To evaluate the accuracy and clinical utility of three-dimensional echocardiography in the assessment of the size and shape of the ventricular septal defect in double inlet left ventricle.

We validated the technique in an autopsy study, and then performed a clinical investigation. Six autopsied hearts were immersed in a waterbath and examined with 3-dimensional echocardiography. We identified the cross-section within the dataset which optimally displayed the ventricular septal defect “en face”, and compared its smallest and largest diameters, as well as its area. The ventricular septal defect was then filled with a silicone sealant and a section prepared for direct measurement. In patients, we measured the diameters and area of the ventricular septal defect in endsystole nad computed the aortic valvar area in endsystole from the cross-section showing the aortic valve “en face”. Ten patients with double inlet left ventricle, aged between 2 and 15 years, were studied using rotational or parallel scanning. All patients had undergone banding of the pulmonary trunk at a mean age of 7 (3–36) days, usually at the time of repair of the coarctation. Two patients had undergone surgical enlargement of the ventricular septal defect prior to echocardiographic examination.

The correlation between the areas of the ventricular septal defect in the specimens measured directly and by 3-dimensional echocardiography was r=0.98, with limits of agreement between −0.1– 0.08 cm2. In the patients, the area of the defect was measured as 3.9±2 cm2, whereas the aortic valvar area was 2.6±0.9 cm2. The ratio between the areas was 1.5 (0.5–2.3). Three patients with areas of the ventricular septal defect smaller than those of the aortic valve had resting Doppler gradients between double inlet left ventricle and the aorta of 16, 20 and 30 mm Hgs, respectively.

3-dimensional echocardiography provides accurate assessment of the area of the ventricular septal defect in double inlet left ventricle, and is helpful in identifying patients with subaortic stenosis caused by restrictive defects.