Ann Thorac Surg 2006;81:746-748
© 2006 The Society of Thoracic Surgeons
Case report
An Alternative Method to Fenestrate the Extracardiac Fontan Conduit in a Patient with Atrial Situs Inversus and Levocardia
Vinay M. Tak, MD, FRCS
a
,
b
,
c
,
Saadeh Al-Jureidini, MD
a
,
b
,
c
,
Ian Balfour, MD
a
,
b
,
c
,
Mary Hohenberg, PA
a
,
b
,
c
,
Barbara Kountzman, RN
a
,
b
,
c
,
Andrew C. Fiore, MD
a
,
b
,
c
,
*
a Division of Pediatric Cardiology, St. Louis University School of Medicine, St. Louis, Missouri
b Department of Cardiothoracic Surgery, St. Louis University School of Medicine, St. Louis, Missouri
c Cardinal Glennon Children's Hospital, St. Louis, Missouri
Accepted for publication December 17, 2004.
* Address correspondence to Dr Fiore, St. Louis University, Pediatric Cardiac Surgery, Cardinal Glennon Children's Hospital, 1465 S Grand Blvd, St. Louis, MO 63104 (Email: fiorem2{at}slu.edu).
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Abstract
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This report describes the technique to fenestrate the extracardiac Fontan conduit without cardiopulmonary bypass in a patient with levocardia and atrial situs inversus.
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Introduction
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The extracardiac Fontan has been increasing in popularity as a means of achieving total caval pulmonary connection especially in the setting of heterotaxy syndrome. In cases of atrial situs solitus and levocardia, fenestration of the extracardiac Fontan can be readily accomplished by a direct atrial-conduit anastomosis or by inserting a short interposition graft between the conduit and the pulmonary venous atrium [1]. However, there is little information on the technique of fenestration to be employed in the situation where the extracardiac conduit lies behind the heart (atrial situs solitus with dextrocardia or atrial situs inversus with levocardia). This report describes an alternative method to fenestrate the extracardiac Fontan in this situation without cardiopulmonary bypass.
The patient is a 5-year-old male child with visceral, atrial, and bronchial situs inversus, and levocardia. The great vessels are transposed with a systemic ventricle to the right and anterior giving rise to the aorta, and a hypoplastic pulmonary ventricle, posterior and to the left, giving rise to a small pulmonary artery, with severe valvar and subvalvar pulmonary stenosis. The aortic arch is right sided with the superior and inferior vena cavae on the left side. The pulmonary venous drainage is normal and the atrial septal defect is nonrestrictive. The patient underwent a 4-mm modified left Blalock-Taussig shunt at 1 month and a left-sided bi-directional superior caval pulmonary anastomosis at 2 years of age.
At age 5, cardiac catheterization demonstrated a mean pulmonary artery pressure of 10 mm Hg and a right ventricular end-diastolic pressure of 8 to11 mm Hg. The branch pulmonary arteries and the bi-directional superior caval pulmonary connection were normal. He underwent a non-fenestrated extracardiac Fontan by interposing a 20-mm ringed Gore-Tex tube (W.L. Gore, Flagstaff, AZ) between the left-sided inferior vena cava and the undersurface of the left pulmonary artery with the heart beating on cardiopulmonary bypass.
His initial postoperative course was complicated by right diaphragm paralysis and persistent bilateral chylous pleural drainage, hepatomegaly, and ascites. The transpulmonary gradient was 8 to 10 mm Hg with venous pressures within the conduit and pulmonary arteries of 15 mm Hg, and an oxygen saturation of 95% on room air. The single ventricle function was normal with no atrioventricular valve insufficiency, yet the pleural drainage increased to 1,000 cc per day despite the use of oxygen, diuretics, nitric oxide, milrinone, total parental nutrition, and octreotide. Surface echocardiography with contrast did not demonstrate any obstruction within the total caval pulmonary connection.
