Sequential Balloon Dilatation for Combined Aortic Valvular Stenosis and Coarctation of the Aorta in a Single Catheterization Pro

Combined aortic valvular stenosis (AVS) and coarctation of the aorta (CoA) is uncommon.^3–5 This combination was detected in 7% of a large group of children with CoA who underwent balloon angioplasty.² The long-term results of percutaneous balloon dilatation for isolated AVS and isolated CoA are favorable, and this treatment is widely used today, particularly for palliation.^3–5 In 1987, Pan et al.⁶ reported the initial results for 2 patients with combined AVS and CoA who were treated with sequential balloon dilatation during a single catheterization. Since then, only a few case reports have documented this combination of disorders and discussed treatment options and prognosis.^7–9 Here we present the immediate and long-term outcomes for a group of children with combined AVS and CoA who underwent percutaneous balloon dilatation in a single catheterization procedure. Patients and Methods We retrospectively evaluated 13 consecutive cases of children with combined AVS and CoA who underwent balloon dilatation in a single catheterization between August 1995 and May 2002. The group comprised 9 boys and 4 girls of mean age 14.9 ± 24.2 months (range = 19 days to 7 years). Only one of the patients was younger than 30 days. All the children had a bicuspid aortic valve, 1 also had a ventricular septal defect (VSD) and patent ductus arteriosus (PDA), 1 had an atrial septal defect (ASD), and 1 had a vascular ring anomaly. Four patients (30.8%) had signs and symptoms of severe heart failure, 6 (46.1%) had specific symptoms such as syncope or chest pain, and 3 (23.1%) were asymptomatic. The mean values for left ventricular ejection fraction (EF) and fractional shortening were 70.1 ± 17.6% (range = 26–88%) and 39.2 ± 11.6% (range = 14–55%), respectively. Four patients (30.8%) had diminished left ventricular systolic function and 2 (15.4%) showed endocardial fibroelastosis on echocardiography. The peak instantaneous pressure gradients determined by CW Doppler echocardiography at the aortic valve and at the coarctation segment were 59.1 ± 22.3 mmHg (range = 6–98 mmHg) and 26.0 ± 10.6 mmHg (range = 15–43 mmHg), respectively. Procedural technique. Each patient was premedicated with oral midazolam and sedated with ketamine. Local anesthesia was administered, and a femoral arterial sheath was introduced percutaneously. Once arterial cannulation was completed, heparin (100 U/kg) was administered. An open-tip catheter was then inserted and the pressure gradient in the coarctation segment was measured and long-axis view aortography was performed. The diameters of the aortic valve annulus and diaphragmatic portion of the aorta were measured on the aortogram. Then, the catheter was advanced through the stenotic aortic valve into the left ventricle using a soft-tip guidewire in most cases. The selected valvuloplasty catheter, the Tyshak-II® or the mini-Tyshak® (NuMed Canada, Inc.) was advanced over the guidewire and inflated at the level of the valve orifice. For each case, the appropriate-sized balloon was established when the diameter of the aortic valve annulus, divided by the diameter of the balloon, was approximately equal to 1 (accepted range = 0.75–1.1). The length of balloon chosen varied with the size of the patient: neonates and young infants = 2 cm; older infants and young children = 3 cm. In 10 of the 13 patients, the same balloon was used to treat both AVS and CoA. In these cases, the balloon was deflated after valvuloplasty, then withdrawn to the level of the coarctation segment and reinflated. The other 3 patients each required 2 different-sized balloon catheters. In these cases, aortic valvuloplasty was carried out first, and then that catheter was removed and a different-sized balloon catheter was introduced and inflated at the coarctation segment (Figures 1 A–E). At the end of the procedure, aortic root angiography was performed and pressure pullback tracings across the aortic valve and at the coarctation segment were obtained to evaluate aortic insufficiency and any residual coarctation. Patients with no complications were discharged the next day. The pressure gradients measured before and after intervention were compared. Some details of the procedure are listed in Table 1. Statistical analysis. The data were analyzed and compared by the Student’s t-test in SPSS for Windows software (Version 11.0). P-values p p 3,11 Most patients with AVS and CoA also have a bicuspid aortic valve. These two defects are also known to be associated with other cardiac lesions, such as Shone complex, VSD and patent ductus arteriosis (PDA). All 13 patients in our series had a bicuspid aortic valve. Coexistence of AVS and CoA is relatively uncommon, but the exact prevalence is unknown.^3,10–12 In left ventricular obstructive diseases such as combined AVS and CoA, the wall of the left ventricle is frequently affected and functionally impaired. Cardiomyopathic changes, with or without endocardial fibroelastosis, may also be detected on echocardiography.¹³ In our series, 4 patients had severe heart failure in early infancy and 2 of those children had endocardial fibroelastosis. Lababidi et al. were the first to report a series of transluminal balloon coarctation angioplasty procedures in 1984.¹⁴ Since then, there have been many reports on the immediate and intermediate results of balloon dilatation for obstructive lesions, and several that have documented long-term results.