The Journal of Pediatrics
Volume 151, Issue 5 , Pages 554-555, November 2007

Most patients with a moderate ventricular septal defect will not require intervention

Cincinnati Children’s Medical Center, Cincinnati, Ohio

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Kleinman CS, Tabibian M, Starc TJ, Hsu DT, Gersony WM. Spontaneous regression of left ventricular dilation in children with restrictive ventricular septal defects. J Pediatr 2007;150:583-6 

Question 

In children and adolescents with moderate to large pressure-restrictive ventricular septal defect (VSD), what is the natural history of the left ventricular (LV) dilation associated with this defect? Is interventional management of such defects warranted?

Design 

Cohort study.

Setting 

Single cardiology division at a university hospital.

Participants 

Patients (n = 70) with a moderate to large VSD were identified. Thirty-three of these patients had LV dilation (LV end-diastolic dimension [LVED] z score ≥2) at the time of enrollment and had serial clinical and echocardiographic follow-up for >2 years.

Outcomes 

Evidence of persistent or progressive LV dilation; signs or symptoms of congestive heart failure (CHF), failure to thrive (FTT), or pulmonary hypertension (PAH); and acquired ventricular outflow obstruction or aortic regurgitation. LVED z scores at enrollment were compared with those at the latest follow-up.

Results 

Mean age at enrollment was 4.6 ± 3.2 years, and mean duration of follow-up was 7.8 ± 4 years (range, 2.8-22 years), during which the mean LVED z score decreased from 3 ± 0.6 to 1.2 ± 1.3 (P < .01). LVED z score decreased in 29 of the 33 patients and decreased to <2 in 26 of these 29 patients (79%).

Conclusions 

Most patients with pressure-restrictive VSD with moderate-to-severe LV dilation without CHF, FTT, or PAH will experience spontaneous resolution of LV dilation and can avoid cardiac surgery or catheter-based intervention.

Commentary 

Natural history studies provide a foundation that supports the development of medical and surgical therapies. A therapeutic intervention is judged worthwhile (that is, effective, safe, and beneficial) when its outcomes are superior to outcomes that occur without treatment. Seminal natural history studies of congenital heart disease were published in 1977 and 1993 and are required reading for any physician or surgeon who cares for children with heart disease.1, 2 Kleinman et al now provide important, new natural history data to guide our treatment of children with a ventricular septal defect (VSD). They reported 70 patients with a moderate VSD (60 membranous defects and 10 muscular-type defects) who were observed without intervention for an average of 7.8 years. At presentation, all children had LV volume overload consistent with at least a moderate shunt, but none had symptoms or pulmonary hypertension that might mandate an early intervention. During follow-up, virtually all children experienced spontaneous diminution in VSD size and improvement in LV volume overload. Of particular interest was a subgroup of 33 children whose initial LV diastolic dimension was >2 SDs higher than the mean. In these children, LV volume overload improved spontaneously by 60% during the follow-up period. Kleinman et al conclude that children with a moderate-sized VSD and volume overload, who do not have symptoms or pulmonary hypertension, are best treated conservatively and rarely require intervention. As the field of pediatric cardiology continues its rapid movement toward earlier surgical repairs and aggressive transcatheter interventions, it becomes even more essential that surgeons and interventional cardiologists be keenly aware of natural history data. The report by Kleinman and et al will help guide our therapies in the future. Certainly, not all children with a moderate VSD require surgery or device implantation. In many children, the natural history of the defect is preferable to the consequences of either therapy. Just because we can close a VSD does not mean we should.3

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References 

  1. Weidman WH, Blount SG, DuShane JW, Gersony WM, Hayes CJ, Nadas AS. Clinical course in ventricular septal defect. Circulation. 1977;56:I56–I69
  2. Kidd L, Driscoll DJ, Gersony WM, Hayes CJ, Keane JF, O’Fallon WM, et al. Second natural history study of congenital heart defects (Results of treatment of patients with ventricular septal defects). Circulation. 1993;87:I38–I51
  3. Beekman RH. Closing the ventricular septal defect because you can: evidence-averse care?. J Pediatr. 2007;150:569–570

PII: S0022-3476(07)00861-X

doi:10.1016/j.jpeds.2007.09.004

The Journal of Pediatrics
Volume 151, Issue 5 , Pages 554-555, November 2007