Surgical repair of hemitruncus: principles and techniques

Ten patients have presented at the World Children's Hospital of Melbourne with hemitruncus, a form of truncus arteriosus. Nine of these patients underwent successful one- or two-stage hemodynamic repair. Early repair is carried out to avoid pulmonary vascular disease of the lung directly supplied from the trunk and to recruit as much of the contralateral pulmonary artery vasculature bed as possible is a secondary goal. The repair should be contemplated within the first six months of life to prevent severe pulmonary vasculature obstructive disease. Anatomy that is repairable in one stage is when the left pulmonary artery originates from the trunk and the complete right pulmonary artery supplied by pulmonary collaterals from the arch to the descending aorta. Anatomy requiring two-stage repair occurs when the RPA rises from the trunk and the left PDA rises from the descending aorta or distal aortic notch and either the right or the left lung blood supply is from multiple origins and requires unifocalization. With these anatomical principles and the general meticulous perioperative and postoperative care of the infants, a high degree of success should be obtainable with repair of this hemitruncus.