Home
|
Inbox
|
Search
|
View Abstract
A Rare, Rapidly Progressive, and Fatal Myocarditis
Description
.abstract img { width:300px !important; height:auto; display:block; text-align:center; margin-top:10px } .abstract { overflow-x:scroll } .abstract table { width:100%; display:block; border:hidden; border-collapse: collapse; margin-top:10px } .abstract td, th { border-top: 1px solid #ddd; padding: 4px 8px; } .abstract tbody tr:nth-child(even) td { background-color: #efefef; } .abstract a { overflow-wrap: break-word; word-wrap: break-word; }
A3501 - A Rare, Rapidly Progressive, and Fatal Myocarditis
Author Block: G. Lin, M. Schivo; UCD Medical Center, Sacramento, CA, United States.
Introduction: First described in 1905, giant cell myocarditis is a rare and frequently fatal disease that is characterized by a mixed myocardial infiltrate with nucleated giant cells and cardiomyocyte necrosis. It is commonly diagnosed by endomyocardial biopsy, autopsy, or wedge resection during left ventricular assist device placement. Based on autopsy case series, its incidence is estimated to be approximately 23.4 per 100,000. The mean age at presentation is 42 years old but has been seen in patients between 16 to 69 years old. The mortality rate is estimated to be between 60% to 90% within the first year of diagnosis.
Case: A 62 year old Caucasian female with a recent diagnosis of myocarditis by cardiac magnetic resonance imaging four months prior, presented to the hospital for worsening chest pain, orthopnea and myalgias. Serial echocardiograms showed the patient’s ejection fraction rapidly reduced from 65% to 35% over four months. Cardiac catheterization showed coronaries without atherosclerosis, and an endomyocardial biopsy showed well-formed granulomas with giant cells, patchy eosinophilic, and mixed inflammatory cell infiltrate concerning for giant cell myocarditis. Given minimal improvement with aggressive diuresis, inotropic support and high dose steroids, she ultimately underwent Arterio-Venous Extra Corporeal Membrane Oxygenation. However, despite these interventions, the patient passed away a few days later before she could undergo a left ventricular assist device placement or heart transplant.
Discussion: Giant cell myocarditis is a rapidly progressive and frequently fatal disease that affects otherwise healthy young and middle aged individuals. Though a rare cause of myocarditis overall, giant cell myocarditis accounts for 17% of all myocarditis fatalities. The pathophysiology is not well understood, but is thought to be related to an autoimmune process involving T-lymphocyte immune dysregulation. The initial presentation is often difficult to distinguish between cardiac sarcoidosis. Most patients present with rapid onset congestive heart failure, ventricular arrhythmias, and atrioventricular blocks. Median survival is 5.5 months from the onset of symptoms. Treatments includes cyclosporine-based immunosuppression and heart transplant; though giant cell myocarditis has been shown to recur in 25% of transplanted hearts. Ultimately, early recognition of this disease is crucial as its management differs from other myocardial processes and early tailored immunosuppressive treatments and heart transplant can significantly alter patient outcomes.
Author Block: G. Lin, M. Schivo; UCD Medical Center, Sacramento, CA, United States.
Introduction: First described in 1905, giant cell myocarditis is a rare and frequently fatal disease that is characterized by a mixed myocardial infiltrate with nucleated giant cells and cardiomyocyte necrosis. It is commonly diagnosed by endomyocardial biopsy, autopsy, or wedge resection during left ventricular assist device placement. Based on autopsy case series, its incidence is estimated to be approximately 23.4 per 100,000. The mean age at presentation is 42 years old but has been seen in patients between 16 to 69 years old. The mortality rate is estimated to be between 60% to 90% within the first year of diagnosis.
Case: A 62 year old Caucasian female with a recent diagnosis of myocarditis by cardiac magnetic resonance imaging four months prior, presented to the hospital for worsening chest pain, orthopnea and myalgias. Serial echocardiograms showed the patient’s ejection fraction rapidly reduced from 65% to 35% over four months. Cardiac catheterization showed coronaries without atherosclerosis, and an endomyocardial biopsy showed well-formed granulomas with giant cells, patchy eosinophilic, and mixed inflammatory cell infiltrate concerning for giant cell myocarditis. Given minimal improvement with aggressive diuresis, inotropic support and high dose steroids, she ultimately underwent Arterio-Venous Extra Corporeal Membrane Oxygenation. However, despite these interventions, the patient passed away a few days later before she could undergo a left ventricular assist device placement or heart transplant.
Discussion: Giant cell myocarditis is a rapidly progressive and frequently fatal disease that affects otherwise healthy young and middle aged individuals. Though a rare cause of myocarditis overall, giant cell myocarditis accounts for 17% of all myocarditis fatalities. The pathophysiology is not well understood, but is thought to be related to an autoimmune process involving T-lymphocyte immune dysregulation. The initial presentation is often difficult to distinguish between cardiac sarcoidosis. Most patients present with rapid onset congestive heart failure, ventricular arrhythmias, and atrioventricular blocks. Median survival is 5.5 months from the onset of symptoms. Treatments includes cyclosporine-based immunosuppression and heart transplant; though giant cell myocarditis has been shown to recur in 25% of transplanted hearts. Ultimately, early recognition of this disease is crucial as its management differs from other myocardial processes and early tailored immunosuppressive treatments and heart transplant can significantly alter patient outcomes.
|
Home
|
Inbox
|
Search
|