Ann Thorac Surg 2002;73:992-993
© 2002 The Society of Thoracic Surgeons
How to do it
Modified extracardiac Fontan in a patient with separate hepatic venous drainage
Jeong Ryul Lee, MD*a,
Cheul Lee, MDa,
Ji Min Chang, MDa,
Eun Jung Bae, MDb,
Chung Il Noh, MDb
a Department of Thoracic and Cardiovascular Surgery, Seoul National University Children’s Hospital, Seoul National University College of Medicine, Seoul National University Clinical Research Institute, Seoul, South Korea
b Department of Pediatrics, Seoul National University Children’s Hospital, Seoul National University College of Medicine, Seoul National University Clinical Research Institute, Seoul, South Korea
Accepted for publication October 16, 2001.
* Address reprint requests to Dr Jeong Ryul Lee, Department of Thoracic and Cardiovascular Surgery, Seoul National University Children’s Hospital, 28 Yongon-dong, Jongro-gu, Seoul 110-744, South Korea
e-mail: jrl{at}plaza.snu.ac.kr
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Abstract
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We describe an alternative technique to the extracardiac Fontan procedure in a patient with a univentricular heart, in which the inferior caval vein and the hepatic vein drained separately into the common atrium and the intraatrial orifice of the hepatic vein was adjacent to the opening of the left lower pulmonary vein.
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Introduction
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In a univentricular heart with complicated systemic or pulmonary venous drainage, intraatrial construction of the Fontan pathway is sometimes challenging because of the complex spatial relationship between their openings into the atrium. In this report, we describe a combined extracardiac and intracardiac conduit inclusion technique of the Fontan pathway in a patient with separate hepatic and inferior caval venous drainage.
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Technique
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A 22-month-old boy who had been diagnosed with {I, L, L} type double-outlet right ventricle (hypoplastic left ventricle), single atrioventricular valve, pulmonary stenosis, and bilateral superior vena cavae was admitted to our unit for surgery. The first operation consisted of a bilateral bidirectional cavopulmonary shunt at the age of 7 months. This procedure was performed under a cardiopulmonary bypass. Hemodynamic data after the first operation showed a mean left superior vena caval pressure of 15 mm Hg, mean pulmonary arterial pressure of 12 mm Hg, mean common atrial pressure of 4 mm Hg, and mean pulmonary vascular resistance index was 1.3 Wood units/m2. At the age of 22 months, the patient underwent a Fontan operation.
The surgical approach was careful redo median sternotomy. Standard techniques for cardiopulmonary bypass were used, with aortic and direct left superior vena cava (SVC) and inferior vena cava (IVC) cannulation under moderate hypothermia. The atrium was vented. After the institution of the bypass, the snares around the SVCs and IVC were tightened. The aorta was cross-clamped and a cold blood cardioplegic solution was administered into the aortic root. The common atrium was opened. The orifices of IVC and hepatic vein were far apart and separately drained into the common atrium. The intraatrial orifice of the hepatic vein was adjacent to the opening of the left lower pulmonary vein. It seemed that a conventional intracardiac lateral tunnel-type diversion might cause distortion of the patch or pulmonary venous obstruction. Thus, we decided to perform an extracardiac conduit Fontan operation with separate diversion of the hepatic vein using an 8-mm intraatrial Gore-Tex tube graft (W.L. Gore & Associates, Flagstaff, AZ). One end of the graft was anastomosed to the margin of the orifice of the hepatic vein, avoiding the obstruction of the left lower pulmonary vein. A 4-mm fenestration was then made on the graft. After a DeVega-type annuloplasty on the single atrioventricular valve, the atriotomy wound was closed, leaving an outlet for the opposite end of the graft. Anastomosis between the graft and the opened atrial flap was done. The aortic clamp was released. A clamp was placed across the IVC proximal to the IVC cannula. The IVC was transected and an 18-mm Gore-Tex tube graft was anastomosed to the transected distal end of IVC. The atrial stump was closed. A longitudinal incision was made at the underside of the pulmonary artery. Anastomosis was done between the pulmonary arteriotomy and the beveled upper end of the graft. End-to-side anastomosis was done between the opening of the intraatrial graft and the side hole made on the extracardiac conduit (Fig 1).
The patient was then weaned from cardiopulmonary bypass.
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Fig 1. Scheme of modified extracardiac conduit Fontan procedure. A = atrium; Ao = aorta; C1 = extracardiac conduit; C2 = intraatrial conduit; CS = coronary sinus; F = fenestration; HV = hepatic vein; LLPV = left lower pulmonary vein; LSVC = left superior vena cava; PA = pulmonary artery; RSVC = right superior vena cava; V = ventricle.)
