Fig. 29.1
Frontal chest radiograph (left) in a previously healthy infant shows pulmonary edema with a moderate right pleural effusion and mild cardiomegaly. Post mortem cardiac dissection of the left ventricle (right) shows thick, fibrotic endocardial lining consistent with endocardial fibroelastosis
29.2.2 Echocardiographic Findings
Decreased ejection fraction
Increased echogenicity along the endocardium of the left ventricle
Variable mitral regurgitation
29.2.3 MRI Findings
Steady-state free precession (SSFP) cine MRI
Assess ventricular filling dysfunction
Decreased E/A ratio—a measurement of transmitral flow, comparing peak early diastole flow (E measurement) with the flow during atrial contraction (A)
Most ventricular filling occurs in late diastole because of impaired ventricular relaxation during early diastole
Delayed gadolinium enhancement
Enhancement of the endocardium from fibrosis
29.2.4 Cardiac Catheterization
Subendomyocardial biopsy can be performed to help differentiate etiology.
29.2.5 Imaging Recommendations
Echocardiography used for evaluation of ventricular dysfunction
Cardiac MR is the optimal imaging modality, accurately evaluating cardiac chamber size, diastolic dysfunction, the presence of myocardial scarring or fibrosis, and differentiation from other causes of cardiomyopathy
Subendomyocardial biopsy to differentiate etiology can be performed with cardiac catheterization
29.3 Differential Diagnosis
Constrictive pericarditis
May also have decreased ventricle filling and diastolic volume
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