Giant cell myocarditis

Myocarditis Microchapters

Home

Patient Information

Overview

Historical Perspective

Classification

Pathophysiology

Adrenergic Myocarditis
Giant Cell Myocarditis

Causes

Differentiating Myocarditis from other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications and Prognosis

Diagnosis

Diagnostic Study of Choice

History and Symptoms

Physical Examination

Laboratory Findings

Electrocardiogram

X-ray

Echocardiography and Ultrasound

CT scan

MRI

Other Imaging Findings

Other Diagnostic Studies

Treatment

Medical Therapy

Surgery

Primary Prevention

Secondary Prevention

Cost-Effectiveness of Therapy

Future or Investigational Therapies

Case Studies

Case #1

Giant cell myocarditis On the Web

Most recent articles

Most cited articles

Review articles

CME Programs

Powerpoint slides

Google Images

American Roentgen Ray Society Images of Giant cell myocarditis

All Images
X-rays
Echo & Ultrasound
CT Images
MRI

Ongoing Trials at Clinical Trials.gov

US National Guidelines Clearinghouse

NICE Guidance

FDA on Giant cell myocarditis

CDC on Giant cell myocarditis

Giant cell myocarditis in the news

Blogs on Giant cell myocarditis

Directions to Hospitals Treating Type page name here

Risk calculators and risk factors for Giant cell myocarditis

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor: Cafer Zorkun, M.D., Ph.D. [2]

Synonyms and keywords: GCM; idiopathic giant cell myocarditis; IGCM.

## Overview

[edit | edit source]

Giant cell myocarditis is a rare but often fatal inflammatory process involving the myocardium. Other than cardiac transplantation, there is no known effective treatment for giant cell mycoarditis.

## Pathophysiology

[edit | edit source]

Data from a Lewis Rat model and from observational human studies suggest that giant cell myocarditis is mediated by T lymphocytes. The disease may therefore respond to treatment aimed at attenuating T cell function.

Numerous autoimmune disorders have been associated with giant cell myocarditis in case reports, but the true magnitude of the association and causality of any association (if any) is not clear.

### Gross Pathology

[edit | edit source]

Images shown below are courtesy of Professor Peter Anderson DVM PhD and published with permission. © PEIR, University of Alabama at Birmingham, Department of Pathology

* Giant Cell Myocarditis: Gross dilated LV with focal fibrosis

* Giant Cell Myocarditis: Gross dilated LV with focal fibrosis

* Giant Cell Myocarditis: Gross dilated LV with focal fibrosis

### Microscopic Pathology

[edit | edit source]

Images shown below are courtesy of Professor Peter Anderson DVM PhD and published with permission. © PEIR, University of Alabama at Birmingham, Department of Pathology

* Boecks Sarcoid Giant Cell Myocarditis

* Boecks Sarcoid Giant Cell Myocarditis

* 22-year-old man was seropositive for HIV-1 and died suddenly. There was widespread inflammation of the myocardium with giant cells, resembling giant cell myocarditis, that may have represented a precursor to lymphoma.

* Diffuse geographic myocardial necrosis at low-power magnification. Numerous giant cells (arrows) can be identified within the inflammatory infiltrate (hematoxylin and eosin, x100)

* Multinucleated giant cells (long arrows) are seen adjacent to degenerating myocytes (short arrows). The cellular infiltrate contains lymphocytes, histiocytes, and collections of eosinophils (arrowheads) (hematoxylin and eosin, x400)

## Epidemiology and Demographics

[edit | edit source]

Giant cell myocarditis often affects young otherwise healthy individuals.

## Natural History, Complications and Prognosis

[edit | edit source]

### Complications

[edit | edit source]

Giant cell myocarditis is generally characterized by progressive congestive heart failure, and is frequently associated with refractory ventricular arrhythmias. The majority of patients die secondary to congestive heart failure, although some have survived for long periods, often after immunosuppressive treatment.[1][2]

### Prognosis

[edit | edit source]

The rate of death or heart transplantation is approximately 70% at one year. Survival following cardiac transplantation is approximately 71% at five years despite a 25% rate of giant cell infiltration in the donor heart.

## Diagnosis

[edit | edit source]

### Endomyocardial Biopsy

[edit | edit source]

The Giant Cell Myocarditis Registry is a clinical and pathologic database from 63 cases of giant cell myocarditis gathered from 36 medical centers. Using pathologic examination at transplantation or autopsy as the gold standard, the sensitivity of endomyocardial biopsy in detecting giant cell myocarditis was 80% in the GCM registry. [3]

### Electrocardiogram

[edit | edit source]

Heart block is frequently observed in giant cell myocarditis.

## Treatment

[edit | edit source]

### Immunosuppressive Therapy

[edit | edit source]

Immunosuppressive therapy including muromonab-CD3, cyclosporine and/or azathioprine, and steroids has been used to treat giant cell myocarditis. Giant Cell Myocarditis Registry subjects who received cyclosporine and/or azathioprine, with steroids and sometimes muromonab-CD3 had prolonged transplant-free survival: 12.6 months vs 3.0 months for those patients treated with no immunosuppression.[4] While this data is encouraging, it should be noted that it is drawn from a modest registry experience and randomized placebo-controlled trials are lacking.

### Cardiac Transplantation

[edit | edit source]

Giant cell myocarditis may recur after cardiac transplantation but may respond to augmented immunosuppression.

## References

[edit | edit source]

1. ↑ Desjardins V, Pelletier G, Leung TK, Waters D. Successful treatment of severe heart failure caused by idiopathic giant cell myocarditis. Can J Cardiol 1992;8:788-92
2. ↑ Ren H, Poston RS Jr, Hruban RH, Baumgartner WA, Baughman KL, Hutchins GM. Long survival with giant cell myocarditis. Mod Pathol 1993;6:402-7.
3. ↑ Cooper LT, Berry GJ, Shabetai R.Idiopathic Giant-Cell Myocarditis — Natural History and Treatment. N Engl J Med. 1997 Jun 26;336(26):1860-6
4. ↑ Cooper LT, Berry GJ, Shabetai R.Idiopathic Giant-Cell Myocarditis — Natural History and Treatment. N Engl J Med. 1997 Jun 26;336(26):1860-6

Template:WikiDoc Sources