Ann Thorac Surg 1998;66:260-262
© 1998 The Society of Thoracic Surgeons
Case Reports
Transcatheter closure of an extracardiac fontan fenestration
Evan M. Zahn, MDa,b,c,
Anthony C. Chang, MDa,b,c,
Redmond P. Burke, MDa,b,c,
Jeffrey P. Jacobs, MDa,b,c
a Division of Cardiology, Miami Children’s Hospital, Miami, Florida, USA
b Division of Cardiothoracic Surgery, Miami Children’s Hospital, Miami, Florida, USA
c Clinica Cardiovascular, Bogota, Colombia
Accepted for publication February 5, 1998.
Address reprint requests to Dr Zahn, Division of Cardiology, Miami Children’s Hospital, 3200 S W 60th Ct, Miami, FL 33155
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Abstract
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As the extracardiac Fontan operation evolves, a reliable method for creating and subsequently closing communications between the systemic and pulmonary venous chambers would be useful. We describe a surgical technique for creating this "fenestration" and a complementary transcatheter technique that allows safe and reliable closure of these communications regardless of size and position.
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Introduction
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Since the original description of the Fontan operation in 1971, numerous modifications have been described [1]. Recently, an extracardiac Fontan operation consisting of a bidirectional cavopulmonary anastomosis and placement of an extracardiac conduit connecting the inferior vena cava to the pulmonary arteries has been reported [2]. This modification preserves the hemodynamic benefits ascribed to the total cavopulmonary connection, yet avoids the disadvantages of aortic cross-clamping and complications related to intraatrial baffling.
As with other modifications of the Fontan operation, the creation of a communication between the systemic venous chamber (extracardiac conduit) and the pulmonary venous chamber (right atrium) may benefit high-risk patients [3]. Ultimate closure of this communication is desirable to maximize systemic oxygen saturation and lower the risk of systemic thromboemboli. We report a case of a child who underwent "fenestrated" extracardiac Fontan operation and subsequent closure of this systemic–pulmonary venous communication with a transcatheter device. This procedure may have broad applicability as use of the extracardiac approach to the Fontan operation increases.
A 34-month-old, 14-kg child had been followed up since infancy with a diagnosis of tricuspid atresia, type IB. At 2 weeks of age, he underwent placement of a left modified Blalock-Taussig shunt. At 21 months of age, he underwent shunt takedown and creation of a right bidirectional cavopulmonary anastomosis. At 34 months of age, he was referred for Fontan procedure because of worsening cyanosis.
Cardiac catheterization before the Fontan procedure demonstrated a patent cavopulmonary anastomosis, no apparent pulmonary arterial stenoses, a mean pulmonary artery pressure of 12 mm Hg, left ventricular end-diastolic pressure of 0 to 7 mm Hg, and a transpulmonary gradient of 4 mm Hg. A 16-mm polytetrafluoroethylene (W.L. Gore and Associates, Inc, Flagstaff, AZ) tube graft was surgically interposed between the inferior vena cava and the anterior wall of the superior vena cava overlying the previously created bidirectional cavopulmonary anastomosis. The graft was sewn to the anterior wall of the superior vena cava to prevent compression of the right superior pulmonary vein and preserve laminar flow. Cardiopulmonary bypass time was 85 minutes. The aorta was not cross-clamped.
Postoperatively the patient had high systemic venous pressure (22 to 25 mm Hg), low cardiac output, ascites, and pleural effusions. Echocardiography revealed severe left ventricular dysfunction. On postoperative day 3, an 8-mm polytetrafluoroethylene tube graft was placed between the extracardiac Fontan tube and the pulmonary venous (anatomic right) atrium. Ventricular function steadily improved, as did the patient’s overall clinical condition; however, supplemental oxygen was required to maintain saturations between 65% and 75%.
Cardiac catheterization was performed 6 weeks after the operation to assess the feasibility of occluding the communication between the systemic and pulmonary venous circulations. Oximetry demonstrated a pulmonary/systemic flow ratio of 0.4 and a cardiac index of 3.8 L · min-1 · min-2 (inferior vena cava = 46%, superior vena cava = 62%, aorta = 74%). Pressures throughout the Fontan circulation were 10 mm Hg, with a 1 mm Hg gradient found across a distal left pulmonary artery stenosis. Angiography revealed a patent extracardiac Fontan conduit and a large right-to-left shunt via the systemic–pulmonary venous communication (Fig 1). The left pulmonary artery stenosis was successfully treated with balloon angioplasty and the systemic–pulmonary venous communication was test occluded with an 8F Berman angiographic balloon catheter (Arrow International, Reading, PA) for 15 minutes. Systemic venous pressure rose to 14 mm Hg, aortic saturation to 92%, and mixed venous saturation to 61%. A 4F Judkins JR 3.5 right coronary artery catheter (Cordis, Miami Lakes, FL) was used to position a 0.035-inch guidewire across the systemic–pulmonary venous communication. An 8F long sheath (Cook Inc, Bloomington, IN) was advanced over the wire into the pulmonary venous end of the communication. After removal of the guidewire, a 9-mm Gianturco-Grifka vascular occlusion device (Cook Inc) was delivered through the sheath as previously described [4]. Angiography confirmed stable device position and complete occlusion of the systemic–pulmonary venous communication before release (Fig 2). Aortic saturation rose to 95% and superior vena caval saturation to 77%. Pressure throughout the Fontan circulation was 12 mm Hg. The patient was discharged 48 hours after the procedure, receiving aspirin. There has been no recurrence of effusions, and the patient is thriving 9 months postoperatively.
