Shaji C Menon, MD,1Patrick W O’Leary, MD,1 Gregory B Wright, MD,4 Rodrigo Rios, MD,2 Susan G MacLellan-Tobert, MD,3
Allison K Cabalka, MD.1
1Departments of Pediatric and Adolescent Medicine, Division of Pediatric Cardiology,
Mayo Clinic, Rochester, MN; 2Pediatric Cardiology, MeritCare Broadway, Fargo, ND; 3Pediatric Cardiology,
Gundersen Lutheran Medical Center, LaCrosse, WI; 4Department of Pediatric Cardiology, Children’s Hospital and
Clinics of Minnesota, St. Paul, MN.
Purpose: Noncompaction of the ventricular myocardium (NCVM) is a rare cardiomyopathy characterized by
numerous, prominent trabeculations and deep intertrabecular recesses caused by arrest in myocardial embryogenesis.
This study reviews our experience with neonatal and fetal NCVM. In contrast to the past reports of fetal and
neonatal NCVM, all pts in this group improved, and survived infancy.
Methods: We reviewed data from 6 consecutive fetus and or neonates with NCVM to elucidate aspects of diagnosis,
clinical presentation and outcome.
Results: Five of 6 pts were diagnosed by fetal echo at 22—25 wks gestation. Two were males, 5 of 6 had biventricular
NCVM. Left ventricular enlargement (or hypertrophy) with decreased ejection fraction (EF) was evident in
all pts at presentation. Inotropes were required in 3 pts postnatally. One fetus had signs of severe cardiac failure and
was delivered at 27wks. Treatment included; diuretics, ACE inhibition, digoxin and beta-blockade. Mean EF at
presentation was 36% and improved to 57% at a mean follow up of 2 yrs. There have been no deaths. All showed
marked improvement in cardiac function and clinical status. One patient had a transient, late decrease in EF, which
improved with intensified therapy. Developmental delay was noted in 4 of 6 pts. Congenital cardiac anomalies were
noted in 3 pts, and 1 was found to have Barth syndrome. One pt had a father with Ebstein anomaly. No familial
cases of NCVM were identified.
Conclusion: Unlike previous studies, all 6 neonates, including the 3 requiring inotropic support, showed significant
early recovery of cardiac function. The majority of these newborns had biventricular involvement. NCVM should
be considered in the differential diagnosis of any fetus presenting with ventricular dysfunction in utero, especially
if ventricular wall thickening is present. However, the prognosis is not necessarily grim. Pts with NCVM may display
variable ventricular function; therefore it will be essential to continue cardiac follow up, even if function normalizes
for a time.

Abstracts 276 – Abstracts of the American Academy of Pediatrics Section on Cardiology and Cardiac
Surgery—2006 AAP National Conference and Exhibition
Congenit Heart Dis. 2006;1:257–277

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