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Transcatheter Closure of Apical Ventricular Muscular Septal Defect Combined With Arterial Switch Operation in a Newborn Infant

Josep Rode´s, MD, Jean Franc¸ois Pie´chaud, MD, Ruth Ouaknine, MD, Sylvie Hulin, MD, Laurence Cohen, MD, Suzel Magnier, MD, Yves Lecompte, MD, and Thierry Lefe` vre, MD

Introduction

Transposition of the great arteries (TGA) is associated with ventricular septal defects (VSDs) in about 25% of cases. Some of these defects are muscular, which complicates the surgical management of the patients due to difficult defect visualization, high rate of reoperation, and subsequent ventricular dysfunction [1–4]. Moreover, TGA repair should ideally be performed within the first two weeks of life, when the left ventricle is still able to support the systemic circulation. In recent years, some devices have been used for percutaneous closure of ventricular septal defects with encouraging early results [5–10]. However, there are no reports about the efficacy and safety of this percutaneous treatment in the neonatal period.We present the case of a newborn infant with TGA associated with apical muscular VSD who underwent a combined treatment of transcatheter closure of VSD followed by a switch operation within 48 hr.

Case report

A full-term female infant weighing 3.2 Kg was found to be cyanotic at 4 hr from birth with room saturation by pulse oxymetry (room air) at 60%. Echocardiography revealed an L-transposition of the great vessels with normal atrioventricular relation, associated with a large apical ventricular septal defect and moderate aortic arch hypoplasia. At the age of 2 days, after prostaglandin infusion had been started, the patient was referred to our center for angiocardiographic evaluation and treatment.A Rashkind procedure was immediately performed via the right femoral vein, improving oxygen saturation to 80%. Cardiac angiography showed a relatively small right ventricle associated with moderate hypoplasia of the aortic arch without isthmic coarctation, a large patent ductus, normal coronary arteries pattern, an apical ventricular septal defect measured at 4 mm (Fig. 1A), and right-to-left shunting. Pressures were similar in the right and left ventricle. In order to avoid surgical closure of the VSD, a combined procedure was attempted, starting with transcatheter closure of the apical septal defect followed by arterial switch intervention. At the age of 6 days, percutaneous closure of VSD guided by transthoracic echocardiography (TTE) was performed under general anesthesia. Heparin was administered (100 UI/kg).A5 Fr Mullins-type sheath (AGA Medical, Golden Valley, MN) was advanced via the right femoral vein as far as the entrance to the right atrium where the dilator sheath was removed while the lumen connection of the catheter was opened, allowing slight bleeding in order to avoid gas embolism. A 5 Fr end-hole balloon Berman catheter (Arrow International, PA) was inserted into the sheath and crossed the VSD from right to left. The Mullins-type sheath was then advanced over the end-hole balloon catheter (used as a wire) into the left ventricle. The balloon of the Berman catheter was inflated in the left ventricle and achieved complete occlusion of the defect as assessed by TTE, resulting in a stretched diameter of 5 mm (Fig. 1B). After removal of the Berman catheter, an 8–6-mm Amplatzer Duct Occluder device (AGA Medical Corp.) was loaded and introduced into the sheath. Partial withdrawal of the sheath allowed the retention disk (left side) of the device to open in the left ventricle, and the whole system was then pulled back against the left side of the septum. Once TTE had confirmed good apposition of the left side of the device, the sheath was withdrawn further to allow the tubular part of the device to open in the right ventricle (Fig. 1C). The device was then released by counterclockwise rotation of the delivery wire. TTE and right ventriculogram (in fact, the systemic one) showed a trivial residual shunt (Fig. 1D). Two days after VSD closure, a switch intervention was performed with no complications and the patient was discharged 1 week after the operation (Fig. 2). At 4-month follow-up, the patient was doing well and echocardiography showed that the device was correctly positioned with no residual shunt.

Fig. 4.
A: Selected frame from a right ventriculogram in a left anterior oblique view showing the defect (arrow) in the apical muscular septum. B: Selected cineradiographic frame showing the end-hole balloon catheter that occludes the septal defect. The sheath was passed from right to left into the left ventricle. C: Selected cineradiographic frame showing the Amplatzer duct occluder device across the defect, immediately before release. D: Selected frame from a right ventriculogram in a left anterior oblique view showing the device within the defect (arrow) and a trivial residual shunt. LV, left ventricle; RV, right ventricle; VSD, ventricular septal defect.