^3–5,15 Balloon dilatation is known to be safe and palliative for either AVS or CoA, especially in neonates and infants.3–5 Some immediate and late complications related to the procedure have been reported, including aneurysms at the coarcted segment and mild-to-moderate aortic regurgitation.^3–5,16,17 According to some authors, balloon dilatation is preferable to surgery.^4,5,17 Although much information has been published about balloon treatment of AVS and CoA separately, the English literature contains only a few case reports of balloon dilatation for combined AVS and CoA.^6–10 It is difficult to treat different levels of left ventricle obstruction in the same patient and it is not well described as a one-level obstructive disease. The earliest report of this was by Pan and colleagues6 who documented the immediate results for 2 patients who underwent balloon dilatation as treatment for two-level left ventricle obstruction. One of these patients had combined AVS and CoA, and the other had combined discrete subaortic stenosis and CoA. In our study, we retrospectively evaluated outcomes for 13 patients with combined AVS and CoA who underwent sequential balloon dilatation in one session. This is a large series and, to our knowledge, is the first time that immediate and long-term outcomes have been described. In our series, there were no life-threatening complications during or immediately after the intervention. As expected, some patients (6 total) showed mild aortic regurgitation after the procedure but this was not as significant as has been reported previously.⁴ The aortic insufficiency progressed from moderate to severe in 2 patients (15.3%) during long-term follow up (30 and 54 months after intervention, respectively). One of these patients had Shone complex and the other had subaortic stenosis. For patients with AVS alone, Moore et al.¹⁵ and Kuhn et al.¹⁸ reported 13% and 14% respective frequencies of need for surgery to treat aortic regurgitation. In contrast to other findings, none of our patients developed an aneurysm at the coarctation segment after balloon angioplasty.^3,4 The other late complication we observed was subaortic stenosis in 2 patients. The stenosis was fibromuscular type in 1 case, and was caused by a discrete membrane in the other. Both of these children underwent surgery for subaortic stenosis. We found that the rate of subaortic stenosis in our patients with combined AVS and CoA (15.4%) was greater than the rates observed in patients with CoA alone (n = 70, 1.4%; unpublished data) and AVS alone (n = 59, 1.6%; unpublished data) who were treated with balloon dilatation in the same period that our study covered. The mechanism for subaortic stenosis development is still unknown. Rao and coworkers³ found that, after balloon intervention, the rate of restenosis during intermediate-term follow up in children with AVS alone was 23%, and the rate of coarctation recurrence during 8.7 years of follow-up in children who initially had CoA alone was 25%. In the above-mentioned patients at our center who were treated for AVS only and CoA only during the study period, we recorded a 5.1% valvular stenosis recurrence in the AVS-only patients (unpublished data), and a 24.3% rate of coarctation recurrence in the CoA-only patients (unpublished data). In our 13 patients with combined AVS and CoA, coarctation recurrence was more frequent than stenosis recurrence (38.5% versus 15.4%, respectively). In the literature and in our series, recoarctation appears to be more frequent than stenosis recurrence. According to Moore et al.¹⁵ and Kuhn et al.,¹⁸ the frequencies of requirement for repeat intervention for stenosis recurrence in patients with AVS alone were 20% and 32%, respectively. An important issue in the treatment of combined AVS and CoA is the order of the interventional procedures. At which level should the balloon be inflated first — the coarctation segment or the aortic valve? Pan and colleagues⁶ and Ohkubo et al.⁷ suggested that the first step should be dilatation of the coarctation segment. However, we inflated the balloon at the aortic valve level first. This was a technical choice, and none of our patients developed acute or serious hemodynamic problems. In our series, 4 deaths occurred in the first 6 months after the intervention. Two of these children had endocardial fibroelastosis and all of the 4 children had severe heart failure. The causes of death were severe heart failure (2 cases) and severe infection (2 cases). These outcomes suggest that endocardial fibroelastosis and cardiac dysfunction at a very young age are predictors of poor prognosis in patients with combined AVS and CoA. Conclusion In conclusion, our results with single-session sequential balloon intervention for these patient groups were favorable. There were no severe complications and none of the patients died during or in the early period following intervention. The findings indicate that cardiomyopathic changes (endocardial fibroelastosis) in children with combined AVS and CoA signal poor prognosis. The first 6 months after the initial session were most important with respect to the need for repeat procedures and survival. We suggest that sequential balloon dilatation for combined AVS and CoA in one session is a safe and effective procedure for palliation.

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