Validation and Re-Evaluation of a Discriminant Model Predicting Anatomic Suitability for Biventricular Repair in Neonates With Aortic Stenosis

Harvard University, Cambridge, Massachusetts, United States
Journal of the American College of Cardiology (Impact Factor: 16.5). 06/2006; 47(9):1858-65. DOI: 10.1016/j.jacc.2006.02.020
Source: PubMed


The purpose of this study was to validate and re-evaluate our previously reported scoring systems for predicting optimal management in neonates with aortic stenosis (AS).
In 1991, we reported a multivariate discriminant equation and an ordinal scoring system for predicting which neonates with AS are suitable for biventricular repair and which are better served by single ventricle management.
Retrospective analysis was performed to: 1) validate our scoring systems in 89 additional neonates with AS and normal mitral valve area, 2) assess the effects of 5% measurement variation on predictive scores, 3) evaluate our cohort with the Congenital Heart Surgeons' Society scoring system, and 4) repeat the discriminant analysis on the basis of all 126 patients.
The original scores each predicted outcome accurately in 68 patients (77%). Minor (5%) measurement variation changed the outcome predicted by the discriminant equation in 8 patients (9%) and by the threshold system in 13 patients (15%). The most accurate model for predicting survival with a biventricular circulation among the full cohort is: 10.98 (body surface area) + 0.56 (aortic annulus z-score) + 5.89 (left ventricular to heart long-axis ratio) - 0.79 (grade 2 or 3 endocardial fibroelastosis) - 6.78. With a cutoff of -0.65, outcome was predicted accurately in 90% of patients.
Both of our original scoring systems are less accurate at predicting outcome than in our original analysis. Revised discriminant analysis yielded a model similar to our original equation that was 90% accurate at predicting survival with a biventricular circulation among neonates with AS and a mitral valve area z-score >-2.

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    • "z scores are used to diagnose aortic dilations and aneurysms, particularly in children. A z score represents standard deviation from the mean aortic diameter, normalized for the patient's age and body surface area [12, 13]. Aortic root dilation is associated with risk factors of coronary artery disease and predicts cardiac failure, stroke, and all causes of cardiovascular mortality [9]. "
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    • "The availability of normal controls and the publication of outcome studies based on the methods used in earlier studies may have argued for the use of this approach in the 2005 guideline paper by Lang et al. [11] On the other hand, early pediatric echocardiographic investigations measured aortic dimensions at maximum expansion (i.e., at the time of peak aortic wall stress) because of the thought that this measurement would be more predictive of the risk for aortic dissection, a relevant worry in children with aortic enlargement. Children’s Hospital Boston group suggested normal control values [19] and measurement method were developed without an awareness of the technique of Roman et al. Some important pediatric studies have utilized the Boston Childrens’/pediatric technique, including an ongoing study on Marfan syndrome [20,21]. "
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    • "The Rhodes score involves the variables of body surface area, indexed aortic root size, left ventricle to heart long axis ratio and indexed mitral valve area (Rhodes et al., 1991). In the Colan score, the equation is based on body surface area, aortic annulus z-score, left ventricle to heart long axis ratio and degree of endocardial fibroelastosis (Colan et al., 2006). The Univentricular Repair Survival Advantage Tool is based on morphologic, functional and pathologic information; the exact equation is not published but the score can be calculated at the freely accessible Congenital Heart Surgeons' Society website "

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