Risk factors determined by the 2010 Task Force Criteria (TFC) could help predict major adverse cardiac events (MACE) in patients with arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) patients.

ARVC/D is a cardiomyopathy with ventricular arrhythmias and fibro-fatty replacement of the right ventricular myocardium. In early phases, patients could be at risk for sudden cardiac death caused by sustained ventricular tachycardia (VT) or ventricular fibrillation (VF).

“There is no single diagnostic tool for ARVC/D, and the clinical diagnosis of ARVC/D is often difficult and complex,” the authors wrote in their study, published in the Journal of the American College of Cardiology.


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“The 1994 Task Force Criteria first provided the clinical diagnosis of ARVC/D based on several categories, such as the structure, function, histology, electocardiography, arrhythmia and family history. These criteria were modified in 2010 to improve diagnostic sensitivity by advances in the diagnostic modalities and the genetic knowledge of ARVC/D.”

The study included 90 patients with ARVC/D who met the diagnosis from the 2010 TFC. Researchers calculated risk scores by adding the major and minor criteria from the subdivided categories of the TFC. Any major criteria equated to 2 points and minor criteria were given 1 point. The risk scores were then sorted into 3 groups: Group A (risk scores between 4 and 6 points), Group B (risk scores between 7 and 9 points), and Group C (risk scores between 10 and 12 points).

During an average follow-up period of 10.2 years, 19 patients died of cardiovascular causes, 28 patients were admitted to the hospital because of worsened heart failure (HF), and 47 patients experienced sustained VT or VF.

The patients with risk scores in Group B and Group C showed an increased risk for MACE when compared with the risk scores from Group A (HR,4.80; 95% CI; P=.001 and HR,6.15; 95% CI; P=.001, respectively).

The mean ARVC/D risk score was 8 ± 2 points. Sustained VT/VF occurred in 64 patients and cardiac arrest in 3. Additionally, 9 patients had a family history of ARVC/D, confirmed in a first-degree relative who met the 2010 TFC.

The data showed an association between higher risk scores and a higher incidence of MACE, HF hospitalization, and sustained VT or VF. 

In addition, beta-blockers failed to prevent sustained VT/VF (HR, 1.05; 95% CI; P=.891) or cardiovascular death (HR, 0.43; 95% CI; P=.557).

“Risk factors of death in ARVC/D patients will change in the future if the strategies for the prevention and treatment of sudden cardiac death are recognized,” the researchers concluded.

They also noted that further research should be conducted to develop risk stratification and management of cardiovascular death and worsening HF for ARVC/D patients.

Reference

  1. Kikuchi N, Yumino D, Shiga T, et al. Long-Term Prognostic Role of the Diagnostic Criteria for Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia. J Am Coll Cardiol. 2015; doi:10.1016/j.jacep.2015.09.009.