Can isolated ventricular inversion survive without atrial or ventricular septal defect?- case report

Background: Isolated ventricular inversion is characterized by atrioventricular discordance and ventriculoarterial concordance, clinically akin to transposition of the great arteries. In the absence of a significant atrial or ventricular septal defect, profound cyanosis is expected at birth. Case Report: A 5-month-old infant with isolated ventricular inversion presented with mild cyanosis. The left sided tricuspid valve straddled the interventricular septum with a closed interventricular communication, a type of “Double Outlet Left Atrium with three atrioventricular valves”, which provided the necessary ‘left to right’ shunt while severe regurgitation through the straddling segment and a patent ductus arteriosus provided the effective pulmonary blood flow. Result: The infant underwent atrial septectomy, closure of the leaky accessory orifice and a Damus-Kaye-Stansel anastomosis and a bidirectional Glen operation. Conclusion: A unique natural mechanism permitting survival in a child with transposition physiology is described. The Damus procedure preserved tricuspid valve, right ventricle and pulmonary valve function.