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You are here: Contents > 2012 > Volume 21 Number 2 March 2012 > AORTIC VALVE DISEASE > Valve-Sparing Aortic Root Replacement for Patients with a Fontan Circulation

Valve-Sparing Aortic Root Replacement for Patients with a Fontan Circulation

Eldad Erez, Vincent K. H. Tam, Carlos Galliani, Andrew Lashus, Nancy A. Doublin, Jana Peretti 

Cook Children’s Medical Center, University of North Texas, Fort Worth, Texas, USA

Background and aim of the study: 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.
Methods: 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.
Results: 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.
Conclusion: 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.

The Journal of Heart Valve Disease 2012;21:175-180

Valve-Sparing Aortic Root Replacement for Patients with a Fontan Circulation

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