ABC | Volume 112, Nº1, January 2019

Elias Nero et al ARVC/D - Diagnosis and treatment Arq Bras Cardiol. 2019; 112(1):91-103 Review Article Figure 1 – Evolution example of ARVC/D. Patient diagnosed with ARVC/D at age 32, after recovery from SAD during sports practice. He underwent implantation of ventricular ICD with multiple episodes of VF in clinical progression. At age 50, he developed sinus dysfunction and episodes of atrial fibrillation with a need for exchange for bicameral ICD. A) 12-lead ECG at diagnosis. Presence of T-wave inversion of V1-V6. Epsilon wave present in all precordial leads and final duration of QRS ≥ 55 ms. B) ECG with atrial fibrillation. C) inappropriate therapy due to atrial fibrillation. Continuation 5. Arrhythmias Major criteria • Non-sustained or sustained VT with LBBB type morphology and upper axis Minor criteria • Non-sustained or sustained VT with RVOT morphology (LBBB type morphology and lower or indeterminate axis) > 500E vs/24h - 24h Holter 6. Family History Major criteria • ARVC/D in first-degree relative who meets TFC2010 criteria • ARVC/D pathologically confirmed in first degree relative (autopsy or biopsy) • Identification of pathogenic mutation classified as associated or probably associated with ARVC/D in the patient under evaluation Minor criteria • History of ARVC/D in first degree relatives • History of ARVC/D in a first-degree relative for whom it is not possible to determine whether it meets TFC criteria • Sudden premature death (< 35 years of age) with suspected ARVC/D in first degree relative • ARVC/D confirmed pathologically or through TFC in second degree relative Adapted from Pinamonti et al., 2014. 16 ARVC/D: right ventricular arrhythmogenic cardiopathy/dysplasia; BSA: body surface area; CMR: cardiac magnetic resonance; ECG: electrocardiogram; EDV: end-diastolic volume; RBBB: right bundle Branch block; LBBB: left bundle Branch block; PLAX: parasternal long axis; PSAX: parasternal short axis; RV right ventricle; RVOT: right ventricular outflow tract; ECG-HR: high resolution electrocardiogram; Ventricular tachycardia; TFC task force criteria. ARVC/D. 1,2,23 It reflects the presence of large late potentials on the surface ECG. Although considered a major criterion for the diagnosis of ARVC/D, LPs may also be present in other pathologies, particularly cardiac sarcoidosis. 9 These activation delays are best diagnosed with ECG-HR. Currently, a positive ECG-HR is considered a minor criterion. Also included as a diagnostic criterion was the detection of a final activation delay, which is defined as prolongation of QRS duration (> 110 ms) and S wave (≥ 55 ms) in V1-3. Arrhythmias Increased susceptibility to ventricular tachyarrhythmia and SAD is the main characteristic of ARVC/D. 1 In general, ventricular arrhythmias, in an isolated and frequent form, or non-sustained and sustained ventricular tachycardia are associated with symptoms of palpitation, dizziness, presyncope and syncope. Due to the most common RV origin, this ventricular arrhythmia presents LBBB morphology with a variable axis depending on the affected site. 5,6,9 5