Endocardial fibroelastosis

Case contributed by Rachael O'Rourke
Diagnosis almost certain


Born with interrupted aortic arch and patent ductus arteriosus with surgical repair. Now with left ventricular dilation and severe dysfunction worsening over a period of 10 years. No previous ischemic events.

Patient Data

Age: 16 years
Gender: Male

Left ventricular dilation and hypokinesia on cardiac cine imaging.

Diffuse subendocardial late gadolinium enhancement involving the lateral segments of the left ventricle including the posteromedial papillary muscle and the apical portion of the interventricular septum.

Case Discussion

Endocardial fibroelastosis is  associated with aortic coarctation and usually diagnosed in the pediatric population, but can also be seen/diagnosed in the adult population.

Patients can present with a restrictive cardiomyopathy, later progressing to a dilated cardiomyopathy, with secondary pulmonary hypertension.

Diffuse calcification within the left ventricle on a non gated CT in the setting of aortic coarctation or other forms of left heart congenital disease are common findings, with fibrosis confirmed in cardiac MRI.

The diagnosis was made in multi-discplinary team discussion, with imaging findings typical for this diagnosis. 

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