Ann Thorac Surg 2009;88:1367-1370. doi:10.1016/j.athoracsur.2009.02.005
© 2009 The Society of Thoracic Surgeons
How To Do It
Novel Modification of Total Cavopulmonary Connection for Isolated Hepatic Vein
Koichi Sughimoto, MD*,
Mitsuru Aoki, MD,
Yuji Naito, MD,
Tadashi Fujiwara, MD
Department of Cardiovascular Surgery, Chiba Children's Hospital, Chiba, Japan
Accepted for publication February 3, 2009.
* Address correspondence to Dr Sughimoto, Chiba Children's Hospital, 579-1 Heta-cho, Midori-Ward, Chiba, 266-0007, Japan (Email: ksughimoto{at}yahoo.co.jp).
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Abstract
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A surgeon needs an innovative technique to establish a Fontan circulation for a patient who has a widely separated hepatic vein from the inferior vena cava. The inferior vena cava was redirected by placing a trimmed GoreTex baffle (W. L. Gore and Associates, Flagstaff, AZ) on the internal side of the atrium connecting the hepatic venous flow, and then directing it to the extra-atrium. Another extracardiac half-circumferential GoreTex baffle was sutured to the epicardial atrial wall and to the pulmonary artery opening. This procedure is efficacious for patients with an isolated hepatic vein and has advantages in terms of using less synthetic material in the conduit.
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Introduction
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Thanks to the use of a Fontan modification, which uses a lateral tunnel or extracardiac total cavopulmonary connection (TCPC) [1, 2], the result of a Fontan operation for patients with a univentricular heart has improved remarkably. However, if a patient has a separated hepatic vein from the inferior vena cava (IVC), a surgeon needs an innovative technique to reroute the venous return to the pulmonary artery.
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Technique
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From August 2005 to July 2007, 3 boys aged 2 to 4 years with asplenia and an isolated hepatic vein underwent an intra-extracardiac TCPC after the staged palliations. During the second-stage palliation, a hemi-Fontan procedure was done as Bove and colleagues [3] described; however, we did not use the pulmonary homograft to augment the pulmonary artery. The right arterial opening was closed with a small GoreTex patch (W. L. Gore and Associates, Flagstaff, AZ) to facilitate the access to the pulmonary route at the third-stage TCPC. Each patient's hepatic vein was on the contrary side of the IVC beyond the vertebra. All of their IVCs were on the left side. The ventricular apex and the IVC were on the same side in patients 1 and 2 (Fig 1). The diagnosis of the previous operation is summarized in Table 1.
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Fig 1. Preoperative morphology of the 3 patients. The inferior vena cava (IVC) and the hepatic vein (HV) were separated by the vertebra in all of the patients. (IAP = intra-atrial GoreTex patch; EAP = external atrial GoreTex patch.)
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At the time of completing the TCPC, the patient was in a supine position, and a repeat sternotomy was made, followed by meticulous dissection. Cardiopulmonary bypass (CPB) was established with ascending aortic, bicaval, and hepatic venous cannulations. Cardioplegic solution was administered to achieve a cardiac arrest. A 5-mm transverse incision was made to the inferior right atrium above the IVC to view the intracardiac anatomy.
In patient 1, the pulmonary veins formed a common chamber and they both returned to the left side of the atrium near the IVC orifice, the isolated hepatic vein returned to the right side of the atrium, and they were separated by the remnant atrial septum; thus, the remnant atrial septum was resected and the pulmonary vein orifice was enlarged.
In patient 2, the remnant atrial septum between the hepatic vein and the common atrioventricular valve was also resected. The IVC flow was redirected with the hepatic vein by placing a trimmed GoreTex baffle on the internal side of the atrium (Fig 2) and then suturing it to the atrial incision. Another trimmed extracardiac half-circumferential GoreTex baffle was sutured to the epicardial atrial wall to the pulmonary artery opening where previously a hemi-TCPC was constructed by meticulous very superficial bites to avoid the sinoatrial nodal artery. The suture lines were remote from the sinoatrial node.
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Fig 2. Operative schema of patient 2. (A) The atrium was incised. (B) Inside view of the atrium. (C) GoreTex baffle (W. L. Gore and Associates, Flagstaff, AZ) was sutured inside of the atrium and the route for the pulmonary artery was created. (D) GoreTex baffle was sutured on the epicardium of the atrium. (Cryo = cryoablation; HV = hepatic vein; IAS = interatrial septum; IVC = inferior vena cava; IVS = interventricular septum.)
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In patient 3, the pulmonary artery trunk was mobilized and sutured to the extracardiac GoreTex baffle. In patient 2, a cryoablation was added at the isthmus between the IVC and the common atrioventricular valve (CAVV), and a CAVV plasty was done with additional sutures in the bridging leaflet.
