Figure 2.

Cardiac Fibrosis in PPA2 Deficiency

(A) Affected individual P3, post mortem section through left ventricle showing a virtually circumferential lamina of scarring in midmyocardium with focal subendocardial involvement. Fibrosis is marked by arrows.

(B and C) Low-power (B) (bar equals 1 mm) and high-power (C) (bar equals 25 μm) microscopy of the posterior freewall of the left ventricle showing prominent midmyocardial loose fibrosis in P3 (hematoxylin and eosin staining).

(D) Cardiac MRI showing prominent midmyocardial fibrosis in affected individual P4 (at 25 years of age), marked by arrows.