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/m 2 (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.
Perimembranous ventricular septal defects (pmVSDs), which include about 70% to 80% of all VSDs, involve the membranous septum and the adjacent area of the muscular septum. Spontaneous and functional closure might occur when the septal leaflet of the tricuspid valve covers the defect. Muscular VSDs are present in 15% of the cases, and 1/2 of them close spontaneously by muscular in-growth. The rare, doubly committed, VSDs, which account for 5% of all VSDs, might also close spontaneously when the right coronary cusp prolapses into the defect. Small VSDs without any hemodynamic repercussion are usually treated conservatively and remain unrepaired. The long-term results of this policy are undetermined. It is unknown whether these patients have a normal life expectancy. However, such information might be critically important with regard to future patients’ employability and insurability. The objective of the present study was to determine the evolution and mid-term outcome of young adults with a perimembranous VSD (pmVSD) that was still open at 16 years of age.
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 pmVSD was unrepaired at 16 years of age were suggested to have a pulmonary output/systemic output (Qp/Qs) ratio of <1.5:1. Patients in whom the VSD closed spontaneously or was closed surgically before 16 years of age and patients who had associated complex congenital anomalies were not included in the present study. The institutional ethics committee of the hospital approved the study protocol.
The patient files were primarily reviewed for demographic data and events after the age of 16 years. The events were defined as death, spontaneous VSD closure, surgical VSD closure, percutaneous VSD closure, the occurrence of endocarditis, implantation of a pacemaker or implantable cardioverter-defibrillator.
Secondarily, the electrocardiographic and transthoracic echocardiographic data were collected. We looked for heart rate, atrioventricular conduction time, QRS duration, type of QRS morphology, and type of rhythm (sinus rhythm or not). The left ventricular ejection fraction (obtained by M-mode and the Teichholz formula), left ventricular diameters (obtained by M-mode through a parasternal long-axis view) and volumes (automatically calculated by the software of the echocardiographic machine from the M-mode data), stroke volume, valve function, and pulmonary hypertension (defined as a pulmonary acceleration time of <100 ms on pulsed wave Doppler or a tricuspid valve regurgitation velocity of >30 mm Hg) were assessed.
Continuous variables are reported as the mean ± SD. If a non-normal distribution was present, the data were plotted as the median and interquartile range (IQR). Proportions are reported as the numbers and percentages. Continuous variables and the proportions between baseline and the latest follow-up were compared using the paired t test and Fischer’s exact test or chi-square test, respectively. The patient outcome is displayed using a Kaplan-Meier survival curve. p values <0.05 were considered statistically significant. All statistical analyses were performed using Statistical Package for Social Sciences, for Windows, version 16 (SPSS, Chicago, Illinois).
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 .
| Variable | At Inclusion (n = 220) | At Latest Follow-Up (n = 220) |
|---|---|---|
| Gender | ||
| Male | 119 | 119 |
| Female | 101 | 101 |
| Age (years) | ||
| Median | 18 | 27 |
| Interquartile range | 7 | 11 |
| Length (cm) | 170 ± 11 | 171 ± 11 |
| Weight (kg) | 66 ± 13 | 71 ± 14 |
| Body surface area (m 2 ) | 1.76 ± 0.21 | 1.83 ± 0.22 |
| Systolic blood pressure (mm Hg) | 125 ± 15 | 127 ± 16 |
| Diastolic blood pressure (mm Hg) | 76 ± 10 | 76 ± 11 |
| Down syndrome | 2% | |
| Echocardiographic data | ||
| Bicuspid aortic valve | 1% | |
| Aortic valve prolapse | 2% | |
| Subaortic stenosis | 1% | |
| Mild mitral valve prolapse | 1% | |
| Mild double chambered right ventricle | 1% | |
| Hemodynamic data (n = 71 ⁎ ) | ||
| Right atrium (mm Hg) | 4 ± 3 | |
| Systolic pulmonary artery pressure (mm Hg) | 24 ± 9 | |
| Diastolic pulmonary artery pressure (mm Hg) | 8 ± 5 | |
| Mean pulmonary artery pressure (mm Hg) | 15 ± 6 | |
| Wedge pressure (mm Hg) | 9 ± 3 | |
| Pulmonary cardiac output/systemic cardiac output ratio | 1.2 ± 0.2 |
⁎ 166 patients underwent right heart catheterization, of whom 71 had data electronically available for review.
