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.
1 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.
2 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.