Supracristal ventricular septal defect in adults: Is it time for a paradigm shift?

Background To determine the risk and predictors of progression of aortic regurgitation (AR) and other adverse events (AE) in adults with supracristal ventricular septal defect (VSD). Methods Retrospective observational study of 62 adults with supracristal VSD followed at the Mayo Clinic from 1994 to 2013. Freedom from AR progression was compared by age and racial groups (Asian vs non-Asian). Predictors of AR progression were determined using Cox proportional hazard model. Composite AE endpoint was defined as AR progression, endocarditis, aneurysm of sinus of Valsalva (aSOV), and rupture of aSOV. Risk of AE was compared between group 1 (no surgical intervention) and group 2 (surgical intervention). Results Sixty-two patients aged 47(SD 12) years were followed for 13 (SD 5) years. Group 1 = 42/62 and group = 20/62. Endpoint of AR progression and aSOV occurred in 13% and 20% respectively. Freedom from AR progression for the entire cohort was 88%, 86% and 86% at 5, 10 and 15 years respectively. Risk of AR progression was significantly lower in patients (aged ≤ 40 years, p = 0.008) but similar between Asians and non-Asians (p = 0.57). Age ≤ 40 years was an independent predictor of AR progression (hazard ratio [HR] 3.5, 95% CI 2.3-5.4, p = 0.001). Composite AE endpoint occurred in 33% and 40% of group1 and 2 cohorts respectively, p = 0.69. Conclusion Adults with supracristal VSD are at lower risk for AR progression but higher risk for aSOV formation compared to historical pediatric cohorts. Younger age was a predictor of AR progression. Surgical intervention had no protective effect on complication rate.