Pulmonary atresia with intact ventricular septum in the setting of D-transposition of the great arteries associated with hypoplastic left ventricle and severe mitral regurgitation.

We report the first successful Glenn procedure for pulmonary atresia with intact ventricular septum in the setting of D-transposition of the great arteries. This case was complicated by hypoplastic left ventricle and severe mitral regurgitation. Partial closure of the mitral valve at the time of Blalock-Taussig shunting resulted in improvement of heart failure.