Myocardial fibrosis in isolated left ventricular non-compaction and its relation to disease severity

Eur J Heart Fail. 2011 Feb;13(2):170-6. doi: 10.1093/eurjhf/hfq222. Epub 2011 Jan 4.

Abstract

Aims: The aim of the present study was to evaluate the prevalence and extent of myocardial fibrosis in patients with isolated left ventricular non-compaction (LVNC) and to determine its relation to clinical status and LV systolic function.

Methods and results: The cardiac magnetic resonance imaging (MRI) database of our institution was searched for all patients with a first diagnosis of isolated LVNC. The diagnosis of isolated LVNC was based on the presence of standard cardiac MRI and clinical criteria. For each patient, cine and contrast-enhanced cardiac MR images were analysed to evaluate LV systolic function and the prevalence and extent of late gadolinium enhancement (LGE), a surrogate of myocardial fibrosis. A total of 42 patients (mean age 46 ± 20 years, 62% male) were identified. Late gadolinium enhancement was observed in 23 (55%) patients with isolated LVNC, occupying 4.8 ± 6.7% of the LV mass. Both the presence and extent of LGE were significantly related to the number of abnormal clinical features (i.e. symptomatic status, resting electrocardiogram abnormalities, and 24 h Holter monitoring abnormalities; P < 0.001 and P= 0.001, respectively). Similarly, LGE was more prevalent and extensive in patients with LV ejection fraction (EF) < 50% compared with patients with LVEF ≥ 50% (90 vs. 23%; P< 0.001 and 8.9 ± 7.6 vs. 1.1 ± 2.4%; P< 0.001, respectively). At multivariate analysis, both the presence and extent of LV LGE were independently related to LVEF (β = -0.63; P < 0.001 and β = -0.62; P< 0.001, respectively).

Conclusion: Myocardial fibrosis is related to clinical disease severity and LV systolic dysfunction in isolated LVNC.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Aged
  • Analysis of Variance
  • Cardiomyopathies / diagnosis*
  • Cardiomyopathies / epidemiology
  • Cohort Studies
  • Databases, Factual
  • Disease Progression
  • Female
  • Fibrosis / pathology
  • Gadolinium
  • Humans
  • Incidence
  • Linear Models
  • Magnetic Resonance Imaging / methods*
  • Male
  • Middle Aged
  • Myocardium / pathology*
  • Prognosis
  • Radiographic Image Enhancement / methods
  • Risk Assessment
  • Severity of Illness Index
  • Statistics, Nonparametric
  • Stroke Volume / physiology
  • Ventricular Dysfunction, Left / diagnosis*
  • Ventricular Dysfunction, Left / epidemiology
  • Young Adult

Substances

  • Gadolinium