Fig. 25.
Transthoracic echocardiogram performed before discharge, showing the device (arrow) correctly positioned across the ventricular septal defect. LV, left ventricle; RV, right ventricle.

Discussion

Surgical closure of apical muscular defects has been associated with high morbidity and mortality, especially in the setting of complex congenital heart disease. The risk increases during the neonatal period. These suboptimal results, differing from the other forms of VSDs, have been attributed to residual ventricular shunts and left ventricular dysfunction caused by the incision performed in the left ventricle [3]. In this case, the aortic crossclamping time can be reduced to 90 min.

Percutaneous closure has become an attractive option in this setting, with preliminary results comparing favorably with surgery [6–11]. Furthermore, Bridges et al. [12] reported promising results obtained through a collaborative catheterization laboratory surgical approach for cases of muscular septal defects associated with complex heart lesions, including three patients with TGA. In these cases, the treatment consisted in the placement of a pulmonary artery band followed by transcatheter closure with a double umbrella device at a mean age of 7.3 years (range, 0.8–20.4 years) and an arterial switch procedure [11]. The originality of our strategy lies in the fact that the VSD closure was performed during the neonatal period, avoiding the need for pulmonary-artery band before final arterial switch. Thus, percutaneous closure of VSD was performed at the age of 6 days, allowing a one-stage arterial switch intervention within 48 hr without incision in the systemic ventricle and with no complication.

To date, several devices have been used for percutaneous closure of VSDs: Rashkind PDA Occluder (Bard), Lock’s Clamshell (Bard), Sideris buttoned device, and Amplatzer Muscular VSD Occluder (AGA). The rates of successful closure (complete closure or trivial residual shunt) ranged from 72% to 100% [6–12]. The Amplatzer duct occluder is a self-expandable device made from a Nitinol wire mesh designed to close patent ductus arteriosus. Preliminary results have shown high closure rates [13], and the device has also been used successfully to close a large coronary arteriovenous fistula [14]. To our knowledge, this is the first report of VSD closure with the Amplatzer duct occluder. The major advantages of this device are that it can be delivered with small catheters (5–6 Fr) and easily repositioned or retrieved before release. The possible limitation of this device for VSD closure is the absence of a retention disk on the right side. A new device, the Amplatzer muscular VSD occluder (AGA Medical), is currently being evaluated in humans [9]. This device consists of two flat disks connected by a 7-mm waist, is available in various sizes (6 to 14 mm), and can be introduced via a 6–7 Fr sheath. At the time of this study, this new device was not available in Europe. The Amplatzer duct occluder seems to be an acceptable alternative mainly for newborn infants and small children, and we have used it in several other cases.

From a technical point of view, an arteriovenous guidewire loop is usually recommended for VSD closure. This approach, however, considerably increases procedure duration and requires multiple accesses. We recommend a single antegrade approach as a first step, using the arteriovenous wire looping only when the left ventricle cannot be reached from the right side. Finally, several authors have advocated the use of transesophageal echocardiography to guide transcatheter VSD closure [10,11]. Nevertheless, this approach is cumbersome in small children and good guidance of the device deployment can also be obtained with TTE as evidenced in the present case. Moreover, visualizing apical defects by transesophageal echocardiography can be difficult and conventional echocardiography may provide superior images of this area. In conclusion, newborn infants with transposition of the great arteries associated with ventricular muscular septal defects may be successfully treated with a combined strategy consisting of transcatheter closure of the VSD and switch operation. Increased collaboration between pediatric interventional cardiologists and pediatric cardiac surgeons may improve the suboptimal surgical results associated with this complex congenital heart disease.

References

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Autor: Josep Rode´s, MD, Jean Franc¸ois Pie´chaud, MD, Ruth Ouaknine, MD, Sylvie Hulin, MD, Laurence Cohen, MD, Suzel Magnier, MD, Yves Lecompte, MD, and Thierry Lefe` vre, MD

Fuente: Catheterization and Cardiovascular Interventions 49:173–176

Ultima actualizacion: 14 DE FEBRERO DE 2008

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