Congenital heart disease
Behavior of Unrepaired Perimembranous Ventricular Septal Defect in Young Adults

https://doi.org/10.1016/j.amjcard.2009.09.047Get rights and content

The number of adolescents and young adults with congenital heart defects, including ventricular septal defect (VSD), increases continuously. We evaluated the mid-term outcome of small and unclosed perimembranous VSDs (pmVSDs). All patients with a known unrepaired pmVSD at 16 years of age were selected from our database. The clinical, electrocardiographic, and echocardiographic changes between baseline and the latest follow-up examination were compared. A total of 220 patients (119 males, median age 18 years, interquartile range 7) could be included. During a median follow-up of 6 years (interquartile range 4, range 38), 2 patients died (1%; 1 from sudden death and 1 from end-stage heart failure). Endocarditis occurred in 8 patients (4%). One patient required pacemaker implantation (0.5%) and one required implantable cardioverter-defibrillator implantation (1%). Fifteen patients (7%) required a closing procedure. In 8 patients (4%), the pmVSD closed spontaneously. In the remaining 203 patients (93%), the QRS morphology changed in 5% and 1% lost sinus rhythm (p = 0.0001 and p = 0.015, respectively). The left ventricular ejection fraction and stroke volume index increased from 62 ± 7% to 67 ± 8% and from 41 ± 11 to 44 ± 15 ml/m2 (p = 0.0001 and p = 0.035, respectively), the end-systolic diameter decreased, and the end-diastolic diameter did not change. Finally, patients with an open pmVSD developed more pulmonary arterial hypertension during follow-up (from 3% to 9%, p = 0.002). In conclusion, mid-term follow-up of adolescents and young adults with a small and unrepaired pmVSD was not uneventful. Some patients required intervention, but in others, spontaneous closure occurred. Electrocardiographic and structural changes were noticed, for which the clinical significance needs to be determined.

Section snippets

Methods

All patients, registered with an unrepaired pmVSD at 16 years of age, were selected from our database of pediatric and congenital cardiology. It contains >20,000 patients with congenital heart defects. Of these patients, 7,000 are >16 years old. Our department of pediatric and congenital cardiology yearly performs >300 interventional catheterizations, and >200 patients are referred to the congenital cardiac surgeon. Only restrictive VSDs were allowed to be included. All patients in whom the

Results

A total of 220 patients were included in the present study. The median age at inclusion was 18 years (IQR 7). The patients (119 males, male/female ratio 1.18:1) were followed for a median of 6 years (IQR 4, range 38). At the end of the study, the median age of the study cohort was 27 years (IQR 11). The demographic clinical, echocardiographic, and hemodynamic characteristics are summarized in Table 1.

Two patients (1%) died during the follow-up period. One patient, with congestive cardiomyopathy

Discussion

We found that the course of patients with an unclosed and small pmVSD at the age of 16 years was not uneventful. Although the pmVSD closed spontaneously in some patients, others required surgical or interventional repair at later follow-up. Moreover, patients with persistent open pmVSD during follow-up developed significant echocardiographic and electrophysiologic changes.

We observed spontaneous pmVSD closure in 4% of our patients >16 years. Of all VSDs 11% to 70.8% close spontaneously during

References (13)

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