All patients were weaned from the CPB safely. The postoperative course was uneventful, without death or morbidity, including thromboembolization.
In patients 2 and 3, a postoperative catheterization study showed no pulmonary vein obstruction in the intracardiac GoreTex baffle, which provided smooth pulmonary vein return flow to the CAVV. The combined flow from the IVC and the hepatic vein drained into the pulmonary artery smoothly without stenosis (Fig 3). Postoperative echocardiography revealed no pressure gradient from the IVC to the hepatic vein and to the pulmonary artery.
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Fig 3. Postoperative angiography from the inferior vena cava is shown for (left) patient 2 and (right) patient 3. Smooth routes were created including the hepatic vein.
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Warfarin was discontinued after 3 months, as scheduled, and no thromboembolic events occurred. The patients are in New York Heart Association functional class I, and their oxygen saturation is 90% to 94% in room air. The follow-up period is 1.5 years in patient 1, 3.5 years in patient 2, and 2.5 years in patient 3. All maintain sinus rhythm without arrhythmia or need for pacemaker implantation.
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Comment
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Asplenia occurs in 2.8% of patients with congenital heart disease, according to the Cardiac Registry of the Children's Hospital in Boston [4]. The hepatic vein drained separately from the IVC to the contralateral atrium in 25% of those and to the same atrium in 3%. Various innovative techniques have been reported on how to reroute the isolated hepatic vein to the pulmonary artery so as not to preclude the smooth pulmonary vein return to the atrioventricular valve in patients with asplenia [1, 2]. In most patients, the hepatic vein and the IVC were close enough to be managed. The veins were removed from the atrium and were addressed together and anastomosed en bloc to the TCPC route. However, this simple method would not be applicable if they were far beyond the vertebra, as in the presented patients. Konstantinov and colleagues [5] described a method in which the isolated hepatic vein was connected to the graft between the pulmonary artery and the IVC. Lee and colleagues [6] reported using a short graft from the intra-atrial orifice of the hepatic vein to the TCPC conduit. However, the issue regarding the isolated hepatic vein is still a challenge.
Our method, using the "intra-extracardiac TCPC" for the isolated hepatic vein resolved those issues. The intra-extracardiac TCPC has three major advantages:
- 1 Use of the artificial graft can be minimized. The extracardiac half-circumferential GoreTex baffle contributed to the minimization of the usage of the graft.
- 2 The extracardiac part does not occupy intrapericardial volume without causing pulmonary vein obstruction. Also by placing the intracardiac GoreTex baffle, the IVC flow was redirected to avoid occupying the pericardial space by a TCPC graft.
- 3 The use of less synthetic material in the conduit may be less thrombogenic. Smooth flow from the IVC including the hepatic vein to the pulmonary artery was confirmed. Warfarin was discontinued 3 months after the TCPC operation with careful follow-up in terms of thrombogenesis.
The intracardiac baffle did not obstruct the flow from the pulmonary vein to the CAVV nor the conduction system. A small atrial incision was made to reduce the postoperative arrhythmia. As a result, all the patients maintained a sinus rhythm and did not require a pacemaker implantation.
In conclusion, using this procedure, "intra-extracardiac TCPC" is efficacious for patients with an isolated hepatic vein.
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References
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- Marcelletti CF, Iorio FS, Abella RF. Late results of extracardiac Fontan repair Pediatr Card Surg Annu Semin Thorac Cardiovasc Surg 1999;2:131-141.
- Stamm C, Friehs I, Mayer JE, et al. Long-term results of the lateral tunnel Fontan operation J Thorac Cardiovasc Surg 2001;121:28-41.[Medline]
- Douglas WI, Goldberg CS, Mosca RS, Law IH, Bove EL. Hemi-Fontan procedure for hypoplastic left heart syndrome: outcome and suitability for Fontan Ann Thorac Surg 1999;68:1361-1367.[Abstract/Free Full Text]
- Rubino M, Van Praagh S, Kadoba K, Pessotto R, Van Praagh R. Systemic and pulmonary venous connections in visceral heterotaxy with asplenia: diagnostic and surgical considerations based on seventy-two autopsied cases J Thorac Cardiovasc Surg 1995;110:641-650.[Abstract/Free Full Text]
- Konstantinov IE, Puga FJ, Alexi-Meskishvili VV. Thrombosis of intracardiac or extracardiac conduits after modified Fontan operation in patients with azygous continuation of the inferior vena cava Ann Thorac Surg 2001;72:1641-1644.[Abstract/Free Full Text]
- Lee JR, Lee C, Chang JM, Bae EJ, Noh CI. Modified extracardiac Fontan in a patient with separate hepatic venous drainage Ann Thorac Surg 2002;73:992-993.[Abstract/Free Full Text]
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Abstract
Introduction
