Closure of the Fenestration in the Extracardiac FontanWith the Amplatzer Duct Occluder Device

Fernando Rueda,1 MD, Cosimo Squitieri,2 MD, and Luigi Ballerini,1* MD

We report the successful use of the Amplatzer duct occluder for the delayed closure ofthe fenestration in three patients who underwent an extracardiac modified Fontan pro-cedure. At the moment of closure, the patients were 5.5, 2.7, and 3 years old (29 months,3 months, and 14 months after the Fontan procedure, respectively). Immediate fullocclusion was achieved in all cases. In addition, arterial saturation increased significantly(> 5%) with no hemodynamic deterioration. There were no complications during or afterthe procedure, and the patients were discharged in good conditions the day after andwith uneventful follow-up. In conclusion, the Amplatzer duct is safe and effective for theclosure of the fenestration in the extracardiac Fontan. Cathet Cardiovasc Intervent 2001;54:88–92. © 2001 Wiley-Liss, Inc.

Key words: Fontan procedure; interventional cardiology

INTRODUCTION

Total cavopulmonary connection (the surgical connec-tion of the systemic venous return to the pulmonaryvascular bed) is the final stage in a wide number ofcongenital heart conditions. One of the surgical optionsincreasingly accepted is the extracardiac Fontan (EF) [1],a connection between the inferior vena cava to the pul-monary artery with a conduit placed outside the heart. Ithas several advantages such as absence of aortic cross-clamping, low cardiopulmonary bypass time, absence oflong intra-atrial suture lines (which might decrease therisk of subsequent arrhythmias), prevention of baffleleaks, and maintenance of the laminar flow in the sys-temic venous return [2,3].

In the EF, the fenestration consists on a surgical poly-tetrafluoroethilene (PTFE) shunt between the extracar-diac conduit and the systemic atrium (extracardiac fenes-trated Fontan procedure, EFFP). This artificially inducedright-to-left shunt decreases systemic arterial saturation,but has been invaluable in improving the condition of thepatient until adaptation to the new hemodynamic situa-tion is achieved.

Different devices (CardioSEAL, coils, Amplatzer sep-tal occluder) have been used successfully in the intracar-diac Fontan procedures for late closure of the fenestration[4–6]. In these cases, it appears as a hole, but in theEFFP it has a different morphology, more similar to apatent ductus-arteriosus (PDA) or a Blalock-Taussig(BT) shunt. Therefore, none of those devices are suitablefor the closure of the fenestration of the EFFP. We report

the successful use of the Amplatzer duct occluder (ADO)in closing the fenestration of the EFFP in three patients.

Amplatzer Duct Occluder

A description of the device has been made elsewhere[7]. Briefly, it is a Nitinol wire mesh, self-expandable,mushroom-shaped device. The core of the device is acone, the shorter diameter being 2 mm less than thelarger. There is a thin retention disk in the distal part 4mm larger than the maximum diameter of the cone. In theproximal wedge, there is a stainless steel sleeve with athread, which makes detachable possible. Actually, with-drawal is possible at every step until the final release.Three patches of polyester fibers are sewn inside toincrease its thrombogenetic properties. Devices are cur-rently available in sizes ranging from 6/4 to 14/12 mm(the numbers refers to the diameters of the cone).

CASE REPORTS

Case 1 is a 5.5-year-old male who was initially admit-ted at our hospital at the age of 25 days. The diagnosis

1Department of Pediatric Cardiology, Ospedale Bambino Gesu,Rome, Italy2Department of Pediatric Cardiac Surgery, Ospedale BambinoGesu, Rome, Italy

*Correspondence to: Dr. Luigi Ballerini, Department of PediatricCardiology, Ospedale Bambino Gesu´, Piazza S. Onofrio, 4, 00165Roma, Italy.

Received 9 October 2000; Revision accepted 3 May 2001

Catheterization and Cardiovascular Interventions 54:88–92 (2001)

© 2001 Wiley-Liss, Inc.

Fig. 1. A: Normal configuration of the device prior to implantation. B: ADO deployed inside thefenestration. The deformation of the retention disk of the device probably represents a stabi-lization mechanism while the cone (tubular part) occludes the fenestration.

Closure of Fenestrated Fontan With Amplatzer 89

was single left ventricle (SLL), hypoplasia of the left AVvalve, extremely restrictive foramen ovale, and moder-ately restrictive bulboventricular foramen. At that time, apalliative banding of the pulmonary artery and an atrialseptectomy were done. A bidirectional Glenn and a mod-ified Damus-Kaye-Stansel were performed at 5 monthsof age and finally a modified EFFP at 3 years of age:end-to-end unidirectional left superior cavopulmonaryanastomosis with an interposition of a 10 mm Gore-Textube and end-to-end inferior vena cava (IVC) connectionto the right pulmonary artery with 16 mm Gore-Texconduit. The fenestration was made with a surgical 8 mmPTFE shunt placed between the IVC conduit and thesystemic atrium.

Case 2 is a 2.7-year-old female who was transferred toour hospital at 48 hr of life suffering from a variant ofhypoplasic left heart syndrome (HLHS) with mitral, sub-aortic, and aortic stenosis. At 18 days of age, a Damus-Kaye-Stansel, a right BT shunt, and an atrial septectomywere performed. A bidirectional Glenn with enlargementof the pulmonary bifurcation were done at 8 months ofage. EFFP was performed at 2.5 years of age connectingthe IVC and the right pulmonary artery with a conduitand interposing a 6 mmPTFE shunt between the sys-temic atrium and the conduit.

