Asian Cardiovasc Thorac Ann 1999;7:121-123
© 1999 Asia Publishing EXchange Pte Ltd
Closure of Residual Shunts After Fontan Operation With Amplatzer Occluder
Zahid Amin, MD,
Albert P Rocchini, MD,
David F Wax, MD
Northwestern University Children's Memorial Medical Center Chicago, Illinois, USA
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For reprint information contact: Zahid Amin, MD Tel: 1 706 721 2336 Fax: 1 706 721 3838 email: zamin{at}mail.mcg.edu Medical College of Georgia, Division of Pediatric Cardiology, 1120 15th Street, BAA 800W, Augusta, GA 30912, USA.
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Abstract
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The fenestrated Fontan operation is commonly performed at several centers to decrease postoperative morbidity. Ultimately, the fenestrations are closed to avoid desaturation and embolism. Closure is safely accomplished in the operating room or in the catheterization laboratory with a device. We report our experience with the Amplatzer septal occluder that was successfully used to close residual shunts and fenestrations in two patients.
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Introduction
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Since the first reports of atriopulmonary connection by Fontan and colleagues1 and Kreutzer and colleagues2 for patients with complex congenital heart disease, numerous modifications have been successfully applied. These modifications led to revision of the selection criteria for patients, with substantial decreases in mortality and morbidity.37 The concept of leaving a residual communication between the systemic and pulmonary venous circulation was introduced by Bridges and colleagues8 as a fenestration and by Laks and colleagues9 as an adjustable atrial septal defect. The communication helps maintain cardiac output at the expense of lower arterial oxygen saturation. Once hemodynamic stability has been achieved, the fenestrations are closed to improve arterial oxygen saturation and prevent systemic emboli. Fenestration closure is performed in the operating room or in the cardiac catheterization laboratory.5,7,8,10 We describe closure of such a fenestration and a residual atrial septal defect using the Amplatzer device (AGA Medical Corp., Golden Valley, MN, USA).
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Patients and Methods
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The Amplatzer is a new occlusion device that is undergoing clinical trials in the United States at centers designated by the Food and Drug Administration. A description of its design and construction material has been published elsewhere.11 Briefly, it is constructed from 0.004 inch Nitinol wires woven into two flat discs with a 4-mm waist. Nitinol is a shape-memory alloy with superelastic properties. The device is self-centering and retrievable. It has a microscrew on one side (right atrial disc) to attach it to the delivery cable. It can be withdrawn into a loader and then introduced into a 7F delivery sheath for deployment.
Patient no. 1 was a 14-year-old Caucasian female who was born with tricuspid atresia, restrictive ventricular septal defect, and normally related great arteries. She initially underwent balloon atrial septostomy and later, a modified Blalock-Taussig shunt. At the age of 2 years she had a Fontan operation during which the ventricular septal defect was closed and the right atrial appendage was connected to the right ventricle and main pulmonary artery with the aid of a composite Gore-Tex graft (WL Gore, Flagstaff, AZ, USA). Subsequent echocardiography and cardiac catheterization revealed excellent repair with residual atrial communication. The patient had a good activity level but complained of intermittent cyanosis. Her room-air arterial blood oxygen saturation was 94%. A Holter monitor study was normal and an exercise test revealed mild desaturation (90%) with fair exercise tolerance. A decision was made to close the atrial communication to improve arterial oxygen saturation.
Patient no. 2 was a 3-year-old Caucasian male who was born with double-inlet left ventricle and left transposition of the great arteries. The diagnosis was made at 7 months of age and he underwent pulmonary artery banding as initial palliation. At the age of 2 years, he underwent total cavopulmonary connection with enlargement of the bulboventricular foremen and total cavopulmonary artery connection using a lateral tunnel. A patch of expanded polytetrafluoroethylene (WL Gore, Flagstaff, AZ, USA) was used to create the medial wall of the lateral tunnel. Two fenestrations approximately 2 mm apart were made with a punch measuring 2.7 mm in the medial wall of the lateral tunnel (polytetrafluoroethylene patch). He made good progress after this operation and his room air arterial oxygen saturation was 90%. An echocardiogram revealed a right-to-left shunt through the fenestration. It was decided to close the fenestration in the catheterization laboratory to prevent paradoxical embolism and improve arterial oxygen saturation.
