Importance of (perimembranous) ventricular septal aneurysm in the natural history of isolated perimembranous ventricular septal defect.

Records were reviewed of 247 patients found to have isolated perimembranous ventricular septal defect (VSD) on cross-sectional echocardiography. Patients were separated into 2 groups: those in whom a perimembranous ventricular septal aneurysm (VSA) was associated with the VSD (group A, 77% of patients) and those in whom a VSA was not present (group B, 23%). The VSD was assessed by clinical (125 in group A, 24 in group B) and hemodynamic criteria (65 in group A, 33 in group B). The median follow-up period was 27 months (range 3 months to 25 years). In group A, the VSD closed spontaneously in 11% of the patients, improved clinically in 33% and required surgical closure in 11%. In group B, the VSD closed spontaneously in only 2%, improved clinically in 16% and required surgical closure in 47%. When considering larger VSDs only, 28% required surgery in group A, whereas 84% required surgery in group B (p less than 0.001). The VSA was found at the first echocardiographic examination in 94% of the studies. Thus, VSA is found in a large proportion of patients with perimembranous VSD. It occurs as an early phenomenon and is an important mechanism of closure. The presence of a VSA appears to confer a more favorable prognosis in perimembranous VSD except in patients with Down's syndrome.