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Idiopathic giant cell myocarditis

MedGen UID:
82685
•Concept ID:
C0264856
•
Disease or Syndrome
Synonyms: Giant Cell Myocarditis; Giant cell myocarditis; idiopathic giant cell myocarditis; IGCM
SNOMED CT: Giant cell myocarditis (60812006)
 
Monarch Initiative: MONDO:0018008
Orphanet: ORPHA329874

Definition

A rare cardiomyopathy characterized by progressive myocarditis with diffuse infiltration of cardiac tissue by lymphocytes, macrophages, multinuclear giant cells, and myocardial necrosis. Clinical presentation includes rapidly progressive heart failure, ventricular arrhythmias, complete heart block, and sudden cardiac death. Some patients have associated autoimmune disorders. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVIdiopathic giant cell myocarditis

Professional guidelines

PubMed

Idiopathic giant-cell myocarditis--natural history and treatment. Multicenter Giant Cell Myocarditis Study Group Investigators.
Cooper LT Jr, Berry GJ, Shabetai R
N Engl J Med 1997 Jun 26;336(26):1860-6. doi: 10.1056/NEJM199706263362603. PMID: 9197214
Recognition and optimum management of myocarditis.
Caforio AL, McKenna WJ
Drugs 1996 Oct;52(4):515-25. doi: 10.2165/00003495-199652040-00005. PMID: 8891464

Recent clinical studies

Etiology

Manifestations and Outcome of Cardiac Sarcoidosis and Idiopathic Giant Cell Myocarditis by 25-Year Nationwide Cohorts.
Nordenswan HK, Lehtonen J, Ekström K, Räisänen-Sokolowski A, Mäyränpää MI, Vihinen T, Miettinen H, Kaikkonen K, Haataja P, Kerola T, Rissanen TT, Kokkonen J, Alatalo A, Pietilä-Effati P, Utriainen S, Kupari M
J Am Heart Assoc 2021 Mar 16;10(6):e019415. Epub 2021 Mar 4 doi: 10.1161/JAHA.120.019415. PMID: 33660520Free PMC Article
Idiopathic giant cell myocarditis or cardiac sarcoidosis? A retrospective audit of a nationwide case series.
Ekström K, Räisänen-Sokolowski A, Lehtonen J, Nordenswan HK, Mäyränpää MI, Kupari M
ESC Heart Fail 2020 Jun;7(3):1362-1370. Epub 2020 Apr 28 doi: 10.1002/ehf2.12725. PMID: 32343481Free PMC Article
Improved diagnosis of idiopathic giant cell myocarditis and cardiac sarcoidosis by myocardial gene expression profiling.
Lassner D, Kühl U, Siegismund CS, Rohde M, Elezkurtaj S, Escher F, Tschöpe C, Gross UM, Poller W, Schultheiss HP
Eur Heart J 2014 Aug 21;35(32):2186-95. Epub 2014 Mar 24 doi: 10.1093/eurheartj/ehu101. PMID: 24667923
A clinical and histopathologic comparison of cardiac sarcoidosis and idiopathic giant cell myocarditis.
Okura Y, Dec GW, Hare JM, Kodama M, Berry GJ, Tazelaar HD, Bailey KR, Cooper LT
J Am Coll Cardiol 2003 Jan 15;41(2):322-9. doi: 10.1016/s0735-1097(02)02715-8. PMID: 12535829
Idiopathic giant-cell myocarditis--natural history and treatment. Multicenter Giant Cell Myocarditis Study Group Investigators.
Cooper LT Jr, Berry GJ, Shabetai R
N Engl J Med 1997 Jun 26;336(26):1860-6. doi: 10.1056/NEJM199706263362603. PMID: 9197214

Diagnosis

Manifestations and Outcome of Cardiac Sarcoidosis and Idiopathic Giant Cell Myocarditis by 25-Year Nationwide Cohorts.
Nordenswan HK, Lehtonen J, Ekström K, Räisänen-Sokolowski A, Mäyränpää MI, Vihinen T, Miettinen H, Kaikkonen K, Haataja P, Kerola T, Rissanen TT, Kokkonen J, Alatalo A, Pietilä-Effati P, Utriainen S, Kupari M
J Am Heart Assoc 2021 Mar 16;10(6):e019415. Epub 2021 Mar 4 doi: 10.1161/JAHA.120.019415. PMID: 33660520Free PMC Article
Idiopathic giant cell myocarditis or cardiac sarcoidosis? A retrospective audit of a nationwide case series.
Ekström K, Räisänen-Sokolowski A, Lehtonen J, Nordenswan HK, Mäyränpää MI, Kupari M
ESC Heart Fail 2020 Jun;7(3):1362-1370. Epub 2020 Apr 28 doi: 10.1002/ehf2.12725. PMID: 32343481Free PMC Article
Small steps for idiopathic giant cell myocarditis.
Shih JA, Shih JA
Curr Heart Fail Rep 2015 Jun;12(3):263-8. doi: 10.1007/s11897-015-0260-x. PMID: 25895034
Idiopathic giant cell myocarditis and cardiac sarcoidosis.
Blauwet LA, Cooper LT
Heart Fail Rev 2013 Nov;18(6):733-46. doi: 10.1007/s10741-012-9358-3. PMID: 23111533
Giant cell myocarditis.
Cooper LT Jr, Berry GJ, Rizeq M, Schroeder JS
J Heart Lung Transplant 1995 Mar-Apr;14(2):394-401. PMID: 7779862

