Management of the Aortic Root in Adult Patients With Conotruncal Anomalies

Conotruncal anomalies such as tetralogy of Fallot, double outlet right ventricle, truncus arteriosus, and transposition of the great arteries are a group of congenital heart defects with abnormalities of the outflow tracts and great vessels. It is common for the ascending aorta and aortic root to be significantly dilated following initial repair of the conotruncal anomaly, and little information is available on the management of this increasing problem. Although there are few case reports of aortic dissection and rupture in the literature, it appears to be rare in the setting of a conotruncal anomaly and may be related to the absence of hypertension and smoking in many of these patients. The timing of operation with regard to the size of the aortic root is difficult. In the absence of a family history of aortic dissection or aneurysm, or documented rapid growth of the ascending aorta, we proceed with replacement of the ascending aorta when the size is ≥ 55 mm. When the size of the ascending aorta is 5.0-5.5, treatment is individualized depending on the associated anomalies that need to be addressed, patient comorbidities, and life expectancy. In this group of patients we consider a simple reduction ascending aortoplasty. We generally proceed with root replacement and coronary reimplantation when there is effacement of the sinotubular junction, or when there is severe dilatation of the aortic root with an intact sinotubular junction. If the ascending aorta is ≥ 55 mm with an intact sinotubular junction and the sinuses are ≤ 4 cm, then we use a supracoronary tube graft.