of acute viral myocarditis. Clin Res Cardiol 98(12):753–763CrossRefPubMedGoogle Scholar
  26. 26.
    Eitel I, Fuernau G, Walther C, Razek V, Kivelitz D, Schuler G, Thiele H (2008) Delayed enhancement magnetic resonance imaging in isolated noncompaction of ventricular myocardium. Clin Res Cardiol 97(4):280–283CrossRefGoogle Scholar
  27. 27.
    Hombach V, Merkle N, Kestler HA, Torzewski J, Kochs M, Marx N, Nusser T, Burgstrahler C, Rasche V, Wöhrle J (2008) Characterization of patients with acute chest pain using cardiac magnetic resonance imaging. Clin Res Cardiol 97(10):760–767CrossRefPubMedGoogle Scholar
  28. 28.
    Debl K, Djavidani B, Luchner A et al (2008) Time course of eosinophilic myocarditis visualized by CMR. J Cardiovasc Magn Reson 10:21CrossRefPubMedGoogle Scholar
  29. 29.
    Fauci AS, Harley JB, Roberts WC, Ferrans VJ, Gralnick HR, Bjornson BH (1982) NIH conference. The idiopathic hypereosinophilic syndrome. Clinical, pathophysiologic, and therapeutic considerations. Ann Intern Med 97(1):78–92PubMedGoogle Scholar
  30. 30.
    Weller PF (1991) The immunobiology of eosinophils. N Engl J Med 324:1110–1118PubMedCrossRefGoogle Scholar
  31. 31.
    Jourdain P, Joneau G, Juilliere Y et al (2007) Plasma brain natriuretic peptide-guided therapy to improve outcome in heart failure the STARS-BNP Multicenter Study. J Am Coll Cardiol 49:1733–1739CrossRefPubMedGoogle Scholar
  32. 32.
    Troughton RW, Frampton CM, Richards AM et al (2000) Treatment of heart failure guided by plasma aminoterminal brain natriuretic peptide (N-BNP) concentrations. Lancet 355:1126–1130CrossRefPubMedGoogle Scholar
  33. 33.
    Hunt SA, Abraham WT, Chin MH et al (2005) ACC/AHA 2005 Guideline update for the diagnosis and management of chronic heart failure in the adult: a report of the American College of Cardiology/American Heart Association Task Force on practice guidelines (Writing Committee to Update the 2001 Guidelines for the evaluation and management of heart failure): developed in collaboration with the American College of Chest Physicians and the International Society for Heart and Lung Transplantation: endorsed by the Heart Rhythm Society. Circulation 112(12):154–235CrossRefGoogle Scholar
  34. 34.
    Teles C, Lopes C, Manique A, Moniz D, Sotto-Mayor R, Bugalho de Almeida A (2007) Pulmonary embolism and difficult-to-treat asthma. Rev Port Pneumol 13(6):775–787Google Scholar
  35. 35.
    Sarazin M, Caumes E, Cohen A, Amarenco P (2004) Multiple microembolic border zone brain infarctions and endomyocardial fibrosis in idiopathic hypereosinophilic syndrome and in Schistosoma mansoni infestation. J Neurol Neurosurg Psychiatry 75(2):305–307PubMedGoogle Scholar
  36. 36.
    Moore PM, Harley JB, Fauci AS (1985) Neurologic dysfunction in the idiopathic hypereosinophilic syndrome. Ann Intern Med 102(1):109–114PubMedGoogle Scholar
  37. 37.
    Spry CJ, Davies J, Tai PC, Olsen EG, Oakley CM, Goodwin JF (1983) Clinical features of fifteen patients with the hypereosinophilic syndrome. Q J Med 52(205):1–22PubMedGoogle Scholar
  38. 38.
    Terrier B, Piette AM, Kerob D, Cordoliani F, Tancrède E, Hamidou L, Lebbé C, Blétry O, Kahn JE (2006) Superficial venous thrombophlebitis as the initial manifestation of hypereosinophilic syndrome: study of the first 3 cases. Arch Dermatol 142(12):1606–1610CrossRefPubMedGoogle Scholar
  39. 39.
    Hurlen M, Abdelnoor M, Smith P, Erikssen J, Arnesen H (2002) Warfarin, aspirin, or both after myocardial infarction. N Engl J Med 347(13):969–974CrossRefPubMedGoogle Scholar
  40. 40.
    Fiore LD, Ezekowitz MD, Brophy MT, Lu D, Sacco J, Peduzzi P (2002) Department of Veterans Affairs Cooperative Studies Program Clinical Trial comparing combined warfarin and aspirin with aspirin alone in survivors of acute myocardial infarction: primary results of the CHAMP study. Circulation 105(5):557–563CrossRefPubMedGoogle Scholar
  41. 41.