A decision was made to fenestrate the extracardiac Fontan tube without cardiopulmonary bypass. Initially, a left fourth innerspace thoracotomy was performed, but this approach was abandoned because there was no left-sided atrial appendage and access to the pulmonary venous atrium was difficult because it was overlapped by the conduit and the ventricle. Two days later, a median sternotomy was employed. An 8-mm ringed Gore-Tex tube (W.L. Gore) was interposed between the extracardiac conduit and the right-sided atrial appendage coursing anteriorly over the ascending aorta using side-biting vascular clamps (Fig 1). On completion of the anastomosis, the oxygen saturations fell to 75% to 80% on 50% oxygen, and the pressures within the extracardiac conduit decreased to 8 to 10 mm Hg. Intraoperative transesophageal echocardiography confirmed a widely patent fenestration.
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Fig 1. Illustration demonstrating the position of the 8-mm Gore-Tex (W. L. Gore, Flagstaff, AZ) fenestration from its origin on the caval pulmonary connection coursing anterior to the ascending aorta and inserting into the right-sided atrium. (IVC = inferior vena cavae; LPA = left pulmonary artery; RPA = right pulmonary artery; SVC = superior vena cavae.)
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The patient's ascites and chylothoraces resolved within 10 days, and a regular diet was well tolerated. He required right diaphragm plication prior to extubation. He was discharged without further complications on Coumadin (sodium warfarin, Bristol Myers-Squibb, Princeton, NJ) and Captopril (Bristol Myers-Squibb) 10 weeks postoperatively.
Computerized tomographic angiography performed at 1 month postoperatively demonstrated a widely patent fenestration and extracardiac Fontan conduit (Figs 2, 3).
Surface echocardiography 6 months post discharge documented a persistent widely patent fenestration (Figs 4, 5).
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Fig 2. A sagittal section of a computerized tomographic angiogram demonstrating the origin of the fenestration from the caval pulmonary connection to its insertion into the right-sided atrium.
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Fig 4. A long axis echocardiogram demonstrating the caval pulmonary connection with laminar flow through the fenestration origin (arrow).
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Fig 5. Cross sectional echocardiogram demonstrating laminar flow through the origin of the fenestration.
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Comment
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The role of the fenestration in the Fontan operation remains controversial. Those surgeons who routinely fenestrate all total caval pulmonary connections believe that fenestration will decompress the systemic venous system, improve ventricular filling, and increase cardiac output during the early postoperative period at the expense of mild cyanosis [2]. Perhaps more importantly, the "fenestrated" Fontan procedure has been associated with decreased mortality in high-risk patients and a decrease in morbidity from pleural effusions and ascites [3].
Patients receiving a lateral tunnel Fontan can be easily fenestrated by creating a hole of appropriate size in the Gore-Tex (W.L. Gore) partition using an aortic punch. In this case, fenestration of the extracardiac Fontan tube could not be performed by the usual methods because of the anatomy associated with heterotaxy syndrome. This report describes a technique to fenestrate the extracardiac Fontan conduit without cardiopulmonary bypass in this case of levocardia and atrial situs inversus. Although the extracardiac conduit can be fenestrated directly to the pulmonary venous atrium at the time of insertion, our technique is a safe and straightforward method to fenestrate the conduit late postoperatively without the use of cardiopulmonary bypass. To our knowledge, this technique has not been reported in the literature previously and provides an alternative reference point for others in a similar situation.
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References
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- Kreutzer C, Schlichter AJ, Simon JL, Parodi WMC, Blunda C, Kreutzer GO. A new method for reliable fenestration in extracardiac conduit Fontan operations Ann Thorac Surg 2003;75(5):1657-1659.[Abstract/Free Full Text]
- Goff DA, Blume ED, Gauvreau K, Mayer JE, Lock JE, Jenkins KJ. Clinical outcome of fenestrated Fontan patients after closure, the first 10 years Circulation 2000;102:2094-2099.[Abstract/Free Full Text]
- Rychik J, Rome JJ, Jacobs ML. Late surgical fenestration for complications after the Fontan operation Circulation 1997;96:33-36.[Abstract/Free Full Text]
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Abstract
Introduction
