Journal of the American Society of Echocardiography
Volume 23, Issue 1 , Pages 46-53, January 2010

Cardiac Segmental Analysis in Left Ventricular Noncompaction: Experience in a Pediatric Population

  • Rajesh Punn, MD
    • ,
  • Norman H. Silverman, MD, DSc (Med)

      Affiliations

    • Corresponding Author InformationReprint requests: Norman H. Silverman, MD, DSc (Med), Stanford University, Division of Pediatric Cardiology, 750 Welch Road, Suite 305, Palo Alto, CA 94304.

Stanford University, Lucile Packard Children's Hospital, Palo Alto, California

published online 26 October 2009.

Background

Echocardiography has been used to diagnose and describe left ventricular noncompaction (LVNC). No other study has investigated LVNC using the 16-segment model described by the American Heart Association and the American Society of Echocardiography in children, some of whom have congenital heart disease. Using the ratio of noncompaction to compaction, the authors analyzed the 16 segments and determined if severity was correlated with poor outcomes in a pediatric population.

Methods

The 16-segment noncompaction/compaction ratio, shortening, and ejection fractions were measured retrospectively in all children with LVNC at a single institution from January 1, 2000, to June 30, 2008.

Results

Forty-four patients had LVNC, an incidence of 0.3% of laboratory admissions. Twenty-eight patients (64%) who remained alive were assigned to group 1, and 16 patients (36%) who either died or were transplanted constituted group 2. Group 2 had more patients with significant associated congenital heart disease than group 1 (50% vs 18%, P < .05). We found similar regions of involvement in the 16-segment model with sparing of basal segments and involvement of the midpapillary and apical regions (P < .001); however, patients in group 2 were noted to have more segments involved (6 vs 4, P < .05), lower shortening fractions (16% vs 29%, P < .001), and lower ejection fractions (24% vs 47%, P < .001). The ejection fraction was inversely related to the number of segments (r = −0.63, P < .01), suggesting that more noncompaction portends a worse outcome.

Conclusions

In younger patients with noncompaction, poor outcomes such as low ejection fractions, death, and transplantation are related to the number of left ventricular segments involved. There is more associated congenital heart disease in the pediatric population, which carries a poorer prognosis than the disease reported in adult populations.

Keywords: Left ventricular noncompaction, Echocardiography, Pediatric

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PII: S0894-7317(09)00809-8

doi:10.1016/j.echo.2009.09.003

Journal of the American Society of Echocardiography
Volume 23, Issue 1 , Pages 46-53, January 2010

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