title: Role of right ventricular three-dimensional electroanatomic voltage mapping for arrhythmic risk stratification of patients with corrected tetralogy of Fallot or other congenital heart disease involving the right ventricular outflow tract.
abstract: BACKGROUND
  The post-surgical history of repaired congenital heart disease (rCHD), in particular tetralogy of Fallot (TOF), is often complicated by sudden death. Electrical myocardial abnormalities could be a substrate for malignant ventricular arrhythmias.
  
  
  RESULTS
  146 patients with TOF or other rCHD involving a subpulmonary right ventricle, considered to be at high arrhythmic risk, underwent right ventricular (RV) electroanatomic voltage mapping (EVM). Maps showed endocardial scars (<0.5mV) in all cases, mainly involving the RV outflow tract (n=141, 96.6%). In 28 cases (19.2%), other areas were involved. Total scar extension, expressed as % of total endocardial area, was significantly higher in patients with QRS ≥180ms [4.5% (±2.5) vs 2.8% (±2.4), p=0.014], left and right ventricular systolic dysfunction [4.5% (±3.2) vs 2.8% (±2.3), p=0.016 and 3.5% (±3.0) vs 2.6% (±1.9), p=0.03, respectively], premature ventricular contractions (PVCs) [3.2% (±2.6) vs 2.2% (±1.8), p<0.05], exercise-induced PVCs [3.8% (±2.4) vs 2.6% (±2.2), p=0.01], previous shunt [4.0% (±2.7) vs 2.6% (±2.2), p=0.01] and reintervention [4.2% (±3.2) vs 2.6% (±2.0), p=0.008]. Scar size also showed a positive correlation with duration of post-surgical follow-up (ρ=0.01), age at correction (ρ=0.01) and absolute QRS duration (ρ=0.05).
  
  
  CONCLUSIONS
  Patients with rCHD involving the right ventricle show electrical scars with variable distribution, not necessarily matching with sites of surgical lesions. Scar extension correlates with some of the risk factors for life-threatening arrhythmias in CHD, such as prolonged QRS. Thus EVM could be considered an additional tool in the assessment of risk stratification in this particular population.
authors: Pediatric Cardiology and Cardiac Arrhythmia/Syncope Unit, Department of Pediatric Cardiology and Cardiac Surgery, Bambino Gesù Children's Hospital and Research Institute, Rome, Italy. Electronic address: fabrizio.drago@opbg.net.
authors: Fabrizio
authors: F
authors: Drago
authors: Pediatric Cardiology and Cardiac Arrhythmia/Syncope Unit, Department of Pediatric Cardiology and Cardiac Surgery, Bambino Gesù Children's Hospital and Research Institute, Rome, Italy.
authors: Vincenzo
authors: V
authors: Pazzano
authors: Pediatric Cardiology and Cardiac Arrhythmia/Syncope Unit, Department of Pediatric Cardiology and Cardiac Surgery, Bambino Gesù Children's Hospital and Research Institute, Rome, Italy.
authors: Corrado
authors: C
authors: Di Mambro
authors: Pediatric Cardiology and Cardiac Arrhythmia/Syncope Unit, Department of Pediatric Cardiology and Cardiac Surgery, Bambino Gesù Children's Hospital and Research Institute, Rome, Italy.
authors: Mario Salvatore
authors: MS
authors: Russo
authors: Pediatric Cardiology and Cardiac Arrhythmia/Syncope Unit, Department of Pediatric Cardiology and Cardiac Surgery, Bambino Gesù Children's Hospital and Research Institute, Rome, Italy.
authors: Rosalinda
authors: R
authors: Palmieri
authors: Pediatric Cardiology and Cardiac Arrhythmia/Syncope Unit, Department of Pediatric Cardiology and Cardiac Surgery, Bambino Gesù Children's Hospital and Research Institute, Rome, Italy.
authors: Massimo Stefano
authors: MS
authors: Silvetti
authors: Pediatric Cardiology and Cardiac Arrhythmia/Syncope Unit, Department of Pediatric Cardiology and Cardiac Surgery, Bambino Gesù Children's Hospital and Research Institute, Rome, Italy.
authors: Salvatore
authors: S
authors: Giannico
authors: Pediatric Cardiology and Cardiac Arrhythmia/Syncope Unit, Department of Pediatric Cardiology and Cardiac Surgery, Bambino Gesù Children's Hospital and Research Institute, Rome, Italy.
authors: Benedetta
authors: B
authors: Leonardi
authors: ECMO Functional Unit, Department of Pediatric Cardiology, Bambino Gesù Children's Hospital and Research Institute, Rome, Italy.
authors: Antonio
authors: A
authors: Amodeo
authors: Unit of Epidemiology, Department of Health, Bambino Gesù Children's Hospital and Research Institute, Rome, Italy.
authors: Vincenzo Maria
authors: VM
authors: Di Ciommo
mesh_terms: D000293:Adolescent; D000328:Adult; D018780:Body Surface Potential Mapping / Q000379:methods*; D002648:Child; D005260:Female; D006329:Heart Conduction System / Q000000981:diagnostic imaging / Q000503:physiopathology*; D006330:Heart Defects, Congenital / Q000175:diagnosis / Q000503:physiopathology; D006801:Humans; D021621:Imaging, Three-Dimensional / Q000379:methods*; D008297:Male; D018570:Risk Assessment; D013771:Tetralogy of Fallot / Q000175:diagnosis / Q000503:physiopathology*; D018497:Ventricular Dysfunction, Right / Q000175:diagnosis / Q000503:physiopathology*; D014694:Ventricular Outflow Obstruction / Q000175:diagnosis / Q000503:physiopathology; D055815:Young Adult
keywords: Congenital heart disease; Electrophysiology mapping; Sudden cardiac death; Tetralogy of Fallot; Ventricular tachycardia arrhythmia