Two patients (1%) died during the follow-up period. One patient, with congestive cardiomyopathy before closure, died suddenly 8 months after successful percutaneous VSD closure, with ventricular fibrillation the most likely cause of death. The second patient, with Down syndrome and congestive cardiomyopathy, died from end-stage heart failure. In 15 patients (7%), the VSD was closed (8 surgically and 7 percutaneously) because of progressive volume overload. The defect closed spontaneously in 8 patients (4%). The corresponding Kaplan-Meier curves are plotted in Figures 1 and 2 . Endocarditis occurred in 8 cases (4%). All 8 cases were cured with antibiotics, and surgical intervention was not needed.
In 203 patients, the defect remained unclosed. Of these patients, 93% were in sinus rhythm at inclusion. During follow-up, 1 patient required pacemaker implantation because of atrioventricular block, and 1 developed a junctional atrioventricular rhythm. An automatic implantable cardioverter-defibrillator was implanted in 1 patient who survived ventricular fibrillation. At inclusion, 77% of the patients had normal QRS morphology. The remaining 25% were characterized by left or right bundle branch block or left or right hemiblock or had signs of left or right ventricular hypertrophy. The electrocardiographic changes are summarized in Table 2 .
| Variable | At Inclusion (n = 203) | At Latest Follow-Up (n = 203) | p Value |
|---|---|---|---|
| Electrocardiographic | |||
| Heart rate (beats/min) | 71 ± 16 | 69 ± 14 | 0.187 ⁎ |
| Atrioventricular conduction time (ms) | 144 ± 22 | 146 ± 24 | 0.199 ⁎ |
| QRS duration (ms) | 94 ± 14 | 98 ± 16 | 0.004 ⁎ |
| Prevalence sinus rhythm | 99% | 98% | 0.015 † |
| Prevalence normal QRS morphology | 77% | 72% | 0.0001 † |
| Echocardiographic | |||
| Left ventricle end-diastolic diameter (mm) | 49 ± 5 | 49 ± 7 | 0.348 ⁎ |
| Left ventricle end-systolic diameter (mm) | 35 ± 5 | 34 ± 6 | 0.003 ⁎ |
| Left ventricle end-diastolic volume index (ml/m 2 ) | 65 ± 19 | 66 ± 24 | 0.691 ⁎ |
| Left ventricle end-systolic volume index (ml/m 2 ) | 25 ± 10 | 22 ± 11 | 0.017 ⁎ |
| Left ventricle ejection fraction (%) | 62 ± 7 | 67 ± 8 | 0.0001 ⁎ |
| Stroke volume index (ml/m 2 ) | 41 ± 11 | 44 ± 15 | 0.035 ⁎ |
| Prevalence degree mitral valve regurgitation (x/4) | 0.0001 ‡ | ||
| 0 | 67% | 38% | |
| 1 | 26% | 50% | |
| 2 | 4% | 8% | |
| 3 | 3% | 2% | |
| 4 | 0% | 1% | |
| Prevalence degree aortic valve regurgitation (x/4) | 0.0001 ‡ | ||
| 0 | 84% | 74% | |
| 1 | 15% | 23% | |
| 2 | 1% | 3% | |
| 3 | 0% | 0% | |
| 4 | 0% | 0% | |
| Prevalence degree tricuspid valve regurgitation (x/4) | 0.0001 ‡ | ||
| 0 | 30% | 10% | |
| 1 | 64% | 78% | |
| 2 | 4% | 9% | |
| 3 | 2% | 3% | |
| 4 | 0% | 0% | |
| Prevalence pulmonary arterial hypertension | 3% | 9% | 0.002 † |
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