Schematic heart slice overview of patients with a clinical phenotype of dilated cardiomyopathy.

<p>The results of the digital quantification in heart slices in percentage of fibrosis or adipose tissue are shown using a color scale. The epicardial region has been excluded from this overview. LV, left ventricle; RV, right ventricle; Ant., anterior; Post., posterior</p

Patient characteristics of the <i>PLN</i> p.Arg14del mutation carriers.

<p>SD, standard deviation; ICD, implantable cardioverter-defibrillator; CRT-D, cardiac resynchronization therapy defibrillator.</p

Schematic overview of fibrosis and adipose tissue in heart slices of control hearts.

<p>The results of the digital quantification in percentage of fibrosis or adipose tissue are shown using a color scale. The epicardial region has been excluded from the analysis. LV, left ventricle; RV, right ventricle; Ant., anterior; Post., posterior</p

Boxplots of mean fibrosis and mean adipose tissue in the different regions.

<p>Boxplots of mean percentage of fibrosis (A) and adipose tissue (B) per condition in 8 regions (C) corrected for surface area. Outliers are represented by small circles or stars. AC, arrhythmogenic cardiomyopathy; DCM, dilated cardiomyopathy; LV, left ventricle; RV, right ventricle; Ant., anterior; Post., posterior; 1 =  LV posterolateral wall; 2  =  LV posterior wall; 3  =  interventricular septum; 4  =  LV anterior wall; 5  =  LV anterolateral wall; 6  =  LV lateral wall; 7  =  RV posterior wall; 8  =  RV anterior wall.</p

Schematic heart slice overview of patients with a clinical phenotype of arrhythmogenic cardiomyopathy.

<p>The results of the digital quantification in heart slices in percentage of fibrosis or adipose tissue are shown using a color scale. The epicardial region has been excluded from this overview. LV, left ventricle; RV, right ventricle; Ant., anterior; Post., posterior</p

Overview of methodology.

<p>LV, left ventricle; RV, right ventricle; Ant., anterior; Post., posterior. <b>A,</b> gross showing a transverse heart slice of arrhythmogenic cardiomyopathy. <b>B,</b> transverse slice dissection scheme. <b>C</b>–<b>F,</b> examples of digital slide processing. Regions of interest are shown in orange lines (defining the epicardium, compact myocardium divided by an equidistant midline and trabeculated part). <b>C,</b> slide from the left ventricle posterior wall. <b>D,</b> slide C after digital processing. Red: cardiomyocytes. Blue: connective tissue. Pseudo green: adipose tissue. <b>E,</b> slide from the right ventricle lateral wall. <b>F,</b> slide E after digital processing. Red: cardiomyocytes. Blue: connective tissue. Pseudo green: adipose tissue.</p

QPCR.

<p>A. QPCR shows presence of genomic pig DNA (Gapdh) in all pig tissues treated with CMPC or placebo and absence of pig DNA in the sample containing human CMPCs (Human) and the negative control (Neg) containing H2O used for PCR. B. Human genomic DNA (HLA-DMA) was detected in the sample containing human CMPCs (Human), but was absent in all the pig tissues (CMPC and Placebo) and the negative control (Neg) containing H2O used for PCR</p

Fibrosis.

<p>Representative picture of a picrosirius red stained borderzone slide (A. bright field, B. polarized light) and a higher magnification of a picrosirius red stained slides isolated from the infarct area (C,D), borderzone (E,F) and remote area (G,H) of the left ventricle (bright field C,E,G, polarized light (D,F,H).</p

Vascular density.

<p>Respresentative picutures of Lectin staining of the borderzone of a CMCP treated animals (A) and a placebo animal (B)</p

Functional outcome measurements by PV loop.

<p>Mean and SD for PVloop parameters. EDV End diastolic volume, ESV End systolic volume, EF Left ventricular ejection fraction, ESPVR End systolic pressures volume relationship, EDPVR end diastolic pressure volume relationship. Placebo n = 8, cell n = 7 for ESV, EDV and EF, n = 6 per group for ESPVR, EDPVR and V0.</p