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An immediate postoperative echocardiogram showed wide and patent intracardiac and extracardiac Fontan pathways with trivial atrioventricular valvular regurgitation. The patient has been followed-up for 14 months with no evidence of thromboembolism, and has remained in sinus rhythm. An echocardiogram performed 12 months after the operation revealed widely patent Fontan pathway with good ventricular function. A small right-to-left shunt remained through the fenestration.
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Comment
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With a variety of modifications of the original Fontan operation, there has been an improvement in the early and late outcomes for repair in patients with univentricular hearts. However, in certain forms of hearts with univentricular morphology, performing the Fontan procedure is technically challenging because of the complicated patterns of systemic and pulmonary venous drainage. For this reason, although it also has been known to have potential disadvantages such as thrombotic complications, conduit stenosis, and lack of growth, the extracardiac conduit Fontan operation can be a better option. Furthermore, the extracardiac conduit approach has advantages such as technical ease, no need to arrest the heart, optimal laminar flow in the conduit, reduction of the risk of obstruction to the pulmonary venous drainage, and a lower frequency of early and late arrhythmias [1–5]. However, in the case of the univentricular hearts with separate hepatic venous drainage, it is not always technically feasible to include both the inferior vena cava and the hepatic vein into a single extracardiac conduit.
In this particular case, neither an extracardiac conduit-type Fontan nor an intracardiac lateral tunnel-type Fontan was indicated, because the orifices of the IVC and hepatic vein were far apart and were adjacent to the orifice of the left lower pulmonary vein. Inclusion of both the inferior vena cava and the hepatic vein into a single extracardiac conduit may cause a kinking of the hepatic vein or pulmonary venous obstruction, whereas construction of a lateral tunnel may cause a distortion of the intraatrial patch or a pulmonary venous obstruction. This reasoning led us to use two separate conduits. The connection between the IVC and the pulmonary artery was constructed with an extracardiac conduit. The connection between the hepatic vein and the extracardiac Fontan pathway was constructed using a smaller intraatrial conduit. A fenestration was made on the superior aspect of the intracardiac conduit. We thought that this would provide a better direction for the follow-up catheterization. We oriented two conduits in an inverted Y shape to minimize turbulence at the confluence. Although an artiotomy incision and cardiac arrest were required to construct the intraatrial pathway, the extensive intraatrial suture lines could be avoided by using this approach. At the same time, possible hemodynamic superiority of the extracardiac conduit procedure over the intraatrial or extracardiac lateral tunnel techniques [6] could be demonstrated. The risk of thromboembolic complication and the potential limitation of the atrial movement were of concern. The patient had been on anticoagulation therapy using a low dose of Coumadin (Du Pont Pharmaeuticals, Wilmington, DE) for the first 6 months and then with antiplatelet agent. A follow-up echocardiogram revealed neither limitation of atrial movement nor intracardiac thrombus formation 18 months after the operation.
This modification of extracardiac conduit Fontan procedure may be useful in selected patients who have either univentricular hearts with separate hepatic venous drainage or complicated patterns of the systemic and pulmonary venous drainage systems.
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References
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Petrossian E., Reddy V.M., McElhinney D.B., et al. Early results of the extracardiac conduit Fontan operation. J Thorac Cardiovasc Surg 1999;117:688-696.[Abstract/Free Full Text]
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Shirai L.K., Rosenthal D.N., Reitz B.A., Robbins R.C. Arrhythmias and thromboembolic complications after the extracardiac Fontan operation. J Thorac Cardiovasc Surg 1998;115:499-505.[Abstract/Free Full Text]
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Alexi-Meskishvili V., Ovroutski S., Dähnert I., Lange P.E., Hetzer R. Early experience with extracardiac Fontan operation. Ann Thorac Surg 2001;71:71-77.[Abstract/Free Full Text]
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Haas G.S., Hess H., Black M., Onnasch J., Mohr F.W., Van Son J.A.M. Extracardiac conduit Fontan procedure: early and intermediate results. Eur J Cardiothorac Surg 2000;17:648-654.[Abstract/Free Full Text]
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Marcelletti C., Corno A., Giannico S., Marino B. Inferior vena cava-pulmonary artery extracardiac conduit. J Thorac Cardiovasc Surg 1990;100:228-232.[Abstract]
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Lardo A.C., Webber S.A., Friehs I., del Nido P.J., Cape E.G. Fluid dynamic comparison of intra-atrial and extracardiac total cavopulmonary connections. J Thorac Cardiovasc Surg 1999;117:697-704.[Abstract/Free Full Text]
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Abstract
Introduction
