PMC3960282_01
12-lead ECG showing sinus-rhythm with premature-atrial and ventricular contractions and high QRS voltages consistent with left-ventricular-hypertrophy.
nihms559902f1.jpg
PMC3960282_01_nihms559902f1.jpg
F1
ECG revealed sinus-rhythm with premature-atrial and ventricular contractions and high QRS voltages consistent with left-ventricular-hypertrophy (LVH) (Figure 1). Echocardiography showed asymmetric-septal-hypertrophy but image quality was very limited due to poor acoustic windows.
(A) Short-axis cine image showing asymmetric-septal-hypertrophy [2.3cm septum (white arrows) vs 1.0cm inferolateral wall] with a mildly enlarged left ventricular cavity. (B) 4-chamber cine image showing asymmetric-septal-hypertrophy [2.3cm septum (white arrows) vs 1.0cm inferolateral wall] with a mildly enlarged left ventricular cavity. (C) Short-axis image showing marked mid-myocardial hyperenhancement of the interventricular-septum. (D) 4-chamber image showing marked mid-myocardial hyperenhancement of the interventricular-septum.
nihms559902f2.jpg
PMC3960282_01_nihms559902f2.jpg
F2
This revealed asymmetric-septal-hypertrophy (2.3cm septum vs 1.0cm inferolateral wall) (White-arrow Figures 2A&B) with a mildly enlarged left ventricular cavity and an ejection fraction of 42% (Online Videos 1&2). Late Gadolinium Enhancement imaging showed marked mid-myocardial hyperenhancement of the interventricular-septum (Figures 2C&D).
(A) Endomyocardial biopsy showing amyloid deposition on Hematoxylin-and-Eosin staining. (B) Endomyocardial biopsy showing amyloid deposition on Congo-red staining.
nihms559902f3.jpg
PMC3960282_01_nihms559902f3.jpg
F3
This showed amyloid deposition on Hematoxylin-and-Eosin (Figure 3A) as well as Congo-red (Figure 3B) staining confirming the diagnosis of cardiac amyloid.