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Anatomical features and clinical correlations in Caucasian patients with definite arrhythmogenic right ventricular

R M Inciardi1, E Maresi, G Coppola

  • 1UOC Cardiologia II con Emodinamica "P. Giaccone" Hospital University of Palermo, Palermo, Italy - riccardo.inciardi@libero.it.

Minerva Cardioangiologica
|October 9, 2014
PubMed

Insights

Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) patients with early onset, high physical activity, or biventricular disease face higher risks. Left ventricular involvement is common, and active myocarditis may contribute to fatal events.

Area of Science:

  • Cardiology
  • Genetics
  • Pathology

Background:

  • Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is an inherited heart condition.
  • It involves fibrofatty tissue replacement, leading to ventricular arrhythmias and sudden cardiac death (SCD).

Purpose of the Study:

  • To investigate the pathological characteristics of ARVD/C patients.
  • To explore clinical correlations and identify predictors of adverse outcomes.

Main Methods:

  • A multicenter study analyzed 47 patients diagnosed with ARVD/C.
  • Patients were categorized into two groups: 28 alive and 19 who experienced sudden death.

Main Results:

  • Age at presentation and history of physical exercise were significantly associated with sudden death risk.
  • Biventricular ARVD/C and earlier age at presentation correlated with negative outcomes like SCD or heart failure.
  • Left ventricular (LV) involvement was noted in 17-32% of patients, and 26% showed inflammatory infiltrates suggesting active myocarditis.

Conclusions:

  • Frequent LV involvement supports the broader term Arrhythmogenic Cardiomyopathy.
  • Early age, sports participation, and biventricular disease are key predictors of adverse outcomes in ARVD/C.
  • Identifying these factors aids in early risk stratification for high-risk patients.
Abstract

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