Long-Term Results of a Strategy of Aortic Valve Repair in the Pediatric Population

Study Questions:

What is the rate of reoperation after primary valve repair in pediatric patients with aortic valve disease?


A retrospective review was performed at a single center. The center had a preference towards aortic valve repair, which increased over the course of the study period, with no patients undergoing percutaneous valvuloplasties over the final 3 years of the study period.


Over the course of the 16-year study period (1996-2011), 142 patients underwent aortic valve repair. Patients whose surgery consisted of simple commissurotomy were excluded. During this period, 97 patients underwent the Ross procedure for aortic valve disease. The median age at surgery was 9 years, with 30 patients younger than 1 year of age. Surgery was performed for aortic stenosis in 76 patients, regurgitation in 55 patients, and mixed disease in 11 patients. Forty-six patients underwent repair with no patch, whereas 96 patients required addition of patches of glutaraldehyde preserved autologous pericardium for cusp extension (n = 51) and other repair (n = 45). Of patients undergoing cusp extension, two had sudden unexplained death and one had a cardiac arrest requiring mechanical circulatory support and cardiac transplantation. Two additional patients showed signs of coronary ischemia in the postoperative period. Seven-year freedom from intervention was 80%. By multivariate analysis, predictors for reintervention were cusp extension (hazard ratio [HR], 5.4; 95% confidence interval [CI], 1.7-16.8; p = 0.005) and age <1 year (HR, 5.6; 95% CI, 1.7-18.4; p = 0.005). At the most recent echocardiogram, 31% of patients had moderate or greater aortic stenosis or insufficiency.


The authors concluded that aortic valve repair in pediatric populations is effective in delaying reintervention. Caution should be used when performing tricuspidization and leaflet extension of bicuspid valves because it may result in occlusion of the coronary ostia.


This study demonstrates favorable long-term outcomes of aortic valve repair in infants and children. Several limitations of the study should be mentioned. This is a single-center study of a highly operator-dependent procedure done at an experienced center. This may limit the generalizability of the study to other centers. Additionally, the fact that 97 patients underwent the Ross procedure during the study period suggests that the investigators were selective in the application of valve repair, and emphasizes the importance of patient selection. The potential for coronary obstruction with tricuspidization and leaflet extension appears to be an important issue, and should be considered at the times of these repairs.

Clinical Topics: Arrhythmias and Clinical EP, Cardiac Surgery, Congenital Heart Disease and Pediatric Cardiology, Invasive Cardiovascular Angiography and Intervention, Valvular Heart Disease, Implantable Devices, SCD/Ventricular Arrhythmias, Cardiac Surgery and Arrhythmias, Cardiac Surgery and CHD and Pediatrics, Cardiac Surgery and Heart Failure, Cardiac Surgery and VHD, Congenital Heart Disease, CHD and Pediatrics and Arrhythmias, CHD and Pediatrics and Interventions, CHD and Pediatrics and Quality Improvement, Heart Transplant, Interventions and Structural Heart Disease

Keywords: Infant, Multivariate Analysis, Heart Defects, Congenital, Heart Arrest, Heart Transplantation, Glutaral, Reoperation, Heart Valve Diseases, Confidence Intervals, Cardiac Surgical Procedures, Thiazolidinediones, Pregnancy

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