Ann Thorac Surg 2005;79:1047-1049
© 2005 The Society of Thoracic Surgeons
Case report
Alternative Fontan Connection for Apicocaval Juxtaposition
Hong Gook Lim, MDa,
Woong-Han Kim, MD*,a,
Sam-Se Oh, MDb,
Cheong Lim, MDa,
Soo-Jin Kim, MDc,
Young Tak Lee, MDd,
Soo-Cheol Kim, MDb
a Department of Thoracic and Cardiovascular Surgery, Seoul National University Children's Hospital, Seoul National University College of Medicine, Seoul, South Korea
b Department of Cardiovascular Surgery, Sejong General Hospital, Sejong Heart Institute, Buchon, Kyonggi-do, South Korea
c Department of Pediatric Cardiology, Sejong General Hospital, Sejong Heart Institute, Buchon, Kyonggi-do, South Korea
d Department of Thoracic and Cardiovascular Surgery, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, South Korea
Accepted for publication September 10, 2003.
* Address reprint requests to Dr Kim, Department of Thoracic and Cardiovascular Surgery, Seoul National University Children's Hospital, Seoul National University College of Medicine, 28 Yongon-dong, Jongro-gu, Seoul 110-744, South Korea
woonghan{at}korea.com
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Abstract
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We present an alternative technique to the modified Fontan procedure used in a 3-year-old boy who had complex heart defects with bilateral superior vena cava and apicocaval juxtaposition. The left superior vena caval pathway was used behind the ventricle to connect the inferior vena cava to the pulmonary artery. We proved the technical feasibility of this with good midterm results at the 5-year follow-up angiography.
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Introduction
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In a malpositioned heart with apicocaval juxtaposition, the completion of a total cavopulmonary connection is complicated because of the difficulty to construct a lateral tunnel or long curved extracardiac conduit route. In this case report we present an alternative surgical option to the modified Fontan procedure using the left superior vena cava (SVC) pathway in a patient with apicocaval juxtaposition.
A 3-year-old boy (weighing 14 kg) was admitted to the hospital because of moderate cyanosis. A grade 3/6 pansystolic murmur was heard over the entire chest. Mild cardiomegaly with a cardiothoracic ratio of 0.58 and dextrocardia were seen on the chest roentgenogram. Echocardiogram showed {S, L, L} type double-outlet right ventricle with pulmonary stenosis, atrioventricular discordance, mitral regurgitation of grade II, ventricular septal defect of perimembranous inlet extension to outlet extension type, and atrial septal defect of the large secundum type. The cardiac apex pointed to the right, and the inferior vena cava (IVC) drained directly into the right-sided atrium. The left SVC drained into the coronary sinus. Neither heterotaxy nor asplenia/polysplenia were seen. Cardiac catheterization demonstrated a left aortic arch, a right posterior sided left ventricle, a left anterior sided right ventricle, a combined pulmonary stenosis with good pulmonary artery (PA) size, left anterior aorta to the PA, bilateral SVC to the right atrium, mitral valve straddling, and usual coronary arterial pattern. The mitral valve straddling and mitral regurgitation precluded a biventricular repair.
The patient underwent a one-stage modified Fontan procedure as a definitive surgery when he was 3 years old. After sternotomy, standard techniques for cardiopulmonary bypass were used with direct cannulation of the aorta, the right SVC, the left SVC, and the IVC. Under moderate hypothermia, bilateral bi-directional cavopulmonary shunt, main PA double ligation, azygos, and hemi-azygos vein division were performed. The proximal end of the divided left SVC was anastomosed to inferior surface of the left PA. After the aorta was cross clamped and a cold blood cardioplegic solution was administered into the aortic root, the IVC was fully mobilized, transected, and anastomosed to the retrograde coronary sinus and left SVC conduit using glutaraldehyde-fixed autopericardium (Fig 1). A lateral tunnel was formed by the retrograde coronary sinus and the left SVC conduit to the PA without fenestration, and the ostium of the coronary sinus was closed from the right atrium. The aortic clamp was released. The patient was then weaned from cardiopulmonary bypass. Aortic cross-clamp time was 121 minutes. Cardiopulmonary bypass time was 274 minutes. An intraoperative transesophageal echocardiogram showed wide and patent Fontan pathways with mitral regurgitation of grade II due to prolapse of both leaflets. Tricuspid regurgitation was not observed. The postoperative course was uneventful. The patient's vital signs were stable, and his central venous pressure was 20 cm H2O. The electrocardiogram showed normal sinus rhythm. The patient was extubated on postoperative day 1. After an uneventful hospital course, the patient was discharged on postoperative day 10. Follow-up echocardiography showed good ventricular contraction, mitral regurgitation of grade I, no tricuspid regurgitation, no aortic regurgitation, good Fontan pathway, and good pulmonary venous inflow. Five years postoperatively, the patient is in excellent clinical condition. Cardiac catheterization and angiography revealed good Fontan pathway and flow pattern with satisfactory hemodynamic variables (Fig 2).
