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Staged Biventricular Repair Of Heterotaxy, Double Outlet Right Ventricle, Unbalanced Complete Atrioventricular Canal Defect, And Transposition Of The Great Arteries In A 5-year-old With Failing Glenn Physiology
Nicholas D. Andersen, MD, Robert D. B. Jaquiss, Shannon Blalock, Munes Fares, Mohammed I. Ahmed, Roger Cruz, Jessica McDermott, Alan P. O'Donnell, Ryan R. Davies.
Children's Health Dallas / UTSW, Dallas, TX, USA.
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Objective: The patient is a 5-year-old male with heterotaxy, double outlet right ventricle, right-dominant complete atrioventricular canal defect and transposition of the great arteries. Initial palliations included pulmonary artery banding and bidirectional Glenn. By age 4 the patient developed severe atrioventricular valve regurgitation and was not considered a candidate for Fontan palliation. We instead elected to pursue biventricular repair in attempt to avoid cardiac transplantation.
Methods: A staged approach was chosen to shorten convalescence and minimize risk. The first operation entailed atrial septation with correction of pulmonary and systemic venous anomalies, and creation of two well-functioning atrioventricular valves capable of supporting a biventricular circulation. The second operation, performed 9 months later, entailed ventricular septation with the aid of virtual and physical 3D modeling. Ventricular septation consisted of pulmonary artery debanding, arterial switch operation, and ventricular septal defect enlargement with placement of a left ventricle to neo-aorta intraventricular baffle. The original pulmonary valve had not been distorted by the band, and was used as the systemic semilunar valve, thus avoiding use of a right ventricle to pulmonary artery conduit.
Results: The patient was discharged home on post-operative day 7 after both operations. Final discharge echocardiography demonstrates a well-functioning two-ventricle circulation without significant valvular abnormalities or outflow tract obstruction and normal sinus rhythm.
Conclusions: This case highlights a successful approach to complex heterotaxy canal repair in an older child, aided by 3D modeling and a staged surgical strategy.
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