Significant atrioventricular (AV) valve insufficiency in patient with single ventricle-physiology is strongly associated with poor survival. Herein we discuss the etiology and mechanism of development of significant AV valve insufficiency in patients with single-ventricle physiology, surgical indication and repair techniques, and clinical outcomes along with our 10-year surgical experience. Our recent clinical series and literature review indicate that it is of prime importance to appreciate the high incidence and clinical effect of the structural abnormalities of AV valve. Valve repair at stage II palliation may minimize the period of volume overload, thereby potentially preserving post-repair ventricular function. Since 85% of the AV valve insufficiency was associated with structural abnormalities, inspection of an AV valve that has more than mild to moderate insufficiency is recommended because they are not likely to be successfully treated with volume unloading surgery alone.
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