Arrhythmogenic Right Ventricular Cardiomyopathy With Left Ventricular Noncompaction: A Phenotypic Overlap With High Arrhythmic Risk

Figures

• 

  Figure 1. Electrocardiogram. 12-lead electrocardiogram showing sinus rhythm, with T-wave inversion in the right precordial leads (V1–V3) (white box), suggestive of right ventricular (RV) involvement in arrhythmogenic cardiomyopathy.

• 

  Figure 2. Left ventricular noncompaction (CMR). CMR steady-state free precession (SSFP) cine imaging demonstrating the left ventricle (LV) with a significant increase in trabeculations (white arrow). A: Long axis, 4-chamber view; B: Long axis, 3-chamber view. The apex (black arrow) has a curve that points to the right, with a course anterior to the apex of the right ventricle (RV). The left ventricular outflow tract (LVOT) is preserved as well as the aorta (Ao). Furthermore, the apical septal segment (segment 8) and the apex (segment 9) are not compacted (shown in A). LA = left atrium.

• 

  Figure 3. Right ventricular structural abnormalities (CMR). CMR, SSFP sequence. A: Right 2-chamber long-axis view; B: Short-axis view. The right ventricle (RV) demonstrates severe structural remodeling with irregular, trabeculated walls and bilobed sacculations involving the free wall and right ventricular outflow tract (RVOT). Marked right atrial (RA) enlargement and mild pericardial effusion (∗) are also observed. MPA = main pulmonary artery.

• 

  Figure 4. Pericardial effusion and epicardial fat (CMR). A: Axial SSFP sequence; Dixon technique, axial view: water (B) and fat (C) series. The pericardial space exhibits pericardial effusion (shown as ∗), with an estimated separation of pericardial layers of 18 mm, without repercussions on the atria. Likewise, significant lipomatosis of the epicardial fat is observed (shown as ✦), predominantly around the left ventricle, with a maximum thickness of 20.7 mm.

• 

  Figure 5. Cardiac magnetic resonance, late gadolinium enhancement. T1-weighted image, late gadolinium enhancement (LGE). A: axial view; B: short axis, two-chamber view. Late intramyocardial septal enhancement is observed in the basal inferoseptal (segment 3), mid inferoseptal (segment 10), and mid inferior (segment 11). No late enhancement was evident in the right ventricle. ✦: Lipomatosis of epicardial fat.

• 

  Figure 6. Cardiac magnetic resonance, Venous structures and angiography. A: SSFP sequence, axial view; B: Tridimensional Contrast-Enhanced Magnetic Resonance Angiography (CE-MRA) sequence, coronal view. This figure demonstrates a moderate enlargement of the inferior vena cava (IVC) shortly before its union with the right atrium (RA). The superior vena cava (SVC) diameter is unremarkable. LV = left ventricle; Ao = Aortic arch; RPA = Right pulmonary artery; ✦ = Lipomatosis of epicardial fat; ∗ = pericardial effusion.