Ann Thorac Surg 2003;75:587-590
© 2003 The Society of Thoracic Surgeons
Case report
Catheter closure of autologous pericardial extracardiac Fontan fenestration
Michael R. Recto, MD*a,
Walter Sobczyk, MDa,
Thomas Yeh, Jr, MD, PhDb,
Erle H. Austin, III, MDa,b
a Division of Pediatric Cardiology, Louisville, Kentucky, USA
b Department of Cardiothoracic Surgery, University of Louisville, Louisville, Kentucky, USA
Accepted for publication August 22, 2002.
* Address reprint requests to Dr Recto, Division of Pediatric Cardiology, University of Louisville, 571 South Floyd, Suite 334, Louisville, KY 40202-3830, USA.
e-mail: mitch.recto{at}louisville.edu
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Abstract
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We describe two patients who required fenestration of an autologous pericardial extracardiac Fontan (APEF), short-medium term angiographic follow-up, and subsequent successful transcatheter closure of the fenestration.
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Introduction
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Since the Fontan operation was first described in 1971 [1], numerous modifications have been made. In 1988 total cavopulmonary anastomosis was proposed as an alternative to atriopulmonary connection [2]. An intraatrial lateral tunnel was used to connect the inferior vena cava (IVC) to the pulmonary artery, together with a bidirectional cavopulmonary shunt (BCPS). In the 1990s the extracardiac approach gained popularity because it avoided myocardial ischemia, along with atrial incision and suture lines [3]. Unfortunately, these extracardiac conduits lacked the potential for growth. To counter this, Gundry in 1997 [4] described the use of autologous living pericardium to construct the extracardiac Fontan. The IVC was routed to the right pulmonary artery using the right atrium as the medial wall, and autologous pericardium with intact blood supply as the lateral wall. In Gundry’s series, none of the patients required fenestration [4, 5]. In our series, 2 out of 23 patients have required fenestration and have now undergone successful transcatheter closure.
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Case reports
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Patient 1
A 6-month-old female with tricuspid atresia type I-B, underwent a BCPS. Pre-Fontan catheterization revealed a patent BCPS. The mean pulmonary artery pressure was 13 mm Hg, left ventricular end diastolic pressure (LVEDP) was 0 to 7 mm Hg, and the transpulmonary gradient was 6 mm Hg. The patient underwent an autologous pericardial extracardiac Fontan (APEF) operation at 2.5 years old. Though initially nonfenestrated, she required fenestration for an elevated central venous pressure (CVP) and hypotension. Under fibrillatory arrest, the pericardial tunnel was reopened anteriorly and a fenestration was created by implanting a 1.5-cm Gore-Tex patch (W.L. Gore & Associates, Flagstaff, AZ) in the right atrial free wall. The Gore-Tex patch had been prepared with a 4-mm calibrated punch. Following cardiopulmonary bypass, the patient’s blood pressure and saturations immediately improved. The patient was discharged on Coumadin (Du Pont Pharmaceuticals, Wilmington, DE) and switched to aspirin in 6 months.
By 7 years of age, her saturation decreased to the low 80’s and she developed clubbing of the fingers. Chest roentgenogram demonstrated mild cardiomegaly with normal pulmonary vascularity. Echocardiography demonstrated a patent fenestration with right to left flow.
At catheterization, her pulmonary to systemic flow ratio (Qp:Qs) was 0.7 (aorta = 86%, superior vena cava [SVC] = 63%, pulmonary artery = 63%, pulmonary venous saturation = 96%). CVP was 10 mm Hg, mean left atrial pressure was 7 mm Hg and LVEDP was 0 to 7 mm Hg (transpulmonary gradient was 3 mm Hg). Angiography demonstrated a widely patent Fontan circuit with right-to-left shunt through the fenestration (Fig 1A).
Twenty-minute occlusion of the fenestration with a 7 Fr Berman catheter (Arrow International, Reading, PA) revealed slight increase in CVP to 12 mm Hg, LVEDP and mixed venous saturation remained unchanged and aortic saturation improved to 94%. After proving that the patient could tolerate trial balloon occlusion of the fenestration, the fenestration was closed with a 17-mm CardioSeal Septal Occlusion Device (NMT Medical, Boston, MA; Fig 1B). The following morning echocardiography demonstrated complete closure of the fenestration. The patient was discharged on aspirin. At 3 months, room air saturation remains 93%.
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Fig 1. (A) Angiogram of autologous extracardiac lateral tunnel Fontan (AP projection) in a patient with tricuspid atresia type I-B before closure of the fenestration (white arrow demonstrates right-to-left flow through fenestration). (B) Angiogram of the autologous extracardiac lateral tunnel Fontal (AP projection) in a patient with tricuspid atresia type I-B after fenestration closure with 17-mm CardioSeal Septal Occlusion Device (white arrow).
