Background: Combined aortic valvular stenosis (AVS) and coarctation of the aorta (CoA) is uncommon. There are only a few case reports that discuss the treatment choices and prognosis. We present the immediate and long-term results for a group of children with combined AVS and CoA who underwent sequential percutaneous balloon dilatation in a single catheterization procedure.
Patients And Methods: The cases of 13 children with combined AVS and CoA who underwent balloon dilatation in a single catheterization session between August 1995 and May 2002 were retrospectively evaluated. The group was comprised of 9 boys and 4 girls of mean age 14.9 +/- 24.2 months (range = 19 days to 7 years).
Results: The pressure gradients at the valvular level before and after the intervention were 51.5 +/- 22.3 mmHg (range = 8 to 85 mmHg) and 22.4 +/- 18.3 mmHg (range = 2 to 57 mmHg), respectively (p < 0.001). The corresponding findings for the coarctation segment were 22.3 +/- 13.5 mmHg (range = 0 to 45 mmHg), and 5.2 +/- 7.0 mmHg (range = 0 to 24 mmHg; p < 0.001). After the intervention, mild aortic regurgitation occurred in 5 children (38.5%). Mild aortic regurgitation became moderate in 1 patient (7.7%). Three patients developed peripheral arterial occlusion treated with heparin and streptokinase after intervention. There were no deaths during or early after the procedures. Four patients (30.8%) died, all in the first 6 months after the intervention, and the mean follow-up time for the 9 survivors was 57.6 +/- 38.9 months (range = 6 to 107 months). Recurrence of stenosis and coarctation occurred in 2 (15.4%) and 4 (30.8%) cases, respectively. Four patients (30.8%) underwent cardiac surgery. The event-free survival rates were 76.9% at 6 months, 61.5% at 12 months through 24 months, and 30.8% at 60 months. The overall survival rates were 76.9% at 3 months, 69.2% at 6 months, and it remained 69.2% for the rest of the follow-up period. NOTE: Outcomes for 13 patients with combined AVS and CoA who underwent single-session sequential balloon dilatation are described. The results were favorable; there were no severe complications related to the procedures, and no deaths occurred during or in the early period after the intervention.
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Thorac Cardiovasc Surg
January 2025
Pediatric Cardiology, University Hospital Tuebingen, Tubingen, Germany.
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Institute of Cardiovascular Sciences, University of Birmingham, Birmingham B15 2TT, UK.
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Background: Neurofibromatosis type 1 (NF1) is an autosomal dominant genetic disorder affecting multiple systems. However, arterial stenosis is a rare manifestation in patients with NF1. Since the symptoms of arterial stenosis caused by NF1 are often atypical and have a high under-diagnosis rate, this can lead to serious complications such as hypertension, ischemic stroke, or even death.
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