The association of atrioventricular septal defect with common atrioventricular junction and malformations of the ventricular outflow tracts presents a significant challenge for the surgeon. In the most common of these, the association with tetralogy of Fallot, several surgical techniques have been described, and shown to deliver excellent results.1–10 On the other hand, in the setting of more extreme malformations, such as double-outlet right ventricle, discordant ventriculo-arterial connections, or common arterial trunk, albeit rare lesions, the combination presents a more formidable surgical challenge, as evidenced by the few reports of successful repair of these lesions. This challenge is both physiological, when dealing with a very sick neonate or infant, as well as anatomical in terms of the complexity of the malformation and the ability to achieve a successful biventricular repair. Our goal in this review is to discuss the surgical treatment in the setting of tetralogy of Fallot and double outlet right ventricle, with emphasis on biventricular repair.
