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Severe AVSD Valve Regurgitation In A Single Ventricle Infant - When The Perfect Repair Results In A Perfect Disaster!
Christopher J. Knott-Craig, MD FACS, Karan Karki, MD, Umar S. Boston, MD, Vijaya Joshi, MD.
Le Bonheur Children's Hospital, Memphis, TN, USA.

Objective(s): Unbalanced complete AVSD (complete A-V canal) to the left associated with severe regurgitation is a formidable surgical dilemma especially when it occurs in a single ventricle pre-term infant with bilateral congenital diaphragmatic hernias (CDH). The surgical management of such a patient is presented and the dilemma of a perfect valve repair is exposed when there is associated tricuspid atresia.
Methods: The echocardiograms, cardiac catheterization data, surgeries and clinical course of a 4 month old Down's syndrome infant is reviewed. This preterm infant required bilateral repair of CDH and continuous respiratory support in the NICU since birth. Preoperative echo demonstrated unbalanced AVSD to the left, very hypoplastic right ventricle, and severe (4/4) AVV regurgitation. There was a large primum ASD, a 6-8 mm VSD, unobstructed pulmonary outflow tract; oxygen saturations were 87% on room air.
Results: Initial operation was complex valve repair and bilateral PA bands. Early postoperative hypoxemia prompted repeat TEE which showed tricuspid atresia and now a 2 mm VSD component. Emergent removal of PA bands and placement of a BT shunt was done and chest left open. Subsequently oxygen saturations were high prompting re-placement of PA bands with chest closure. A protracted but successful postoperative course ensued.
Conclusions: Rarely complete AVSD may co-exist with tricuspid atresia; the inflow into the diminutive RV is through the cleft in the left-sided AVV in the context of severe AVV regurgitation. Successful repair of the AVV results in rapid shrinkage of the VSD and associated lack of pulmonary blood flow.


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