PMC9163303_01
Electrocardiogram (ECG) of patient 1. (A) ECG before operation showing sinus rhythm and 74 ms of PR interval. Delta-wave (black arrowhead) was present in II and III leads. RV5 > 2.5 mV and RV5 + SVl > 4.0 mV in precordial leads. RI > 1.5 mv, RaVL > 1.2 mv, RaVF > 2.0 mv with secondary ST-T changes (red arrow). (B) Narrow QRS tachycardia indicating atrioventricular reentrant tachycardia (AVRT). (C) ECG after operation showing sinus rhythm and 144 ms of PR interval. No deltawave presented, but the hypertrophic remodeling of left ventricle still existed.
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F1
The narrow QRS tachycardia indicated atrioventricular reentrant tachycardia (Figures 1A,B). After the treatment, the pre-excitations in the ECG disappeared and the PR interval was restored, but the hypertrophic remodeling of the left ventricle remained (Figure 1C).
Gene sequence of the III4 (Pt 1, subject), II3 (subject's father) and II4 (Pt 2, subject's mother).
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F2
Echocardiography detected hypertrophy in the ventricular septum and left ventricular wall (Supplementary Figure 2). A genetic test revealed a hemizygous mutation in the LAMP2 gene (LAMP2: c.963G > A: Figure 2).
ECG of patient 2. (A) The earliest ECG of patient showing sinus rhythm and complete left bundle branch block (CLBBB) with the width of QRS wave of 150 ms. (B) Sinus rhythm, CLBBB and frequent ventricular premature beat. (C) Atrial fibrillation and CLBBB. (D) Recovered sinus rhythm after radiofrequency ablation. (E) The pre-operative ECG showing sinus rhythm, CLBBB and 220 ms of QRS wave width. (F) Post-operative ECG showing sinus rhythm and 190 ms of QRS wave width.
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F3
A diffused thickness of the left ventricle and multiple patchy delayed enhancements were observed on the magnetic resonance imaging (MRI, Supplementary Figure 3).