The protocol for placement of the device was approved by the Food and Drug Administration and by the Committee on Clinical Investigations at Children's Memorial Hospital. Informed consent was obtained prior to placement of the device. A transesophageal echocardiography (TEE) probe was placed in both patients under general endotracheal anesthesia. The size and location of the defects were measured. A 7F sheath was placed percutaneously in the right femoral vein and a 5F sheath was placed in the femoral artery. The patients were anticoagulated with 100 unitskg1 heparin. The oxygen saturations and hemodynamic data at rest and during a test occlusion were obtained to confirm that the patients were suitable candidates for closure of the atrial communication. A 7F Berman angiocatheter (Arrow International, Inc., Reading, PA, USA) was advanced into the left atrium through the defect and a cineangiogram was obtained with the catheter astride the defect to delineate the atrial septum (Figure 1
). The Berman angiocatheter was replaced with a balloon wedge (Arrow International, Inc., Reading, PA, USA) and a 0.038 inch stiff wire (Cook, Inc., Bloomingdale, IN, USA) was passed through the catheter into the left atrium followed by removal of the catheter. A 7F delivery sheath with a dilator was passed over the wire into the left atrium. The dilator and the wire were removed. In both cases, a 4-mm Amplatzer septal occluder was attached to the delivery cable and loaded into the 7F sheath. The sheath was carefully aspirated prior to loading. The device was advanced into the left atrium and the left atrial disc was deployed. The sheath and the delivery cable were withdrawn under fluoroscopic and TEE guidance until a mild resistance was felt. Then the sheath was gently withdrawn while keeping the delivery cable stable, to deploy the right atrial disc. The device was disconnected by counterclockwise rotation of the delivery cable. An angiogram was performed and the hemodynamic measurements were repeated. TEE was used throughout the deployment and to assess any residual shunting. Cefazolin (15 mgkg1) was given intravenously in 3 doses while the patients were observed in the hospital overnight. At discharge, baby aspirin (82 mg) was started for 6 months.

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Figure 1. Angiogram in the lateral tunnel with the catheter astride the fenestration (black arrow) to delineate the atrial septum.
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Results
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There was no right-to-left or left-to-right shunt through or around the device determined by angiography and TEE (Figure 2
). The discs approximated the septum on both sides (Figure 3
). In patient no. 1, the right atrial oxygen saturation increased to 97% from 95%. Her arterial oxygen tension (PaO2) increased to 523 from 400 mm Hg on FiO2 (fraction of inspired oxygen 100%) of 1. In patient no. 2, room air saturations increased to 96% from 90% and the PaO2 increased to 485 from 185 mm Hg on FiO2 of 1. There was no change in the pulmonary arterial pressures after closure of the shunt. One device was sufficient to close both fenestrations in patient no. 2 because of the close proximity of the two holes.

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Figure 2. Lateral tunnel angiogram in the same patient after placement of the device (white arrow). No shunt is seen.
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At the 6-month follow-up, both patients had a room air oxygen saturation of 96%. Echocardiography revealed no evidence of a shunt through or around the devices. Both patients reported excellent exercise tolerance.
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Discussion
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Closure of fenestrations in an asymptomatic patient who is minimally desaturated remains controversial.12 However, we believe that if the fenestration does not close spontaneously after hemodynamic stability has been achieved, it should be closed. The Bard clam shell occluder device and recently, Gianturco coils, have been used for closure of Fontan fenestrations.5,7,8,10,12 The Amplatzer septal occluder is under clinical trial in the United States and has been used in several European countries for the last three years. Unlike other devices, the Amplatzer is circular in shape (no arms) and stents the hole, which may lead to a high closure rate. The advantages of this device include a small (7F) delivery system, complete retrievability after deployment (to the point of release), self-centering, and simple mechanics. We believe that this device will be a valuable tool for closing such defects in the future.
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References
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