A Case Series on Cardiac and Skeletal Involvement in Two Families with PRKAG2 Mutations

Figure 4

Short-axis and four chamber cine images from the initial and latest cardiovascular magnetic resonance scans of (a, b) II:1 and (c, d) II:2 from family B. Top row for each set of four images shows cine images, and the bottom row shows late gadolinium enhancement images. (a) The initial study showed asymmetric left ventricular hypertrophy (white arrowheads) with a maximum wall thickness of 15 mm, and no areas of late gadolinium enhancement were present. (b) On the latest follow-up scan, there is marked left ventricular hypertrophy (42 mm; dotted arrows) and patchy midwall v along with regions of subepicardial enhancement (white arrows). (c, d) Mild asymmetric left ventricular hypertrophy on the initial scan and the latest scan (15–17 mm). No regions of late gadolinium enhancement are present.