The purpose of the study was to assess the long-term outcome of patients with small
ventricular septal defects (VSDs) considered not to require surgical closure during
childhood.
Although patients with small VSDs have generally been considered not to require surgery,
more recent data suggest that a significant percentage of these patients develop serious
problems during adult life.
A total of 229 consecutive patients (115 females) with a VSD considered too small
to require surgery during childhood as defined by normal pulmonary artery pressure,
less than 50% shunt, pulmonary vascular resistance < or =200 dynes x s cm(-5), no
VSD-related aortic regurgitation (AR), and no symptoms and who had no additional hemodynamically
relevant heart defect were followed in an adult congenital heart disease program.
Physical examination, electrocardiography, and echocardiography were performed in
all patients in one- to three-year intervals; exercise tests and Holter monitoring
were performed in 140 and 127 patients, respectively.
Follow-up was completed in 222 patients (97%). Mean age at last visit was 30 +/- 10
years. Spontaneous VSD closure was observed in 14 patients (6%). No patients died,
four patients (1.8%) had an episode of endocarditis, of whom two required aortic valve
replacement, and one additional patient (0.4%) had surgical closure for hemodynamic
reasons. For 118 patients who entered the study between 1993 and 1996 and were prospectively
followed for 7.4 +/- 1.2 years, event-free survival with end points defined as death,
endocarditis or heart surgery was 99.1 +/- 0.8% at three years, 96.5 +/- 1.7% at six
years and 95.5 +/- 1.9% at eight years. At last visit, 94.6% of all patients studied
were symptom free. Left ventricular (LV) size by echocardiography was normal in 198
(89%) patients, borderline in 23 patients and definitely enlarged in only one patient.
None had systolic LV dysfunction, and pulmonary artery pressure (PAP) was normal in
all patients. Mean exercise capacity was 92 +/- 21% of expected, and 87% of patients
had no arrhythmias on Holter monitoring, with the remainder showing benign rhythm
disorders.
Outcome in well-selected patients with a small VSD is good. Surgical closure does
not appear to be required during childhood as long as left-to-right shunt is <50%
and signs of LV volume overload are absent, when PAP is not elevated, and no VSD-related
AR or symptoms are present.