Prognostic Implications of Initial Echocardiographic Findings in Adolescents and Adults with Supracristal Ventricular Septal Defects

Abstract

Although surgery is recommended for pediatric patients with supracristal ventricular septal defects (sVSDs) to prevent progression of aortic regurgitation (AR), outcomes in adolescents and adults with sVSDs are not known. In this retrospective observational study, clinical data without surgery were obtained in 60 patients with sVSDs (group 1; mean age, 36 ± 13 years), 120 age- and defect size-matched patients with perimembranous ventricular septal defects (group 2), and 52 patients with sVSDs who underwent surgery (group 3; mean age, 32 ± 11 years). Aortic sinus wall prolapse (38% vs 3%, P < .0001) and moderate to severe AR (7% vs 0%, P= .012) were more frequently observed in group 1 than in group 2. Five, three, and two patients in group 1 had surgery during follow-up because of rupture of the aneurysm of the sinus of Valsalva, endocarditis, and heart failure, respectively. Group 1 had a lower 12-year clinical event-free (surgery and endocarditis) rate (76 ± 9% vs 94 ± 4%, P= .031) but an equivalent overall survival rate (100% vs 94 ± 3%, P= .143) compared with group 2. Patients with maximal prolapsing aortic sinus wall length > 7 mm showed a higher frequency of aneurysm of the sinus of Valsalva rupture than those with no prolapse or maximal prolapsing length ≤7mm (80% [four of five] vs 2% [one of 55], P< .001). The event-free and overall survival rates were comparable between groups 1 and 3, with equivalent 10-year AR progression-free survival rates (94 ± 5% vs 91± 5%, P= .301). Aneurysm of the sinus of Valsalva rupture, rather than AR progression, was the main clinical event. Watchful monitoring of patients with high-risk echocardiographic features may be a rational option.