Arrhythmogenic Left Ventricular Cardiomyopathy: A Clinical and CMR Study

Figure 1. The flowchart shows the patient selection process based on inclusion and exclusion criteria detailed in the methods section.

Figure 2. A typical case of arrhythmogenic left ventricular cardiomyopathy in a 54 year-old male patient with frequent ventricular premature beats and syncope. Four chamber view of (A) End-diastolic SSFP cine, (B) End-systolic SSFP cine, (C) Late gadolinium enhancement imaging (LGE) (D) T1 weighted image and (E) fat image from water-fat separation imaging all show fibro-fatty infiltration in the interventricular septum and the epicardial LV lateral wall. Note that the contour of the lateral LV wall is irregular with a “serrated” shape. LGE shows significantly delayed enhancement of the LV basal to mid lateral wall, interventricular septum and adjacent anterior wall. (F) Histology of endomyocardial biopsy shows areas of fibro-fatty infiltration and replacement of the myocardium.

Figure 3. A typical case of arrhythmogenic left ventricular cardiomyopathy with right ventricular involvement in a 31 year-old male with dyspnea on exertion and syncope. Four chamber SSFP cine images during (A) diastole and (B) systole show that the left ventricle is significantly dilated(LVEDD 65 mm) and there is also mild to moderate right ventricular dilation. The wall of the LV apex is thin and aneurysmal. (C) fat image from the water-fat separation imaging shows fat within the left ventricular walls. (D) 4 chamber LGE image shows significant enhancement within the LV basal to mid lateral wall, interventricular septum, and LV apex. (E,F) Histology of the explanted heart showed severe fibro-fatty infiltration and replacement of the left ventricular wall.

Figure 4. Bar graph of AHA 17-segment model demonstrating the segments with LGE and fat (Segment 17 was not analyzed).

Figure 5. Receiver operating curves showing the predictive performance of RVESVi and RVEF in the differentiation of RV involvement in patients with ALVC. The RVEF has the larger area under curve when the threshold was 44.1%, with a sensitivity of 100% and a specificity of 94.4%. ROC = receiver operating curve; RVESVi = Right ventricular end-systolic volume index, RVEF = right ventricular ejection fraction.

Figure 6. Negative linear relationship between LVEF and (A) FAT, (B) LGE, (C) FAT/LGE and (D) LVEDVI.

Figure 7. Kaplan-Meier estimates of the proportion of patients with cardiac mortality and heart transplantation free in the ALVC patients with No RV involvement(n = 36) and RV involvement (n = 17).

Figure 8. Bland and Altman Analyses of LGE and Fat Volume for intra- and inter-observer Variability. Variability of intra-observer (A) and inter-observer (B) for fat quantification, intra-observer (C) and inter-observer (D) for LGE quantification. Red dashed line indicates the mean difference and pink dashed lines indicate SD.