Congenital left ventricular inflow obstructions--an echocardiographic study.

Thirty nine consecutive cases of congenital left ventricular inflow obstructions (LVIO) diagnosed by two dimensional and Doppler echocardiography are described. The commonest referral diagnosis was severe pulmonary arterial hypertension. Confirmation by cardiac catheterisation and/or surgery was available in twenty one of these cases. The ages ranged from one month to 15 years (mean 3.8 +/- 4.5 years) and 28 were males. Four types of LVIO were defined: i) Congenital mitral stenosis (22 cases). Ventricular septal defect was the commonest association. ii) Cor-triatriatum (7 cases) Out of three patients operated, in one it was found to be a supravalvular mitral ring and not cor-triatriatum as earlier thought on echocardiography. iii) Pulmonary venous obstruction (8 cases). Atrial septal defect and ventricular septal defect were the common associations. iv) Supravalvular mitral ring (2 cases). Double outlet right ventricle and a large ventricular septal defect were associated with one case each. In our experience, two dimensional and Doppler echocardiography is a very useful and reliable investigation for defining the various types of congenital left ventricular inflow obstructions and their hemodynamic significance.