    Buresly K, Eisenberg MJ, Zhang X, Pilote L (2005) Bleeding complications associated with combinations of aspirin, thienopyridine derivatives, and warfarin in elderly patients following acute myocardial infarction. Arch Intern Med 165(7):784–789CrossRefPubMedGoogle Scholar
  42. 42.
    Turpie AG, Gent M, Laupacis A et al (1993) A comparison of aspirin with placebo in patients treated with warfarin after heart-valve replacement. N Engl J Med 329(8):524–529CrossRefPubMedGoogle Scholar
  43. 43.
    Klion AD, Bochner BS, Gleich GJ et al (2006) Approaches to the treatment of hypereosinophilic syndromes: a workshop summary report. J Allergy Clin Immunol 117:1292–1302CrossRefPubMedGoogle Scholar
  44. 44.
    Metzgeroth G, Walz C, Erben P, Reiter A et al (2008) Safety and efficacy of imatinib in chronic eosinophilic leukaemia and hypereosinophilic syndrome: a phase-II study. Br J Haematol 143(5):707–715CrossRefPubMedGoogle Scholar
  45. 45.
    Rothenberg ME et al (2008) Treatment of hypereosinophilic syndrome with mepolizumab. N Engl J Med 358:12CrossRefGoogle Scholar
  46. 46.
    Nadarajah S, Krafchik B, Roifman C et al (1997) Treatment of hypereosinophilic syndrome in a child using cyclosporine: implication for a primary T cell abnormality. Pediatrics 99:630–633CrossRefPubMedGoogle Scholar
  47. 47.
    Schneider U, Jenni R, Turina J, Turina M, Hess OM (1998) Long term follow up of patients with endomyocardial fibrosis: effects of surgery. Heart 79:362–367PubMedGoogle Scholar
  48. 48.
    Metras D, Coulibaly AO, Ouattara K (1985) The surgical treatment of endomyocardial fibrosis: results in 55 patients. Circulation 72:II274–II279PubMedGoogle Scholar
  49. 49.
    Dubost C, Prigrent C, Gerbaux A, Maurice P, Passeleq J, Rulliere R, Carpentier A, Deloche A (1981) Surgical treatment of constrictive fibrous endocarditis. J Thorac Cardiovasc Surg 82:585–591PubMedGoogle Scholar
  50. 50.
    Metras D, Coulibaly AO, Ouattara K (1985) The surgical treatment of endomyocardial fibrosis: results in 55 patients. Circulation 72:II274–II279PubMedGoogle Scholar
  51. 51.
    Moraes C, Buffolo E, Lima R, Victor E, Lira V, Escobar M, Rodrigues J, Saravia L, Andrade J (1983) Surgical treatment of endomyocardial fibrosis. J Thorac Cardiovasc Surg 85:738–745PubMedGoogle Scholar
  52. 52.
    Joshi R, Abraham S, Kumar AS (2003) New approach for complete endocardiectomy in left ventricular endomyocardial fibrosis. J Thorac Cardiovasc Surg 125(1):40–42CrossRefPubMedGoogle Scholar
  53. 53.
    Fuzellier JF, Chapoutot L, Torossian PF (2004) Mitral valve repair in idiopathic hypereosinophilic syndrome. J Heart Valve Dis 13(3):529–531PubMedGoogle Scholar
  54. 54.
    Radford DJ, Garlick RB, Pohlner PG (2002) Multiple valvular replacements for hypereosinophilic syndrome. Cardiol Young 12(1):67–70CrossRefPubMedGoogle Scholar
  55. 55.
    Boustany CW, Murphy GW, Hicks GL (1991) Mitral valve replacement in idiopathic hypereosinophilic syndrome. Ann Thorac Surg 51(6):1007–1009PubMedCrossRefGoogle Scholar
  56. 56.
    Korczyk D, Taylor G, McAlistair H et al (2007) Heart transplantation in a patient with endomyocardial fibrosis due to hypereosinophilic syndrome. Transplantation 83(4):514–516CrossRefPubMedGoogle Scholar
  57. 57.
    Jeong Yeon Joo (2007) Eosinophilic lung diseases: a clinical, radiologic, and pathologic overview. Radiographics 27:617–637CrossRefPubMedGoogle Scholar
  58. 58.
    Starr J et al (2000) Myiasis due to hypoderma lineatum infection mimicking the hypereosinophilic syndrome. Mayo Clin Proc 75:755–759CrossRefPubMedGoogle Scholar

Copyright information

© Springer-Verlag 2010

Authors and Affiliations

  • T. Kleinfeldt
    • 1
  • C. A. Nienaber
    • 1
  • S. Kische
    • 1
  • I. Akin
    • 1
  • R. G. Turan
    • 1
  • T. Körber
    • 1
  • H. Schneider
    • 1
  • H. Ince
    • 1
  1. 1.Medizinische Klinik I-KardiologieUniversity of RostockRostockGermany

Personalised recommendations