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Patient 2
At 3 months of age, a boy with double outlet left ventricle and left atrioventricular valve atresia, underwent a modified left Blalock-Taussig (BT) shunt. At 7 months, the BT shunt was taken down and a BCPS was created. Pre-Fontan catheterization demonstrated mild left pulmonary artery hypoplasia. At 3.5 years old, he underwent a fenestrated APEF as previously described. He was discharged on Coumadin (Du Pont Pharmaceuticals) for 6 months, and converted to aspirin.
By 7.5 years old, his saturation had fallen to 80% at rest, 60% with exercise, and he reported dizziness without loss of consciousness or neurologic sequelae. At catheterization his Qp:Qs was 0.54 (aorta = 84%, SVC = 70%, pulmonary artery = 70%, pulmonary venous saturation = 96%). His CVP was 13 mm Hg. Mean left atrial pressure was 7 mm Hg. LVEDP was 0 to 7 mm Hg. Transpulmonary gradient was 6 mm Hg. Angiography demonstrated a patent Fontan circuit with right to left shunting across the fenestration. In addition, the patient had a large left superior vena cava (LSVC) draining to the coronary sinus (additional source of right to left shunting; Fig 2A).
Simultaneous trial occlusion of the fenestration and LSVC revealed a slight increase in mean CVP to 14 mm Hg, no change in LVEDP, an increase in mixed venous saturation to 77%, and increase in aortic saturation to 96%. The LSVC was permanently occluded with a 7 mm Gianturco Grifka Vascular Occlusion Device (Cook, Bloomington, IN), and the fenestration closed with a 17 mm CardioSeal Septal Occlusion (CSO) device. Angiography demonstrated complete occlusion of the LSVC and Fontan fenestration (Fig 2B). The following morning echocardiography demonstrated complete closure of the fenestration and LSVC. The patient was discharged home on aspirin and at 1 month is well with a saturation of 94%.
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Fig 2. (A) Angiogram demonstrating the presence of a patent Fontan fenestration (white arrow) and large left superior vena cava to coronary sinus (black arrow) in a patient with double outlet left ventricle with left atrioventricular valve atresia (AP projection). (B) Angiogram demonstrating closure of Fontan fenestration with 17-mm CardioSeal Septal Occlusion Device (white arrow) and occlusion of left superior vena cava with a 7-mm Gianturco Grifka Vascular Occlusion Device (black arrow; AP projection).
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Comment
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The theoretical advantage of the autologous extracardiac pericardial Fontan [4] is that it is made of living tissue with the potential for growth. As such, the need to attain a certain weight or size before Fontan is less of a consideration than when nonliving, prosthetic or homograft conduits are used. As demonstrated by hemodynamic and angiographic data (Figures 1 and 2), these conduits are widely patent 4- to 4.5-years post-APEF.
To decrease the risk of thromboemboli and cyanosis, our institution has chosen not to employ routine fenestration. However, 2 of 23 patients [6] have required fenestration, and have now undergone successful transcatheter closure. Because the size of the fenestration was calibrated, it was not necessary to balloon size the fenestration before closure. The CSO contains less metal (nitinol) and has a lower profile (Figs 1B and 2B), than the Amplatzer Septal Occluder (ASO), posing less risk of Fontan obstruction; however, the CSO requires a larger sheath (10F) than the ASO (6F).
In summary, 2 patients have undergone successful transcatheter closure of Fontan fenestration, following their autologous pericardial extracardiac Fontan operation. In early angiographic follow-up, these circuits appear to have grown in the first 4 to 4.5 years, but longer follow-up is required. Because these conduits are difficult to visualize echocardiographically, serial angiography or magnetic resonance imaging will be performed to assess the growth potential of these conduits.
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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]
- de Leval M.R., Kilner P., Gewillig M., Bull C. Total cavopulmonary connection: a logical alternative to atriopulmonary connection for complex Fontan operations-experimental studies and early clinical experience. J Thorac Cardiovasc Surg 1988;96:682-695.[Abstract]
- Marcelletti C., Como A., Giannico S., et al. Inferior vena cava-pulmonary artery extracardiac conduit: a new form of right heart bypass. J Thorac Cardiovasc Surg 1990;100:228-232.[Abstract]
- Gundry S.R., Razzouk A.J., del Rio M.J., Shirali G., Bailey L. The optimal Fontan connection: a growing extracardiac lateral tunnel with pedicled pericardium. J Thorac Cardiovasc Surg 1997;114:552-559.[Free Full Text]
- Gundry SR, Razzouk AJ, del Rio M, Feikes R, Bailey L. The autologous pericardial lateral tunnel eliminates the need for fenestrations in the Fontan procedure. In the Proceedings of the 34th Annual Society of Thoracic Surgeons Annual Meeting January 26–28, 1998 (abstract)
- Pagni S, Sobczyk W, Yeh T, Boone W, Austin EH. Clinical and echocardiographic result with the extracardiac pericardial Fontan operation. In: 3rd World Congress of Pediatric Cardiology and Cardiac Surgery. Cardiology in the Young 2001;11:301
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Abstract
Introduction
