Myocardial ischaemia in children with isolated ventricular non-compaction

Eur Heart J. 1999 Jun;20(12):910-6. doi: 10.1053/euhj.1998.1398.

Abstract

Aims: Isolated ventricular non-compaction is a rare congenital cardiomyopathy with a high morbidity and mortality due to malignant arrhythmias and pump failure. Areas affected by non-compaction are characterized by increased trabecularization and deep inter-trabecular spaces. We hypothesized perfusion defects in these areas and performed positron emission tomography to evaluate the myocardial perfusion in non-compacted areas.

Methods and results: Five children (age 10-14 years) with isolated ventricular non-compaction underwent positron emission tomography using N-13-ammonia as flow marker and intravenous dipyridamole for stress testing. Myocardial blood flow was quantified using the positron emission tomography time-activity curves in non-compacted areas and normal myocardium, which were diagnosed by echocardiography, magnetic resonance imaging, and angiography. Coronary angiography, performed in two children with extensive forms of left ventricular non-compaction, demonstrated normal coronary arteries. Myocardial blood flow measurements at rest and after dipyridamole application demonstrated 16-33% and 32-57% perfusion impairment, respectively, in non-compacted areas compared to normal myocardium. Areas of restricted myocardial perfusion corresponded well to the non-compacted areas, defined echographically and by magnetic resonance imaging.

Conclusion: Positron emission tomography demonstrates restricted myocardial perfusion and decreased flow reserve in areas of ventricular non-compaction in children. The myocardial perfusion defects in non-compacted areas may be the cause of myocardial damage and possibly form the basis of arrhythmias and pump failure.

MeSH terms

  • Adolescent
  • Child
  • Coronary Circulation*
  • Female
  • Heart / diagnostic imaging
  • Heart Diseases / congenital*
  • Heart Diseases / diagnostic imaging
  • Heart Diseases / physiopathology*
  • Heart Ventricles
  • Humans
  • Magnetic Resonance Imaging
  • Male
  • Myocardium / pathology
  • Regional Blood Flow
  • Tomography, Emission-Computed
  • Ultrasonography
  • Ventricular Dysfunction / etiology