Congestive Heart Failure as Initial Manifestation of Biventricular Arrhythmogenic Cardiomyopathy With MYBPC3 and DSG2 Variants

Figures

• 

  Figure 1. Initial Evaluation (A) Electrocardiography demonstrated sinus tachycardia, biatrial enlargement, and premature ventricular complexes, (B) Transthoracic echocardiography parasternal long-axis view showing chamber dilation and septal thickness of 9 mm (asterisk). Ao = aorta; LA = left atrium; LV = left ventricle; RV = right ventricle.

• 

  Figure 2. Results of 24-Hour Holter Monitoring (A) Maximum heart rate, (B) minimum heart rate, (C) ventricular triplets, (D) ventricular couplets, and (E) ventricular bigeminy.

• 

  Figure 3. Cardiac Magnetic Resonance at Admission (A) Cine 4-chamber view showing global chamber dilation and biventricular wall microaneurysms. (B) Native T1 mapping with elevated values (1,090 ms). (C) T2 mapping within normal limits (48 ms). (D) First-pass perfusion without perfusion defects. (E) Late gadolinium enhancement demonstrating midwall fibrosis in the inferior and lateral left ventricular walls, with extension to the right ventricle.

• 

  Figure 4. Follow-Up Cardiac Magnetic Resonance Cine images demonstrating biventricular microaneurysms: (A) 4-chamber view in diastole, (B) 4-chamber view in systole, (C) 3-chamber view, (D) right ventricular outflow tract view, and (E) 2-chamber view. (F) Native T1 mapping within normal range (943 ms), (G) postcontrast T1 mapping with an extracellular volume of 33%, (H) T2 mapping within normal range (42 ms), (I) T2∗ mapping within normal range (29 ms), (J) late gadolinium enhancement showing midwall fibrosis in the inferior and lateral left ventricular walls, with extension into the right ventricle.