Therapy

Unraveling the Uncommon: A Case Report of Giant Cell Myocarditis and Examination of Existing Literature.
Gonzalez Moret YA, Jarrett SA, Ahktar H, Moghbeli N, Hasni S, Bozorgnia B, Bhat RR
Am J Case Rep 2024 Mar 3;25:e942381. doi: 10.12659/AJCR.942381. PMID: 38431769Free PMC Article
Small steps for idiopathic giant cell myocarditis.
Shih JA, Shih JA
Curr Heart Fail Rep 2015 Jun;12(3):263-8. doi: 10.1007/s11897-015-0260-x. PMID: 25895034
Rituximab in recurrent idiopathic giant cell myocarditis after heart transplantation: a potential therapeutic approach.
Toscano G, Tartaro P, Fedrigo M, Angelini A, Marcolongo R
Transpl Int 2014 May;27(5):e38-42. Epub 2014 Feb 17 doi: 10.1111/tri.12270. PMID: 24444397
Combined immunosuppression for the treatment of idiopathic giant cell myocarditis.
Menghini VV, Savcenko V, Olson LJ, Tazelaar HD, Dec GW, Kao A, Cooper LT Jr
Mayo Clin Proc 1999 Dec;74(12):1221-6. doi: 10.4065/74.12.1221. PMID: 10593350
Recognition and optimum management of myocarditis.
Caforio AL, McKenna WJ
Drugs 1996 Oct;52(4):515-25. doi: 10.2165/00003495-199652040-00005. PMID: 8891464

Prognosis

Manifestations and Outcome of Cardiac Sarcoidosis and Idiopathic Giant Cell Myocarditis by 25-Year Nationwide Cohorts.
Nordenswan HK, Lehtonen J, Ekström K, Räisänen-Sokolowski A, Mäyränpää MI, Vihinen T, Miettinen H, Kaikkonen K, Haataja P, Kerola T, Rissanen TT, Kokkonen J, Alatalo A, Pietilä-Effati P, Utriainen S, Kupari M
J Am Heart Assoc 2021 Mar 16;10(6):e019415. Epub 2021 Mar 4 doi: 10.1161/JAHA.120.019415. PMID: 33660520Free PMC Article
Idiopathic giant cell myocarditis or cardiac sarcoidosis? A retrospective audit of a nationwide case series.
Ekström K, Räisänen-Sokolowski A, Lehtonen J, Nordenswan HK, Mäyränpää MI, Kupari M
ESC Heart Fail 2020 Jun;7(3):1362-1370. Epub 2020 Apr 28 doi: 10.1002/ehf2.12725. PMID: 32343481Free PMC Article
Giant cell myocarditis presenting with acute heart failure.
Kasouridis I, Majo J, MacGowan G, Clark AL
BMJ Case Rep 2017 May 22;2017 doi: 10.1136/bcr-2017-219574. PMID: 28536222Free PMC Article
Idiopathic giant cell myocarditis and cardiac sarcoidosis.
Blauwet LA, Cooper LT
Heart Fail Rev 2013 Nov;18(6):733-46. doi: 10.1007/s10741-012-9358-3. PMID: 23111533
Giant cell myocarditis.
Cooper LT Jr, Berry GJ, Rizeq M, Schroeder JS
J Heart Lung Transplant 1995 Mar-Apr;14(2):394-401. PMID: 7779862

Clinical prediction guides

Manifestations and Outcome of Cardiac Sarcoidosis and Idiopathic Giant Cell Myocarditis by 25-Year Nationwide Cohorts.
Nordenswan HK, Lehtonen J, Ekström K, Räisänen-Sokolowski A, Mäyränpää MI, Vihinen T, Miettinen H, Kaikkonen K, Haataja P, Kerola T, Rissanen TT, Kokkonen J, Alatalo A, Pietilä-Effati P, Utriainen S, Kupari M
J Am Heart Assoc 2021 Mar 16;10(6):e019415. Epub 2021 Mar 4 doi: 10.1161/JAHA.120.019415. PMID: 33660520Free PMC Article
Idiopathic giant cell myocarditis in childhood: A case report.
Pehlivan S, Akçan R, Heybet ER, Cavlak M, Pehlivan A
Leg Med (Tokyo) 2016 Mar;19:93-5. Epub 2015 Aug 7 doi: 10.1016/j.legalmed.2015.08.001. PMID: 26275338
Improved diagnosis of idiopathic giant cell myocarditis and cardiac sarcoidosis by myocardial gene expression profiling.
Lassner D, Kühl U, Siegismund CS, Rohde M, Elezkurtaj S, Escher F, Tschöpe C, Gross UM, Poller W, Schultheiss HP
Eur Heart J 2014 Aug 21;35(32):2186-95. Epub 2014 Mar 24 doi: 10.1093/eurheartj/ehu101. PMID: 24667923
A clinical and histopathologic comparison of cardiac sarcoidosis and idiopathic giant cell myocarditis.
Okura Y, Dec GW, Hare JM, Kodama M, Berry GJ, Tazelaar HD, Bailey KR, Cooper LT
J Am Coll Cardiol 2003 Jan 15;41(2):322-9. doi: 10.1016/s0735-1097(02)02715-8. PMID: 12535829
The role of right ventricular endomyocardial biopsy for idiopathic giant cell myocarditis.
Shields RC, Tazelaar HD, Berry GJ, Cooper LT Jr
J Card Fail 2002 Apr;8(2):74-8. doi: 10.1054/jcaf.2002.32196. PMID: 12016630

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