Successful Treatment of an Infant with Left Ventricular Noncompaction Presenting with Fatal Ventricular Arrhythmia Treated with Cardiac Resynchronization Therapy and an Implantable Cardioverter Defibrillator

Figures

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  Figure 1. Electrocardiogram (ECG) and arterial blood pressure monitoring on admission (a). (Top panel) ECG shows polymorphic ventricular tachycardia, with a rate of approximately 300 bpm. (Bottom panel) arterial blood pressure monitoring. (b) Lead II of the ECG after polymorphic ventricular tachycardia was stopped. The heart rate (HR) was 79 bpm, PR interval was 160 msec, and QTc interval was 510 msec with alternate T waves. (c) Chest X-ray on admission showing a cardiothoracic ratio of 52%. Transthoracic echocardiography on admission. (d) Transthoracic echocardiography shows left ventricular noncompaction on the parasternal short axis view, with a ratio of noncompacted to compacted area at end-systole > 2. (e) Color Doppler imaging reveals communication between myocardial recesses and the left ventricular cavity. (f) The 12-lead ECG before implantable cardioverter defibrillator (ICD) implantation, showing 2 : 1 atrioventricular block. The HR was 76 bpm, PR interval was 170 msec, and QTc interval was 462 msec. P indicates the P wave in lead II.

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  Figure 2. Chest X-rays pre- and postcardiac resynchronization therapy (CRT). (a) The defibrillator was placed into the left upper abdomen, and the shock lead was fixed at the level of the superior vena cava. The right ventricular (RV) epicardial lead was placed at the RV free wall. (b) The left ventricular (LV) lead was placed at the LV lateral wall. The 12-lead ECG pre- and post-CRT. Before CRT (c), the QRS duration was 114 msec. After CRT (d), the QRS duration was shortened to 80 msec. Speckle-tracking radial dyssynchrony pre- and post-CRT (Artida™; Toshiba Medical Systems, Tokyo, Japan). Pre-CRT (e), dyssynchrony is shown as a time difference (arrow) between time to peak strain in the anterior wall (yellow) and septum peak strain (purple) (dual chamber (DDD) 90–150 bpm with an atrioventricular (AV) delay of 130 msec). Post-CRT (C-II), dyssynchrony disappeared (DDD 80–160 bpm, with an AV delay of 120 msec and a LV-RV delay of 0 msec).