Left ventricular fibro-fatty replacement in arrhythmogenic right ventricular dysplasia/cardiomyopathy: prevalence, patterns, and association with arrhythmias

Table 3 Demographic, clinical, and CMR data for the entire cohort, comparing subjects with and without PKP2 variant

	All (n = 73)	No mutation (n = 38)	PKP2 mutation (n = 30)	Other mutations^a (n = 5)	p-value**
Demographics and clinical data
Age (years)	34.2 ± 13.5	34.8 ± 13.6	33.7 ± 14.0	33.1 ± 12.8	0.934
Male sex (%)	37 (50.7)	19 (50.0)	18 (60.0)	0 (0)	0.045
Proband (%)	41 (56.2)	25 (65.8)	13 (43.3)	3 (60.0)	0.177
VT prior to CMR	29 (39.7)	16 (42.1)	12 (40.0)	1 (20.0)	0.637
VT during follow-up	37 (50.7)	20 (52.6)	16 (53.3)	1 (20.0)	0.363
CMR findings
RVEDVI (mL/m²)	106.7 ± 31.0	111.8 ± 30.9	104.0 ± 30.7	84.8 ± 25.7	0.152
RVEF (%)	41.7 ± 10.0	41.6 ± 9.8	42.0 ± 10.7	40.0 ± 8.9	0.918
LVEDVI (mL/m²)	89.3 ± 19.8	93.2 ± 23.1	85.0 ± 14.5	87.2 ± 20.0	0.245
LVEF (%)	54.1 ± 6.6	54.7 ± 6.7	54.5 ± 5.9	47.3 ± 7.8	0.057
LV-LGE (%)	27 (37.0)	15 (39.5)	7 (23.3)	5 (100.0)	0.004
LV Fat (%)	25 (34.3)	14 (36.8)	9 (30.0)	2 (40.0)	0.808
LV-WMA (%)	9 (12.3)	3 (7.9)	2 (6.7)	4 (80.0)	< 0.001
Low LV-EF (< 50%) (%)	18 (24.7)	9 (23.7)	6 (20.0)	3 (60.0)	0.155

LV left ventricle, TFC Task Force Criteria, RV-EDVI right ventricular end-diastolic volume indexed to body surface area, RVEF right ventricular ejection fraction, LV-EDVI LV end-diastolic volume indexed to body surface area, LVEF LV ejection fraction, RV-LGE right ventricular late gadolinium enhancement, RV fat right ventricular fat, RV-WMA right ventricular wall motion abnormality, VT ventricular tachycardia
**p-value derived from ANOVA test. No significant difference was found between the No Mutation and PKP2 Mutation groups on Rank-Sum or Ficher’s Exact tests for all the above demographic and CMR variables
^aMutations include DSP, DSG2, DSC2, PLN, and compound non-PKP2 mutations. One patient had both a PKP2 and a DSP mutation and was included in PKP2 + ve group

ISSN: 1532-429X