SCMR 22nd Annual Scientific Sessions
Description of Clinical Presentation:
A 30-year-old female, presented to the Emergency Department (ED) with acute shortness of breath, chest pain, ankle swelling and generalized weakness. She had a prior history of ulcerative colitis but was otherwise healthy. There was no family history of cardiac disease. Laboratory tests, including TnI and serology for HIV, hepatitis and CMV were negative. There was a positive IgG test for EBV in keeping with past infection. Antinuclear antibodies (ANA) were also positive (1:80).
Diagnostic Techniques and Their Most Important Findings:
ECG demonstrated sinus tachycardia. Echocardiography revealed significant global left ventricular (LV) hypokinesis and the LV ejection fraction (EF) was estimated at 24%. The right ventricular (RV) systolic function was also moderately reduced. Coronary angiography was normal.
Cardiac magnetic resonance (CMR) was subsequently performed confirming biventricular systolic dysfunction (LVEF= 19% and RVEF= 22%) with global biventricular hypokinesis and preserved ventricular volumes. There was diffuse late-gadolinium enhancement (LGE) involving the subepicardial and subendocardial layers of the LV and the RV side of the septum and RV free wall. Two small intracavitary LV apical thrombi were seen. SAO T2-SPAIR images showed no increase in signal to suggest acute inflammation. There were pericardial and bilateral pleural effusions.
RV endomyocardial biopsy was performed which demonstrated giant cell myocarditis. The patient underwent orthotopic cardiac transplantation and pathological examination of the explanted heart confirmed areas of active and healing myocarditis containing multiple groups of giant cells. There was extensive biventricular scarring involving the LV to a greater extent than the RV, in a pattern that mirrored the distribution of LGE on MR.
Learning Points from this Case:
Giant cell myocarditis is very rare type of myocarditis, characterized by development of acute heart failure.
The pattern of enhancement is nonspecific and commonly involves multiple layers of the myocardium. The appearance on MR overlaps with other types of cardiomyopathy such as sarcoidosis, ARVC and viral myocarditis. The diagnosis is usually obtained from endomyocardial biopsy.
CMR may help direct endomyocardial biopsy as well as monitor ventricular function.