66 | Chapter 4 Each dataset was used three times as input for the parameter estimation framework described above. Inter- and intra-observer agreement, as well as the uniqueness of parameter estimation were determined using the intraclass correlation coefficient (ICC).22 To calculate this coefficient, the average estimation of patient was considered the true estimation ( ) and the errors origin from inter-observer variability , intra-observer variability , and inter-simulation variability . Yo,m,e,i =αi +ϵo,i +ϵm,i +ϵe,i The interclass correlation coefficient for each of the error origins is ICCtype = σ2 α σ2 α +σ2 ϵtype Statistical analysis and Computational Setup Continuous data were presented as mean ± SD. Normal distribution was tested using the Shapiro-Wilk test. Comparison between subgroups of continuous variables was done using a one-way analysis of variance, t-test, Kruskal-Wallis, or Mann-Whitney U test as appropriate. Bonferroni correction was used to adjust for multiple comparisons. All statistical analyses were performed in Python 3.6.4 using the packages SciPy (modified BSD License) and statsmodels (modified BSD License). The CircAdapt model was coded in C++ and compiled using MSVC 14.1. All other code was written in Python and interpreted with Python 3.6.4. The parameter estimation protocol was written for single thread computation. The estimation of subjects ran parallel on Windows 10 system (AMD Ryzen Threadripper 3970X 32-Cores 4.5GHz and 128GB RAM). RESULTS From the 87 AC mutation carriers who were evaluated, 3 subjects were excluded due to inadequate echocardiographic imaging quality and 16 were excluded due to missing CMR data. All remaining exams were obtained during sinus rhythm. The 68 AC mutation carriers included in this study (18 probands and 50 family members) had a mean age of 39 ± 17 years and 41 (60%) were female. Among the different pathogenic mutations, PKP2 was most common in this cohort (90%). A total of 20 control subjects (9 (45%) females, mean age 28 ± 6 years) were also included in the study. Using the 2010 TFC, 18 (26%) mutation carriers were classified in the subclinical stage, 15 (22%) in the electrical stage, and 35 (51%) in the structural stage. The median time between echocardiography and CMR was 26 days (interquartile range (-7, 398 days)). (Table 1) αi αi ϵo,i ϵm,i ϵe,i (9) (10)
RkJQdWJsaXNoZXIy MTk4NDMw