PMC9897811_01
Electrocardiogram showing sinus rhythm (51/min), first degree atrioventricular block (PR interval of 218ms), and left bundle branch block.
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FIG1
At presentation, his electrocardiogram (ECG) showed first-degree atrioventricular block and left bundle branch block (Figure 1).
Echocardiography showing grade III diastolic dysfunction with an E/A ratio superior to 2.. B = E wave; A = A wave
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FIG2
Transthoracic echocardiogram (TEE) revealed the presence of a restrictive/infiltrative cardiomyopathy, showing grade III diastolic dysfunction (Figure 2), concentric severe left ventricular hypertrophy and right ventricular hypertrophy, severe right and left atrial enlargement, thickened heart valves (Figure 3) and interatrial septum, moderate tricuspid regurgitation estimating a pulmonary artery systolic pressure of 57mmHg, moderately reduced left ventricular ejection fraction (38%) and mild pericardial effusion close the right atrium.
Parasternal long-axis view echocardiography revealing severe concentric left ventricular hypertrophy (posterior wall 19 mm), thickened interventricular septum (interventricular septum 17 mm), and atrioventricular valves. . A = interventricular septum; B = posterior wall
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FIG3
Transthoracic echocardiogram (TEE) revealed the presence of a restrictive/infiltrative cardiomyopathy, showing grade III diastolic dysfunction (Figure 2), concentric severe left ventricular hypertrophy and right ventricular hypertrophy, severe right and left atrial enlargement, thickened heart valves (Figure 3) and interatrial septum, moderate tricuspid regurgitation estimating a pulmonary artery systolic pressure of 57mmHg, moderately reduced left ventricular ejection fraction (38%) and mild pericardial effusion close the right atrium.
Bull's eye graph of the left ventricle displacing the magnitude and homogeneity of longitudinal strain in color code map. It shows relative preservation of longitudinal strain at the apex resulting in a "cherry on top"/"apical sparing" pattern.
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FIG4
Scintigraphy with 99m-Technetium labeled 3,3-diphosphono-1,2-propano-dicarboxylic acid (DPD) showing myocardial uptake greater than bone with reduced/absent bone uptake - Perugini grade 3.
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FIG5
Radionuclide scintigraphy with 99m-Technetium labeled 3,3-diphosphono-1,2-propano-dicarboxylic acid (DPD) revealed a myocardial uptake greater than bone with reduced bone uptake - grade 3 (Figure 5).