Multimodality imaging of a rare case of cardiac lipomatosis

Abstract

A 39-year-old female from the Philippines was referred to our cardiology service following work-up by her local medical practitioner for atypical chest pain. Computed tomography coronary angiogram (CTCA) was negative for coronary artery disease but suspicious for an infiltrative cardiomyopathy, with increased left ventricular wall thickness and myocardial hypoattenuation streaks of −25HU (Panels A and B). Transthoracic echocardiography (TTE) revealed an increased basal biventricular wall thickness (RV-free wall 10 mm) with hyper-echogenicity (Panels C and D). Cardiovascular magnetic resonance (CMR) steady-state-free precession cine images confirmed increased basal biventricular wall thickness with inhomogeneous signal intensity (Supplementary data, Video). Black blood T2-weighted images showed hyperintense signal intensity along the RV and LV epicardial layer, and within the interventricular septal myocardium (Panels E and F). After application of a fat-saturation pulse, these same segments were hypointense compared with the normal myocardium (Panels G and H). Late gadolinium enhancement (LGE) imaging revealed increased signal intensity (Panel I), while post-contrast T1-mapping revealed low T1 values in the corresponding epicardial and mid-septal areas (Panel J), supporting the diagnosis of myocardial fatty infiltration and cardiac lipomatosis.