Medium-term outcomes of idiopathic ventricular fibrillation survivors and family screening: a multicentre experience

Europace. 2017 Nov 1;19(11):1874-1880. doi: 10.1093/europace/euw251.


Aims: Early repolarization (ER) has been linked to poorer outcomes in idiopathic ventricular fibrillation (IVF). The role of family screening in IVF is not clear. Our aim was to review predictors for poorer outcomes and evaluate the role of family screening in IVF.

Methods and results: This was a retrospective multicentre cohort study including all patients diagnosed with IVF. Data were collected on baseline characteristics, ECG findings, and recurrence of ventricular arrhythmia (VA) during follow-up. Electrocardiogram findings were reviewed in first-degree relatives that were screened. A total of 66 patients were included with male predominance (42/66, 64%) and Caucasian ethnicity (47/66, 71%). Mean age at cardiac arrest was 38 years ± 11. Thirty-one patients had ER (47%) predominantly with J-point amplitude ≥2 mm and horizontal ST segments (18/31, 58%). Recurrent VA was seen in 13 patients (20%). Horizontal ST segments were associated with increased rates of VA recurrence (OR 11, 95% CI 2.7-43.7; P = 0.0007). Early repolarization was seen in 20% of the 72 first-degree relatives and was more common if the proband had persistent ER pattern (OR 10.7, 95% CI 2.2-51.5; P = 0.003).

Conclusion: Ventricular arrhythmia recurrence was lower than previously reported. Early repolarization was common in this IVF cohort, and horizontal ST segments were suggestive predictor for poorer outcomes. Persistent ER in proband was associated with ER in first-degree relatives. With better understanding of its predictive value and the relationship to IVF, this information could potentially be used to guide family screening and identify new mutations using family members with persistent ER.

Keywords: Early repolarization; Family screening; Idiopathic ventricular fibrillation; Sudden cardiac death; Ventricular arrhythmia.

Publication types

  • Multicenter Study
  • Observational Study

MeSH terms

  • Adult
  • Clinical Decision-Making
  • Death, Sudden, Cardiac / etiology
  • Electrocardiography
  • Female
  • Genetic Predisposition to Disease
  • Genetic Testing / methods*
  • Heart Arrest / genetics
  • Heart Arrest / mortality
  • Heredity
  • Humans
  • Male
  • Middle Aged
  • Mutation*
  • Odds Ratio
  • Pedigree
  • Phenotype
  • Predictive Value of Tests
  • Prognosis
  • Recurrence
  • Retrospective Studies
  • Risk Factors
  • Time Factors
  • Ventricular Fibrillation / diagnosis*
  • Ventricular Fibrillation / genetics
  • Ventricular Fibrillation / mortality
  • Ventricular Fibrillation / physiopathology

Supplementary concepts

  • Paroxysmal ventricular fibrillation