Left ventricular non-compaction cardiomyopathy in children: Is segmental fibrosis the cause of tissue Doppler alterations and of EF reduction?

Giovanni Fazio, Giuseppina Novo, Calogero Casalicchio, Gabriele Di Gesaro, Loredana Sutera, Emanuele Grassedonio, Eliana Cracolici, Valentina Pitruzzella, Egle Incalcaterra, Salvatore Pipitone, Massimo Midiri, Salvatore Novo

Research output: Contribution to journalArticlepeer-review

Abstract

Noncompaction of the ventricular myocardium (LVNC) is a rare congenital cardiomyopathy resulting from an arrest in normal endomyocardial embryogenesis. In 2002 Jenni et al. [Jenni R, Wyss CA, Oechslin EN, Kaufmann PA. Isolated ventricular noncompaction is associated with coronary microcirculatory dysfunction. J Am Coll Cardiol 2002; 39:450-454.] reported a microvascular dysfunction in 12 patients affected by non compaction: areas of restricted myocardial perfusion have been documented by scintigraphy, suggesting a reduction of Coronary flow reserve. McMahon et al reported in a recent article a reduction of TD velocities in children with noncompaction of the left ventricle, compared with normal controls. The authors concluded their work saying that the reduction of lateral mitral Ea velocity helps to predict children with LVNC who are at risk of adverse clinical outcomes including death and need for cardiac transplantation. In a precedent report our group reported a strong correlation between pathological tissue Doppler and reduction of ejection fraction. Recently we scanned with a Signa HD 1.5 T (GE, Milwaukee, USA) 8 patients affected by non compaction. Transmural Gd-enhancement was detected in 5/8 patients (62%). In all patients with late enhancement a reduction of EF has demonstrated. In our opinion the late enhancement can depend on a CFR, and is the determinant of the tissue Doppler alterations. So the TD alteration is associated with EF, and is an indirect index of poor clinical outcome, like EF.

Original languageEnglish
Pages (from-to)278-280
Number of pages3
JournalInternational Journal of Cardiology
Volume132
Issue number2
DOIs
Publication statusPublished - Feb 20 2009

Keywords

  • fibrosis
  • Noncompaction
  • RMN
  • tissue Doppler

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine

Fingerprint Dive into the research topics of 'Left ventricular non-compaction cardiomyopathy in children: Is segmental fibrosis the cause of tissue Doppler alterations and of EF reduction?'. Together they form a unique fingerprint.

Cite this