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We report 12 patients with ventricular noncompaction who were echocardiographically identified at our institution since 1991. The mean age at presentation was 3.5 years. Five patients had isolated noncompaction. Three of them had subnormal left ventricular systolic function at presentation. Noncompaction was associated with complex congenital heart defect in 3 patients. Four patients had simple congenital heart defects: pulmonary stenosis, coarctation of aorta with aberrant origin of right subclavian artery, ventricular septal defect, and partial anomalous pulmonary venous return. The observed rhythm abnormalities were Wolff-Parkinson-White syndrome and paroxysmal supraventricular tachycardia, bigemini ventricular extrasystoles, and left bundle branch block. A transvenous pacemaker was implanted in a patient because of complete heart block. Noncompaction of the ventricular myocardium is rare. Our patients clearly represent the clinical and morphological spectrum of this disorder. Distinct morphological features can be diagnosed on 2-dimensional echocardiography.

Visceroatrial situs, ventricular morphology, and atrioventricular and ventriculoarterial alignments can reliably be assessed with fetal echocardiography. We herein describe, to our knowledge, the first reported fetal echocardiographic diagnosis of a fetus with visceroatrial situs solitus, atrioventricular alignment discordance, double outlet right ventricle, and superoinferior ventricles of a 22-year-old gravida III para II woman with a previous history of intrauterine fetal death. She had been evaluated at another center and was referred to our unit because of the suspicion about her diagnosis and underwent fetal ultrasonographic evaluation at 39 weeks gestation. Neonatal echocardiographic evaluation and autopsy findings confirmed the diagnosis. Accordingly there was (solitus, L-loop ventricle, and anterior aorta) double outlet right ventricle, superoinferior ventricles with diminutive right ventricle, noncommitted trabecular ventricular septal defect, and pulmonary hypertension.