Abstract

Background: Congenital mitral valve disease (CMVD) presents major challenges regarding medical and surgical management. Three dimensional echocardiography (3DE) allows precise delineation of area tracing from cross-sectional planes for more precise measurements and outcome analysis. However, there are no available data to inform clinicians on how 3DE can help guide management. We hypothesized that quantitative 3DE measurements can provide improved risk stratification and guide management of CMVD. Methods and Results: Demographic, procedural and outcome data were obtained for all 183 children who underwent MV reconstruction for CMVD from 2002 to 2017. 92 patients had mitral stenosis (MS), 68 mitral regurgitation (MR), and 23 mixed disease (MD). 13 patients (7%) died. MD patients had higher rates of mortality, reoperation, and valve replacement. Quantitative 3DE measurements of the effective orifice area (EOA) and vena contracta regurgitant area (VCRA) were determined preoperatively and before discharge. Changes from baseline in EOA and VCRA were significant independent predictors of reoperation with excellent area under the curve (AUC) values (EOA: AUC = 0.888; VCRA: AUC = 0.962; both P<0.001). As shown in Figure 1, decision analysis revealed that an increase in EOA<30% for patients with MS is associated with 76% risk of reoperation (HR=11.4; 95% CI: 3.4-37.8; P<0.001), whereas a decrease in VCRA <40% for MR patients is associated with 93% risk of reoperation (HR=18.1; 95% CI: 2.3-142; P=0.006). Comparison of the AUC indicated that the change in EOA is a significantly stronger predictor of reoperation than change in mean gradients (MG; P=0.018; DeLong’s test). Conclusions: Reconstructive surgery for CMVD can be performed with good outcomes. 3DE EOA and VCRA aids in postoperative clinical and surgical management. Further, 3DE is more predictive of patient outcomes than 2DE measurements of MG. Thus, we now incorporate 3DE EOA and VCRA into clinical decision making.

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