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

 Serial images of a case. (A) Left ventricular angiography shows the severe aortic valvular stenosis;
(B) Balloon aortic valvuloplasty;
(C) coarctation segment at the right anterior oblique view of the aorta;
(D) balloon coarctation angioplasty
(E) aortography after the interventions.
Sequential Balloon Dilatation for Combined Aortic Valvular Stenosis and Coarctation of the Aorta in a Single Catheterization Pro
Sequential Balloon Dilatation for Combined Aortic Valvular Stenosis and Coarctation of the Aorta in a Single Catheterization Pro
Author(s): 

Kursad Tokel, MD, Selman Vefa Yildirim, MD, Birgul Varan, MD, Enver Ekici, MD

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 < 0.05 were considered statistically significant.

Results
The mean pressure gradients at the valvular level before and after intervention were 51.5 ± 22.3 mmHg (range = 8–85 mmHg) and 22.4 ± 18.3 mmHg (range = 2–57 mmHg), respectively (p < 0.001). The corresponding findings for the coarctation segment were 22.3 ± 13.5 mmHg, (range = 0–45 mmHg) and 5.2 ± 7.0 mmHg (range = 0–24 mmHg), respectively (p < 0.001).
During intervention, 1 patient developed ventricular fibrillation due to digoxin intoxication. This patient had been digitalized at another center, and the serum digoxin level measured at our center was 5.2 µg/dL. One patient developed cardiac arrest at the beginning of the catheterization. After the intervention, 5 children (38.5%) exhibited mild aortic regurgitation, and 1 patient (7.7%) exhibited mild regurgitation that progressed to severity. Three patients developed peripheral arterial occlusion and received anticoagulation and fibrinolytic therapy (heparin and streptokinase). During long-term follow-up, 1 child required an iliofemoral bypass graft operation. None of the patients died during or early after intervention.
Four patients died all within the 6 months of the procedure (details about the deaths below). Excluding these 4 cases, the mean follow-up time was 57.6 ± 38.9 months (range = 6–107 months). Two (15.4%) of the 13 patients developed recurrent aortic valve stenosis during follow-up. One of these children underwent two separate reinterventions to address this, and the second attempt was unsuccessful. The recurrence of coarctation was detected in 4 patients (30.8%) at 2, 4, 5, and 8 months of follow-up, respectively. The pressure gradients measured at the coarctation segment using CW Doppler echocardiography ranged from 25–80 mmHg. All of these patients underwent reintervention, and the attempt was unsuccessful in 2 cases. Four patients (30.8%) underwent cardiac surgery during the follow-up period. In one case, subaortic fibromuscular-type stenosis was diagnosed 12 months after the intervention, and myectomy was performed. Another patient required coarctation surgery 12 months after the intervention and also required aortic valve replacement (AVR) at 54 months due to severe aortic regurgitation. The third patient underwent a Konno procedure with AVR 36 months after the intervention and the fourth child underwent AVR for severe aortic stenosis 60 months after the intervention.
As noted above, 4 patients (30.8%) died. Three deaths occurred in the first 3 months after the intervention, and the causes of death were meningitis and severe heart failure. The fourth patient died 5 months after the intervention, and the cause in this case was severe gastroenteritis. The 2 patients who died as a result of extracardiac problems also had endocardial fibroelastosis and severe heart failure at the time of death.
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 survival rates were 76.9% at 3 months, 69.2% at 6 months, and 69.2% for the rest of the follow-up period. Table 2 summarizes the events, outcomes and follow-up after intervention.


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