Echocardiographic diagnosis and prognosis of fetal left ventricular noncompaction.
BACKGROUND
Left ventricular noncompaction (LVNC) has rarely been described in the fetus.


METHODS
The presence of associated congenital heart disease and rhythm disturbance was identified and the presence of heart failure was assessed using the cardiovascular profile score in all fetuses with LVNC presenting from January 1999 to July 2010. The left ventricle was divided into 12 segments-four segments each at the base, midpapillary, and apical regions-in the short-axis view to calculate the noncompaction/compaction ratio for each segment.


RESULTS
Of 24 fetuses with LVNC included in the study, 22 had significant congenital heart disease, and 15 had complete heart block. Of the 16 patients with adequate follow-up and not electively terminated, 12 (81%) died or progressed to heart transplantation. The average noncompaction/compaction ratios were 2.02 in patients who died or underwent heart transplantation and 1.67 in survivors (P = .2034). Fifty-seven of 93 measured segments (61%) of the left ventricle in the patients who died or underwent heart transplantation had noncompaction/compaction ratios ≥ 2 compared with five of 17 measured segments (29%) in survivors (P = .0837). The average cardiovascular profile score was 6. The apical region had greater involvement of noncompaction than the midpapillary and basal regions, with ratios of 2.27, 2.14, and 1.10, respectively (P = .00035).


CONCLUSIONS
Fetuses with LVNC have a poor prognosis that may be related to associated congenital heart disease, increased segmental involvement of noncompaction, and complete heart block and can be predicted by the cardiovascular profile score.
Lucile Packard Children's Hospital, Stanford University Medical Center, Division of Pediatric Cardiology, Palo Alto, California 94304, USA. alisa.arunamata@stanford.edu
Alisa
A
Arunamata
Rajesh
R
Punn
Bettina
B
Cuneo
Saroja
S
Bharati
Norman H
NH
Silverman
D004452:Echocardiography / Q000379:methods*; D005260:Female; D006801:Humans; D056830:Isolated Noncompaction of the Ventricular Myocardium / Q000000981:diagnostic imaging*; D008297:Male; D011296:Prenatal Diagnosis / Q000379:methods*; D011379:Prognosis; D015203:Reproducibility of Results; D012680:Sensitivity and Specificity