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Fig 1. Angiogram of the Fontan circuit before occlusion of the systemic–pulmonary communication. (A) Frontal injection in the superior vena cava reveals a patent caval pulmonary anastomosis, right to left shunting via the surgically created communication (*), and a stenosis of the distal left pulmonary artery (arrow). (B) Lateral injection in the extracardiac Fontan tube profiles the 8-mm polytetrafluoroethylene communication (*) connecting the posteriorly located extracardiac conduit and the anterior pulmonary venous atrium (morphologic right atrium). (ECF = extracardiac Fontan; PVA = pulmonary venous atrium.)
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Fig 2. Frontal (A) and lateral (B) angiography after use of the Gianturco-Grifka vascular occlusion device (arrow) implantation and left pulmonary artery angioplasty. The left pulmonary arterial stenosis has been relieved and there is no longer any shunting via the previously patent communication.
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Comment
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The extracardiac Fontan procedure offers many potential benefits when compared with more traditional atrial–pulmonary or total cavopulmonary connections. A symmetric extracardiac conduit may improve laminar flow, thereby reducing energy loss. The avoidance of atrial suture lines may lessen the incidence of atrial dysrhythmias and interatrial baffle leaks. The avoidance of aortic cross-clamping, and cardiopulmonary bypass in selected patients [5], should minimize immediate postoperative ventricular dysfunction.
Black and associates [6] reported the technique of a tube graft communication to fenestrate an extracardiac Fontan circulation. Although they described use of a 4- to 6-mm prosthesis, we chose an 8-mm graft because this patient had a thick-walled atrium and because we believe that it is easier to narrow than to enlarge this type of communication.
A systemic–pulmonary venous communication reduces systemic venous pressure and maintains cardiac output in the early postoperative period. As with the fenestrated intracardiac lateral tunnel Fontan operation, these benefits are realized with a decrease in systemic oxygen saturation and perhaps an increased risk of systemic thromboemboli. We believe these communications should therefore be closed when possible. Creation of an adjustable polytetrafluoroethylene connection has been described previously [6].
The systemic–pulmonary venous communication created with an extracardiac Fontan procedure is a tubular structure. The Gianturco-Grifka vascular occlusion device has a number of features that are advantageous in occluding this type of structure, including (1) delivery through a relatively small sheath (8F), (2) a variety of sizes to accommodate various graft diameters, (3) easy repositioning, (4) angiographic assessment of the result before device release, and (5) complete retrievability before release. These characteristics allow for superior control in a setting where suboptimal placement could result in residual cyanosis or systemic embolization.
As the extracardiac Fontan operation evolves, a reliable method for creating and subsequently closing communications between the systemic and pulmonary venous chambers would be useful. We describe a transcatheter technique that allows safe and reliable closure of these communications regardless of size and position and affords the operator excellent control for device repositioning and retrieval.
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Acknowledgments
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We thank Luis Horacio Diaz-Medina, MD, and Jorge Sanchez Zapata, MD, for their contribution to patient care.
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References
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- Fontan F., Baudet E. Surgical repair of tricuspid atresia. Thorax 1971;26:240-248.[Abstract/Free Full Text]
- Marcelletti C., Corno A., Giannico S., Marino B. Inferior vena cava–pulmonary artery extracardiac conduit. J Thorac Cardiovasc Surg 1990;100:228-232.[Abstract]
- Bridges N.D., Mayer J.E., Jr, Lock J.E., et al. Effect of baffle fenestration on outcome of the modified Fontan operation. Circulation 1992;86:1762-1769.[Abstract/Free Full Text]
- Grifka R.G., Vincent J.A., Nihill M.R., Ing F.F., Mullins C.E. Transcatheter patent ductus arteriosus closure in an infant using Gianturco-Grifka vascular occlusion device. Am J Cardiol 1996;78:721-723.[Medline]
- Burke R.P., Jacobs J.P., Ashraf M.H., Aldousany A., Chang A.C. Extracardiac Fontan operation without cardiopulmonary bypass. Ann Thorac Surg 1997;63:1175-1177.[Abstract/Free Full Text]
- Black M.D., van Son J.A.M., Haas G.S. Extracardiac Fontan operation with adjustable communication. Ann Thorac Surg 1995;60:716-718.[Abstract/Free Full Text]
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Abstract
Introduction
