Valve-sparing aortic root replacement for patients with a Fontan circulation

Aortic root aneurysm is an emerging disease related to the treatment of patients with hypoplastic left heart syndrome (HLHS) and other single-ventricle physiology. The authors' experience with valve-sparing aortic root replacement and concomitant procedures in patients with a single ventricle was reviewed.

Four patients aged 11 to 19 years presented with progressive aortic root dilatation. All had undergone a Fontan completion: two patients for HLHS, one patient for tricuspid valve atresia, and one for pulmonary atresia with a ventricular septal defect. Two patients developed more than grade 2+ aortic valve insufficiency, and one patient developed symptomatic left anterior descending coronary artery compression. The preoperative root dimension ranged from 38 to 56 mm (mean 45mm). A valve-sparing aortic root replacement, annular reduction and ascending aorta replacement was performed in all patients. Two patients underwent a Fontan revision, two an aortic valve repair, and one patient a tricuspid valve repair.

At a mean follow up of 2.6 years, all four patients were in NYHA functional class I. Two patients underwent neo-aortic valve replacement at about two years after aortic root replacement and valve repair, as they had developed severe neo-aortic valve insufficiency. Both of these patients had HLHS. In the other two patients, the most recent echocardiography demonstrated grade 0 to 1+ aortic insufficiency, with good function of the single ventricle.

Aortic root reimplantation is an effective treatment for aortic root dilatation in patients with a single ventricle. However, if associated with significant aortic insufficiency, then a long-term freedom from valve replacement is questionable.