PMC7938323_01
PMC7938323_02
(A-C), Four-chamber transthoracic echocardiography (A) and Cardiac magnetic resonance (CMR) images (B) reveal concentric hypertrophy with moderately impaired left ventricular function and multiple cystic structures in the septal, mediolateral and anterior parts of the left ventricle. (C) Late gadolinium enhancement (LGE) demonstrated high signal intensity of the cystic lesions indicating fibrotic tissue (D-F), Short axes of the corresponding sequences: transthoracic echocardiography (D), Cine-CMR (E) and LGE (F).
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Tesla Scanner one month after transplantation ( Figure 1 ). The exam revealed concentric left ventricular (LV) hypertrophy with moderately impaired biventricular function in line with prior TTE findings.
Endomyocardial biopsy findings. (A-D), Electron microscopy: (A), intracellular presence of fatty infiltrations (black star) and degenerative products (white arrow). (B), presence of numerous interstitial collagen fibrils. (C), degenerative structures non-typical for Fabry's disease. (D), normal mitochondria. (E, F), Masson's trichome stain: (E), high extent of interstitial fibrosis (blue tissue) and abnormal fatty infiltration (arrows). (F), Cardiomyocytes show dystrophic changes (indicated myocyte diameter 11 microm).
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Ultrastructural findings revealed the presence of intracellular fat droplets, a high amount of fibrous tissue, and degenerative products ( Figure 2 ).
Cardiovascular magnetic resonance mapping of T1 (A), T2 (B) and extracellular volume (C) at 32 months post-transplant FU revealed increased values. A: T1 Mapping (1425 +- 144); B: T2 Mapping (60.5 +- 8.2); C: Extracellular volume (32.7 +- 9.6).
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However, measures of extracellular volume remain slightly elevated (ECV 33%, cutoff 30%) indicating diffuse fibrosis of the myocardium ( Figure 3 ).
Timeline of postoperative course after heart transplantation. ATG, Antithymocyte globulin; CMR, Cardiac magnetic resonance tomography; HLA-AB, Human leukocyte antigen antibodies; ICU, Intensive care unit; IgG, Immunoglobulin G; IMC, Intermediate care unit; LV, Left Ventricle; LVEF, Left ventricular ejection fraction; LVAD, Left ventricular assist device; MELAS, Mitochondrial encephalomyopathy, lactic acidosis and stroke-like episodes; MMF, Mycophenolate Mofetil; nsVT, Non sustained ventricular tachycardia: pAMR, Pathologic Antibody mediated rejection; RV, Right ventricle; RVEF, Right ventricular ejection fraction; TDI-Sm, Tissue Doppler Imaging Systolic peak velocities (radial/longitudinal); TTE, Transthoracic echocardiography.
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The described postoperative course is summarized as a Timeline in  Figure 4 .