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MAIN TOPIC REVIEWS June Huh, MD Department of Pediatrics, Cardiac and Vascular Center, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea Arrhythmias in postoperative congenital heart diseses ABSTRACT Congenital heart diseases (CHD) are commonly associated with early- and late-onset arrhythmias after hemodynamic and/or anatomical surgical treatment. In order to diagnose postoperative arrhythmias in CHD, an understanding of the complex anatomy, predisposing factors and hemodynamic abnormalities is reguired. Substrates for arrhythmias in postoperative arrhythmias are related to hemodynamic abnormalities and anatomical lesions caused by surgery as well as by underlying congenital cardiac defects. According to the cardiac conduction components, postoperative arrhythmias in CHD can be classified into five groups; arrhythmias related to sinus node function, related to atrioventricular node function, arrhythmias related to intraatrial conduction, arrhythmias arrhythmias related to ventricular conduction abnormalities, mixed. The management of arrhythmias in CHD should be integrated with the care of the underlying heart defects and hemodynamic abnormalities. Key words: congenital heart defects cardiac arrhythmia Received: August 30, 2010 Revision Received: November 11, 2010 Accepted: Correspondence: June Huh, MD, Division of Pediatric Cardiology, Department of Pediatrics,Grown-up Congenital Heart Clinic, Cardiac and Vascular Center, Samsung Medical Center, Sungkyunkwan University School of Medicine, 50 Irwon-dong, Gangnam-gu, Seoul 135-710, Korea Tel: 82-2-3410-3539, Fax: 82-2-3410-0043 E-mail: [email protected], or [email protected] 34 Journal of Cardiac Arrhythmia surgery risk factors VOL.11 NO.4 35 MAIN TOPIC REVIEWS MAIN TOPIC REVIEWS 36 Journal of Cardiac Arrhythmia MAIN TOPIC REVIEWS > Symptomatic supraventricular arrhythmias in congenital heart disease Medication 1) Unrepaired CHD Not need repir ASD small 2) Unrepaired CHD Need repair ASD large Ebstein A 3) Surgical atrial scar (+) Any CHD 4) Accessory pathyway twin AV node SAN dysfunction 5) Correctable hemodynamic factor (+) Ebstein A cc-TGA Isomerism Device closure EPS±RFCA EPS± RFCA± PPM Surgery±concomitant arrhythmia op. ±PPM if need AVV Dysfunction Figure 1. Approach to symptomatic supraventricular arrhythmias in congenital heart disease. AV; atrioventricular, AVV; atrioventricular valves, cc-TGA; congenitally corrected TGA, CHD; congenital heart disease, Ebstein A; Ebstein's anomaly, EPS; electrophysiology study, op; operation, PPM; permanent pacemaker, RFCA; radiofrequency catheter ablation, SAN; sinoatrial node VOL.11 NO.4 37 MAIN TOPIC REVIEWS Symptomatic ventricular arrhythmias in congenital heart disease VT from pulmonary ventricle VT from systemic ventricle or single ventricle - Wide QRS >180 ms - Severe dilated RV - Ventricular scar (+) - RV dysfunction - Ventricle function - Ventricular scar (+) EPS/RFCA - Fragmented QRS - Hemodynamics of VT High LVEDP VT induction (+) Nonsustained VT Prior to surgery for PVR Stable VT Unstable VT Activation mapping Entrainment Pace mapping 3D mapping Successful RFCA? ICD? CRT? Surgery? Figure 2. Approach to symptomatic ventricular arrhythmias in congenital heart disease. ICD; implantable cardioverter defibrillator, LVEDP; left ventricle end-diastolic preesure, PVR; pulmonary valve replacement, RFCA; radiofrequency catheter ablation, RV; right ventricle, VT; ventricular tachycardia Figure 3. MRI showed dilatation RV and RA. RA; right atrium, RV; right ventricle 38 Journal of Cardiac Arrhythmia MAIN TOPIC REVIEWS Figure 4. ECG showed atrial flutter with RBB QRS morphology. ECG; electrocardiogram, RBB; right bundle branch Figure 5. Isthmus dependent atrial flutter was noted during EPS. EPS; electro-physiological study VOL.11 NO.4 39 MAIN TOPIC REVIEWS Figure 6. Ventricular tachycardia was induced with LBBB morphology. LBBB; left bundle branch block Figure 7. 3D mapping confirmed that ventricular tachycardia had macroreentry around RVOT scar. RVOT; right ventricular outflow tract 40 Journal of Cardiac Arrhythmia 1. van der Velde ET, Vriend JW, Mannens MM, Uiterwaal CS, Brand R,Mulder BJ. 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