Serial changes in patients with isolated VSD during adolescence have not previously been investigated. Hemodynamic status, diameter of the defect, and growth were studied yearly in 106 children with VSD. The mean duration of the follow-up was 13.16 years and ranged in 80% of subjects from 7 to 19 years (1395 patients years). The mean ages at pre- and postpuberty were 8.62 and 16.67, respectively. The presented longitudinal study, in which losses due to death and operation were minimal (4%), ideally reflected the natural history of VSD. Although weight showed retardation during prepuberty, this lag was caught up by the end of adolescence. Stature showed no retardation in pre- and postpuberty. Cardiothoracic ratio decreased significantly from a mean of 0.48 to 0.44 and showed normal variation. Although the mean defect diameter at prepuberty was 5.33 mm, this decreased to 2.7 postpubertally. The individual decrease (1.7 +/- 2.34 mm) was significant (t = 5.349, p < 1/10(5)). The defect closed spontaneously in 24 (22.6%). In the 75 patients without pulmonary hypertension and with mild left-to-right shunting, 52 remained in the same class and spontaneous closure was observed in 23. In the 24 patients with moderate to severe left-to-right shunt, this decreased in 23 and only one remained stable. The 2 patients (1.9%) with Eisenmenger syndrome remained stable and 1 died. Aortic regurgitation developed in 10 patients (9.4%); however, this was of mild degree in most of them. No infective endocarditis was observed. It is concluded that patients with VSD should be followed closely through adolescence, because the diameter of the defect, as well as left-to-right shunting, can decrease, and it is concluded that the spontaneous closure of the defect is to be expected in a considerable 23%, and aortic prolapse or mild regurgitation may develop in approximately 10%.

Aortic valve prolapse (AVP) was detected in 82 (7.5%) of 1096 patients with ventricular septal defect (VSD) (in 50 at initial echocardiographic examination and in 32 at follow-up) by echocardiography. Of 82 patients with AVP, aortic regurgitation (AR) was detected in 53 (65%) (in 28 at initial echocardiographic examination and in 25 at follow-up), resulting in an incidence of AR of 4.8% (of VSD). The percentage of AVP (20.8%) and AR (16.7%) in muscular outlet VSDs was larger than the percentage of AVP (10.6%) and AR (6.8%) in perimembranous VSDs (p <0.05). Fourty-four patients were followed medically after AVP appeared (3 months to 10.8 years; median, 2.1 years). Initially, there was no AR in 24 of these patients, trivial AR in 7, and mild AR in 13. Trivial AR developed in 6 (25%) and mild AR developed in 3 (13%) of 24 patients who had no AR (in 5 of them within 1 year and in 9 of them within 2 years). In 2 (29%) of 7 patients, trivial AR progressed to mild AR during a median of 2 years, and in 4 (31%) of 13 patients, mild AR progressed to moderate AR during a median of 1.1 years. We recommend frequent echocardiographic evaluation (every 6 months) for detecting of appearance of AR in patients with perimembranous or muscular outlet VSD after AVP develops and for evaluating the progression of AR in patients with perimembranous or muscular outlet VSD, AVP, and trivial AR. In addition, we recommend surgical intervention in patients with perimembranous or muscular outlet VSD, AVP, and mild AR because of rapid progression of mild AR to moderate AR.

The medical records of 685 patients with ventricular septal defect (VSD) were reviewed to determine spontaneous closure, left ventricular-to-right atrial shunt, subaortic ridge, and aortic valve prolapse. Patients had been followed for a mean of 3 +/- 2.5 years and median 2.2 years by echocardiography. VSD was perimembranous in 65.7% (450), trabecular muscular in 30.8% (211), muscular outlet in 2.3% (16), muscular inlet in 0.7% (5), and doubly committed subarterial in 0.5% (3). Defect size was classified in 76% (517) as small, in 18% (124) as moderate, and in 6% (44) as large. VSD closed spontaneously in 27% (186 of 685 patients) by ages 40 days to 13.7 years (mean, 2.1 +/- 2.2 years; median, 1.3 years). Sixty-six of 450 perimembranous defects (15%) and 120 of 211 trabecular muscular defects (57%) closed spontaneously (p <0.001). Defect size became small in 15% of patients with VSD at mean 2.9 +/- 2.3 years (median, 2.3 years). Aneurysmal transformation was detected in 56% (254), left ventricular-to-right atrial shunt in 8.4% (38), subaortic ridge in 5.8% (26), aortic valve prolapse in 11.7% (53), and aortic regurgitation in 7.3% (33) of 450 patients with perimembranous defect. There was no statistical significance between the age at closure and the age of detection of aneurysmal transformation in the patients with perimembranous defect (p = 0.25).