Double inlet left ventricle: Comprehensive echocardiographic anatomy evaluation with special focus on the morphology of atrio-ventricular valves and ventricular septal defect: Case report and literature review

In double inlet left ventricle (DILV), the ventricle most frequently has left ventricle (LV) morphology, although right ventricle (RV), mixed, indeterminate, or undifferentiated morphologies have been reported. The main ventricle is largely a morphological LV with an outlet chamber connected to it which has a RV morphology. Ventricular septal defect (VSD) or bulboventricular foramen size and anatomy may be variable. The AV valves may be normal, or one of them may be hypoplastic, stenotic, or even atretic. The great arteries are most frequently transposed and the aorta arises from the hypoplastic RV, and the pulmonary artery comes off the main LV chamber. L-transposition of great arteries (L-TGA) happens more often than d-transposition of great arteries (D-TGA). The great vessels are normally related in 30% of cases. Double-outlet right ventricle (DORV) may be seen, in which both great vessels arise from the rudimentary RV. Pulmonary stenosis (PS) is present in two thirds of patients and such stenosis is seen irrespective of the great artery relationship. Rarely subaortic obstruction maybe present in patients with transposition of the great arteries.
Here, we are presenting a case of complex cyanotic congenital cardiac defect of DILV with VSD complicated by L-TGA and severe tricuspid regurgitation (TR) in a 3-year-old male toddler.