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Fig 1. Modified Fontan procedure using a contralateral superior vena cava (SVC) tract. (A) The inferior vena cava (IVC) was fully mobilized and transected. (B) The IVC was anastomosed to the left pulmonary artery using the retrograde coronary sinus and left SVC conduit and augment angioplasty with glutaraldehyde-fixed autopericardium. (CS = coronary sinus; GA = glutaraldehyde; LPA = left pulmonary artery; RPA = right pulmonary artery.)
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Fig 2. Angiograms 5 years postoperatively showing (A) good Fontan pathway and (B) good pulmonary flow pattern.
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Comment
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There have been modifications of the Fontan operation for repair in patients with univentricular hearts. Modified Fontan procedures include lateral tunnel and extracardiac conduit. The extracardiac Fontan operation has several advantages compared with the lateral tunnel Fontan operation regarding technical ease, not having to arrest the heart, an ideal laminar flow in the conduit, a reduction of risk of obstruction to the pulmonary venous drainage, and a lower frequency of early and late arrhythmias. The extracardiac conduit Fontan operation has also been known to have potential disadvantages, such as thromboembolism, conduit stenosis, and lack of growth potential [1–3].
Apicocaval juxtaposition makes it difficult to construct a lateral tunnel or a long curved extracardiac conduit route. If the heart is malpositioned with apicocaval juxtaposition, the ideal course of IVC and the PA pathway should be created with careful consideration. When the conduit is placed between the IVC and the opposite side of the PA, the conduit has to cross the spinal column. Crossing the spine may cause a kink and collapse of the conduit at the crossing point and result in Fontan failure. On the other hand, if the conduit is positioned straight between the IVC and the same side of the PA, the conduit has to run behind the ventricle. Potential stenosis of the conduit due to compression caused by the ventricle is of concern [4]. Because the patient in this case had the left pulmonary vein draining into the left atrium, bringing the conduit to the left PA by crossing the vertebrae may have caused left pulmonary venous obstruction, as well as a kink and stenosis of the conduit. Therefore, because the patient had the left SVC draining to coronary sinus, we elected to use the in situ retrograde coronary sinus and the left SVC conduit behind the ventricle as the conduit material.
This modification has all the advantages of using the native tissue, including growth potential at a pediatric age, as well as reduced risk of thrombus formation (which avoids the need for anticoagulation), and the elimination of peel formation, lower infection rate, and reduced cost [5, 6].
Long-term effects of the described technical modification using the left SVC pathway are still unknown. However we experienced the feasibility and good midterm results of the technique we described on a patient with an excellent 5-year follow-up catheterization result. This report presents a useful alternative to the management of patients who have complicated heart defects with apicocaval juxtaposition during the completion of the total cavopulmonary connection. We conclude that a modified lateral-tunnel type Fontan procedure using the left SVC pathway in the apicocaval juxtaposition is a good alternative surgical option with excellent midterm results.
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References
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- Petrossian E, Reddy VM, McElhinney DB, et al. Early results of the extracardiac conduit Fontan operation. J Thorac Cardiovasc Surg. 1999;117:688–696[Abstract/Free Full Text]
- Alexi-Meskishvili V, Ovroutski S, Dähnert I, Lange PE, Hetzer R. Early experience with extracardiac Fontan operation. Ann Thorac Surg. 2001;71:71–77[Abstract/Free Full Text]
- Woods RK, Dyamenahalli U, Duncan BW, Rosenthal GL, Lupinetti FM. Comparison of extracardiac Fontan techniques: pedicled pericardial tunnel versus conduit reconstruction. J Thorac Cardiovasc Surg. 2003;125:465–471[Abstract/Free Full Text]
- Kitayama H, Oku H, Matsumoto T, Onoe M. Total cavopulmonary connection using a pedicled pericardial conduit for a patient with apicocaval juxtaposition. Ann Thorac Surg. 2001;72:1393–1394[Abstract/Free Full Text]
- Hashimoto K, Kurosawa H, Tanaka K, et al. Total cavopulmonary connection without the use of prosthetic material: technical considerations and hemodynamic consequences. J Thorac Cardiovasc Surg. 1995;110:625–632[Abstract/Free Full Text]
- van Son JAM, Reddy VM, Hanley FL. Extracardiac modification of the Fontan operation without use of prosthetic material. J Thorac Cardiovasc Surg. 1995;110:1766–1768[Free Full Text]
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Abstract
Introduction
