PMC7308432_01
PMC7308432_02
(A) ECG on the day of admission showed a regular sinus rhythm, complete left bundle branch block with a QRS complex of 175 ms, first-degree atrioventricular block and bilateral atrial enlargement. (B) ECG when the proband experienced syncopal attack again showed third-degree atrioventricular block with a ventricular rate of 40 bpm. (C) ECG after LBP showed a narrowing of the QRS and complete right bundle branch block.
fped-08-00312-g0001.jpg
PMC7308432_02_fped-08-00312-g0001.jpg
F1
The electrocardiogram (ECG) showed a regular sinus rhythm, complete left bundle branch block with a QRS complex of 175 ms, first-degree atrioventricular block and bilateral atrial enlargement (see Figure 1A). ECG showed III AVB (see Figure 1B), which may account for the patient's recurrent syncope. ECG showed a narrower QRS complex (from 175to 154 ms) and complete right bundle branch block (see Figure 1C).
(A) Echocardiogram on the day of admission showed damaged systolic function, interventricular septum predominantly thickened (23 mm), biatrial enlargement (LA: 44 mm, RA: 40 mm), posterior wall of left ventricle slightly thickened (10 mm), and moderate mitral regurgitation. (B) Echocardiogram during the operation showed the right ventricular pacing electrode located at the upper interventricular septum with a depth of 14.5 mm and a distance of about 3 mm from the left ventricular endocardium.
fped-08-00312-g0002.jpg
PMC7308432_02_fped-08-00312-g0002.jpg
F2
The echocardiogram showed interventricular septum predominantly thickened (maximum: 23 mm), biatrial enlargement (LA: 44 mm, RA: 40 mm), posterior wall of left ventricle slightly thickened (10 mm), moderate mitral valve regurgitation (see Figure 2A), damaged systolic and diastolic function with left ventricular ejection fraction of 51.8%. Figure 2B). Finally, a significantly narrow QRS complex was achieved and CLBBB was reversed with threshold of 1V at 0.4 ms and sensing of 4 mV.
(A,B) Chest X-rays showed the 5,076 pacing lead located in the anterior wall of the right atrium (red arrow). The 3,830 electrode passed through the right ventricular septum and reached the sub-endocardium of the left ventricle (blue arrow).
fped-08-00312-g0003.jpg
PMC7308432_02_fped-08-00312-g0003.jpg
F3
The 5,076 pacing lead was placed on the anterior wall of the right atrium while the 3,830 pacing lead passed through the right ventricular septum and reached the sub-endocardium of the left ventricle side (see Figure 3).
Pedigree analysis of the family in the case. The arrow points out the proband. Circles stood for female. Squares stood for male. The variant MYH7 c.2155C>T is indicated +/- if heterozygous and -/- if negative.
fped-08-00312-g0004.jpg
PMC7308432_02_fped-08-00312-g0004.jpg
F4
Familial clinical evaluation showed that both her father and younger brother had HCM (see Figure 4). Genetic screening showed that the p.R719W mutation inherited from her father (see Figure 4).