PMC9879840_01
Pedigree chart depicting family history of EMD gene-associated in an X-linked recessive mode of inheritance in (A) proband 1 and 2, (B) proband 3, and (C) proband 4. Proband 3 refused to share family history regarding his children.
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PMC9879840_04_ytad013f1.jpg
ytad013-F1
An 18-year-old male XLR-EDMD patient referred to the NMMD Clinic presenting with leg and ankle weakness, scoliosis, and a history of falls (Figure 1C).
Electrocardiograms from (A) patient 1 depicting atrial fibrillation with ventricular-paced rhythm at 60 bpm, and (B) Patient 4 depicting junction escape rhythm at the rate of 50 bpm with normal axis and no hypertrophy.
ytad013f2.jpg
PMC9879840_04_ytad013f2.jpg
ytad013-F2
Pre-device ECG showed a junctional escape rhythm with normal QRS duration of 94 ms, and a prolonged QTc interval of 443 ms (Figure 2B).
(A) Cardiac monitoring and management strategies for X-linked EDMD patients (B) illustrated by 12-lead electrocardiogram (QRS duration, qtc interval) and echocardiogram (LVEF, LVIDd, LVIDs, LVMI, LAVI) parameters in patients 1-4 to track disease progression from baseline to median 3-year first follow-up and 5-year second follow-up.
ytad013f3.jpg
PMC9879840_04_ytad013f3.jpg
ytad013-F3
Ventricular dimension remained stable (LVIDd = 56-47 mm; LVIDs = 35-25 mm), and systolic function remained preserved within normal limits (LVEF = 60-70%) (Figure 3B).
PMC9879840_02
Pedigree chart depicting family history of EMD gene-associated in an X-linked recessive mode of inheritance in (A) proband 1 and 2, (B) proband 3, and (C) proband 4. Proband 3 refused to share family history regarding his children.
ytad013f1.jpg
PMC9879840_04_ytad013f1.jpg
ytad013-F1
An 18-year-old male XLR-EDMD patient referred to the NMMD Clinic presenting with leg and ankle weakness, scoliosis, and a history of falls (Figure 1C).
(A) Cardiac monitoring and management strategies for X-linked EDMD patients (B) illustrated by 12-lead electrocardiogram (QRS duration, qtc interval) and echocardiogram (LVEF, LVIDd, LVIDs, LVMI, LAVI) parameters in patients 1-4 to track disease progression from baseline to median 3-year first follow-up and 5-year second follow-up.
ytad013f3.jpg
PMC9879840_04_ytad013f3.jpg
ytad013-F3
Ventricular dimension remained stable (LVIDd = 56-47 mm; LVIDs = 35-25 mm), and systolic function remained preserved within normal limits (LVEF = 60-70%) (Figure 3B).
PMC9879840_03
Pedigree chart depicting family history of EMD gene-associated in an X-linked recessive mode of inheritance in (A) proband 1 and 2, (B) proband 3, and (C) proband 4. Proband 3 refused to share family history regarding his children.
ytad013f1.jpg
PMC9879840_04_ytad013f1.jpg
ytad013-F1
An 18-year-old male XLR-EDMD patient referred to the NMMD Clinic presenting with leg and ankle weakness, scoliosis, and a history of falls (Figure 1C).
(A) Cardiac monitoring and management strategies for X-linked EDMD patients (B) illustrated by 12-lead electrocardiogram (QRS duration, qtc interval) and echocardiogram (LVEF, LVIDd, LVIDs, LVMI, LAVI) parameters in patients 1-4 to track disease progression from baseline to median 3-year first follow-up and 5-year second follow-up.
ytad013f3.jpg
PMC9879840_04_ytad013f3.jpg
ytad013-F3
Ventricular dimension remained stable (LVIDd = 56-47 mm; LVIDs = 35-25 mm), and systolic function remained preserved within normal limits (LVEF = 60-70%) (Figure 3B).
PMC9879840_04
Pedigree chart depicting family history of EMD gene-associated in an X-linked recessive mode of inheritance in (A) proband 1 and 2, (B) proband 3, and (C) proband 4. Proband 3 refused to share family history regarding his children.
ytad013f1.jpg
PMC9879840_04_ytad013f1.jpg
ytad013-F1
An 18-year-old male XLR-EDMD patient referred to the NMMD Clinic presenting with leg and ankle weakness, scoliosis, and a history of falls (Figure 1C).
Electrocardiograms from (A) patient 1 depicting atrial fibrillation with ventricular-paced rhythm at 60 bpm, and (B) Patient 4 depicting junction escape rhythm at the rate of 50 bpm with normal axis and no hypertrophy.
ytad013f2.jpg
PMC9879840_04_ytad013f2.jpg
ytad013-F2
Pre-device ECG showed a junctional escape rhythm with normal QRS duration of 94 ms, and a prolonged QTc interval of 443 ms (Figure 2B).
(A) Cardiac monitoring and management strategies for X-linked EDMD patients (B) illustrated by 12-lead electrocardiogram (QRS duration, qtc interval) and echocardiogram (LVEF, LVIDd, LVIDs, LVMI, LAVI) parameters in patients 1-4 to track disease progression from baseline to median 3-year first follow-up and 5-year second follow-up.
ytad013f3.jpg
PMC9879840_04_ytad013f3.jpg
ytad013-F3
Ventricular dimension remained stable (LVIDd = 56-47 mm; LVIDs = 35-25 mm), and systolic function remained preserved within normal limits (LVEF = 60-70%) (Figure 3B).