The natural history of the hypoplastic left heart syndrome

In a fetus, examined initially at 22 weeks gestation, we identified the echocardiographic features of a dilated, hypertrophied and poorly contracting left ventricle. The presumptive diagnosis was critical aortic stenosis. Subsequent scans at 32 weeks and at term showed that the left ventricle had not grown since the first study such that the left ventricle had developed the appearance of a hypoplastic and densely echogenic chamber. Thus, in some forms of the hypoplastic left heart syndrome, the left ventricle can be of normal size or even dilated in early pregnancy. This may mean that the more subtle sign of poor left ventricular contraction could be overlooked in a routine four-chamber view obstetric scan.