Case 3 is a 3-year-old male admitted at our hospital at24 hr of age with the diagnosis of right isomerism,unbalanced atrioventricular septal defect, pulmonaryatresia, and supradiaphragmatic total anomalous pulmo-nary venous return. A BT shunt was made in the neonatalperiod and a bidirectional Glenn with simultaneous cor-rection of the anomalous venous return were performedat 7 months of age. EFFP was finally done at 20 monthsof age with a PTFE fenestration of 6 mm.

RESULTS

Under general anesthesia, a complete catheterizationwas performed. Prophilaxis antibiotic and heparin (100U/kg; maximum, 5,000 U) were routinely administered.Angiographies were made in both cavae and inside thefenestration. In all cases, temporary occlusion of thefenestration with a balloon catheter was carried out to testthe hemodynamic response, after which a standard

0.0350 exchange guidewire was placed in the atrium bycrossing the fenestration with a multipurpose or rightcoronary 5 Fr catheter. An Amplatz 6 Fr sheath wasadvanced over the wire. Special care was taken to re-move the dilator while the sheath was entering the atriumin order to avoid damaging the atrium. In cases 2 and 3,the ADO device was chosen with the maximum conediameter of equal size as the PTFE conduit. In case 1, asmaller device was chosen as there was a stenosis in thefenestration, being the maximum diameter of the deviceequal to the diameter of the stenosis. The device wasloaded in the delivery system as usual and introducedinto and up to the end of the sheath. Then the device-sheath, as a unit, was pulled back inside the fenestrationuntil the desired location was reached. Further slightretraction of the sheath allowed total deployment of thedevice. Prior to release, an angiography was performed inorder to verify immediate full occlusion, which wasachieved in all cases (Fig. 1).

The mean increase in arterial saturation was 5.7%without hemodynamic deterioration (Table I). Mean flu-oroscopy time for the interventional procedure was 5min. All the patients were discharged the day after ingood conditions. There were no complications during theprocedure or during the follow-up (medium 4.7 months;range 1–7).

DISCUSSION

There is no current exact definition of high-risk patientfor a modified Fontan procedure. Mean pulmonary arterypressure, anatomy of the pulmonary arteries, and thefunction of the systemic ventricle are the main consider-ations. For the last 10 years, use of the fenestrations hasallowed these so-called risk Fontan patients to undergo atotal cavopulmonary venous connection with lower mor-bidity and mortality. Based on its beneficial effects andthe fact that the subsequent closure of the fenestration iseasily performed in the catheterization laboratory, somegroups have extended its application to lower-risk pa-tients. At present, in our hospital, we use the extracardiacconduit modification whenever possible, and we stronglyrecommend a fenestration. The fenestration is made witha PTFE graft, which connects the extracardiac conduit to

TABLE I. Patients Characteristic*

Prior Fontan Fenestration Device Before occlusion After occlusion

Case 1 MPAP 14 mm Hg SaO2 83% PTFE 8 mm ADO 6/4 IVCP 14 mm Hg SaO2 92% IVCP 15 mm Hg SaO2 97%Case 2 MPAP 10 mm Hg SaO2 84% PTFE 6 mm ADO 6/4 IVCP 12 mm Hg SaO2 93% IVCP mm Hg SaO2 99%Case 3 MPAP 10 mm Hg SaO2 89% PTFE 6 mm ADO 6/4 IVCP mm Hg SaO2 94% IVCP mm Hg SaO2 99%

*MPAP: mean pulmonary artery pressure; IVCP: mean inferior vena cava pressure; ADO: Amplatzer duct occluder.

90 Rueda et al.

the atrium, and its precise location may vary dependingon the surgeon’s preferences and the anatomy of thepatient.

Since its appearance in March 1998, the Amplatzerduct occluder has been used successfully not only in theclosure of the ductus arteriosus but also in the emboli-zation of other native or surgical created structures likecoronary fistulas, arteriovenous pulmonary fistulas, and

left ventricle to descending aorta conduit [8,9]. There arevarious reasons for these multiple applications: high ef-ficacy, security (detachable device), low vascular mor-bidity (6 Fr sheath or less), and high adaptability todifferent vascular anatomies (self-expanded device).

In these three patients, following our hospital crite-ria, an EFFP was chosen to complete the cavopulmo-nary connection. The decision to close the fenestration

Fig. 2. Occlusion technique. A: Balloon occlusion test. B: Final result.

Closure of Fenestrated Fontan With Amplatzer 91

was based on clinical outcome and the availability ofthe device. In all patients, transient balloon occlusionwas attempted in order to verify a good response to themaneuver, looking at changes in systemic or meaninferior caval pressure and in the systemic saturation.The position of the device inside the fenestration wasconsidered to be secure enough to avoid embolization.The distal disk tends to stabilize the device while thetubular part occludes the fenestration (Fig. 2). The riskof embolization is low also in contrast to ductal oc-clusion due to its position between low-pressure cham-bers. In two cases, the size of the device was equal tothe diameter of the fenestration and graft, while in theother it was smaller due to a stenosis in the PTFEconduit. In all cases, then, the size of the device wasequal to the smaller PTFE’s diameter. To our knowl-edge, there are only two previous reports of transcath-eter closure of the fenestration in the EFFP. In the first,a successful occlusion of an 8 mm PTFE graft wasachieved with a Gianturco-Grifka vascular device butan 8 Fr sheath was needed [10]. In the second report,two detachable coils were used to closed a 8 mmGore-Tex graft, which has had a previous surgicalmodification done at the time of the Fontan procedure(reduction of the central portion with a surgical clip todecrease the diameter to 4 mm) [11]. In both cases,fluoroscopy time was not reported.

We conclude that closure of the fenestration in theextracardiac Fontan procedure with the Amplatzerduct occluder is a very simple, safe, and effectiveprocedure.

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