PMC8728857_01
Transthoracic echocardiography (parasternal long-axis view) shows LV hypertrophy and the involvement of the mitral leaflets (arrow) and the subvalvular apparatus by deposits in a patient with mucopolysaccharidosis syndrome type I-S.
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F1
Both mitral leaflets were very thick (Figure 1) with severe stenosis (mean diastolic gradient = 7 mmHg and peak diastolic gradient =17 mmHg) (Figure 2), and there was mild regurgitation.
Transthoracic echocardiography (apical 4-chamber view) by continuous-wave Doppler shows a mean mitral valve gradient of 7 mmHg and a mitral valve area of 1.3 cm2 via the pressure half-time method.
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F2
Both mitral leaflets were very thick (Figure 1) with severe stenosis (mean diastolic gradient = 7 mmHg and peak diastolic gradient =17 mmHg) (Figure 2), and there was mild regurgitation.
Transthoracic echocardiography (short-axis view) shows an MVA of 0.9 cm2 (arrow) via the planimetry method.
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F3
The mitral valve area was 0.9 cm2 via planimetry (Figure 3) with a score of 10 to 11.
Transthoracic echocardiography (apical 5-chamber view) by continuous-wave Doppler shows a peak aortic valve gradient of 152 mmHg.
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F4
The aortic valve was severely thick and stenotic (peak systolic gradient = 152 mmHg and mean systolic gradient = 85 mmHg) (Figure 4) with moderately severe regurgitation.
Transesophageal echocardiography (short-axis view) shows an aortic valve area of 0.7 cm2 (arrow) via the direct planimetry method.
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F5
According to 2D transesophageal echocardiography, the aortic valve area was 0.8 cm2 via the continuity equation method and 0.7 cm2 via the direct planimetry method (Figure 5).