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Flow Cytometry-Based Quantification and Analysis of Myocardial B-Cells
Published on: August 17, 2022
Myocarditis and Kawasaki disease
1Department of Cardiology, Boston Children's Hospital, Boston, MA, USA.
Insights
Kawasaki disease (KD) causes myocarditis in children, often without symptoms. Echocardiograms show cardiac dysfunction that improves with treatment, though subtle issues may remain.
Area of Science:
- Pediatric Cardiology
- Rheumatology
- Immunology
Background:
- Kawasaki disease (KD) is the leading vasculitis in children.
- Cardiovascular complications, including myocarditis and coronary artery aneurysms, are frequent in KD.
- While histological myocarditis is universal in KD, clinical symptoms are less common.
Purpose of the Study:
- To investigate the prevalence and characteristics of myocarditis in children with Kawasaki disease.
- To evaluate the impact of treatment on cardiac function in KD patients.
- To identify potential long-term cardiac sequelae of KD-associated myocarditis.
Main Methods:
- Histological examination of cardiac tissue from KD patients.
- Echocardiographic assessment of cardiac function, including systolic and diastolic parameters, strain, and dimensions.
- Comparison of echocardiographic findings and recovery between patients treated with intravenous immunoglobulin (IVIG) and aspirin versus aspirin alone.
Main Results:
- Histological evidence of myocarditis is present in all KD patients.
- Echocardiography reveals systolic dysfunction (depressed shortening fraction), increased ventricular dimensions, and diastolic dysfunction in a significant proportion of patients.
- Echocardiographic signs of myocarditis resolve more rapidly with IVIG treatment compared to aspirin alone.
- Systolic function typically normalizes long-term, but subtle abnormalities in strain and diastolic function may persist.
- Myocarditis can occur independently of coronary artery abnormalities.
Conclusions:
- Myocarditis is a universal finding in Kawasaki disease, presenting with varied clinical and echocardiographic manifestations.
- Intravenous immunoglobulin therapy accelerates the echocardiographic recovery of cardiac function in KD patients.
- While significant cardiac dysfunction often resolves, persistent subtle abnormalities underscore the potential for long-term sequelae from KD myocarditis.
Abstract:
Kawasaki disease (KD) is the most common vasculitis of childhood. Coronary artery aneurysms and myocarditis are common cardiovascular complications of KD. While evidence of myocarditis can be found in all patients with KD on histology specimens, only a minority of patients are clinically symptomatic. Occasionally children can present with KD shock syndrome and hemodynamic instability as a result of decreased systolic function and vasoplegia. Several children with KD have depressed shortening fraction on echocardiography. Increased end-systolic and end-diastolic dimensions, strain abnormalities and diastolic dysfunction are also found in a significant proportion of patients. Echocardiographic signs of myocarditis improve after the acute phase and do so more quickly in patients who have received intravenous immunoglobulins, as opposed to those given only aspirin. Normalization of systolic function is typically observed over long-term follow-up; however, more subtle abnormalities (strain, diastolic function) may persist. It is noteworthy that myocarditis associated with KD can occur in absence of coronary artery abnormalities. KD myocarditis can result in long-term sequelae.
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