65. A female neonate with unguarded mitral valve orifice

This is a rare congenital heart disease case of unguarded mitral valve orifice, transposition of great vessels, and pulmonary atresia with a dilated left ventricle that has not been reported. A 3kg, Saudi, female neonate presented from a local hospital with oxygen saturations of 85%. She came with a prenatal diagnosis of Ebstein’s anomaly and pulmonary atresia. Post natal echocardiogram showed situs solitus, atrial ventricular concordance and ventricular arterial discordance, confluent pulmonary arteries that are ductal dependent and atrial septal defect. There was no mitral valve apparatus with to and fro movement into a dilated thinned out left ventricle. She was started on prostaglandin. Physical exam revealed non dysmorphic features. The precordium was hyperactive with single s1, s2, and early systolic murmur radiating to apex. She had good quality pulses. Electrocardiogram showed sinus tachycardia without pre-excitation and prominent right forces. She did develop supraventricular tachycardia that was controlled. Chest film showed massive cardiomegaly. MRI confirmed echocardiography findings. Surgical decision was to initiate a single ventricle approach with exclusion of the left ventricle. Biopsy showed myocardial muscle disarray, excluded diagnosis of Uhl’s anomaly and ischemia. Postoperatively had heart block but surgical shunt was patent. She succumbed to arrest after extubation. This case raises many questions regarding embryologic cardio genesis, specifically the association of pulmonary atresia, transposition of the great vessels, and unguarded mitral valve orifice. What is the optimal surgical technique in such a patient and does left ventricle exclusion impair right ventricular function?