Ann Thorac Surg 2004;78:697-699
© 2004 The Society of Thoracic Surgeons
Case report
Regression of severe pulmonary arteriovenous malformations after Fontan revision and "hepatic factor" rerouting
Nancy A. Pike, RN, FNPa*,
Luca A. Vricella, MDa,
Jeffrey A. Feinstein, MDb,
Michael D. Black, MDb,
Bruce A. Reitz, MDb
a Division of Cardiothoracic Surgery, David Geffen School of Medicine at University of California Los Angeles, Los Angeles, California, USA
b Division of Cardiothoracic Surgery, Pediatric Cardiology, Lucile Packard Children's Hospital at Stanford, Stanford University School of Medicine, Stanford, California, USA
Accepted for publication October 2, 2003.
* Address reprint requests to Ms Pike, Cardiothoracic Surgery, David Geffen School of Medicine, UCLA, Box 951741, Los Angeles, CA 90095-1741, USA
e-mail: npike{at}mednet.ucla.edu
 |
Abstract
|
|---|
Although previously described in patients undergoing staged palliation for univentricular heart disease, the mechanism by which hepatic venous flow prevents development of pulmonary arteriovenous malformations is still not completely understood. We present a case in which successful H-type Fontan revision with rerouting of hepatic venous flow through a hemiazygous vein successfully reversed the progression of severe left pulmonary arteriovenous malformations.
|
Introduction
|
|---|
Pulmonary venous malformations (AVM) have been known to develop after a classic or bidirectional Glenn anastomosis owing to the exclusion of hepatic venous blood or "hepatic factor" to the lungs. This malformation can develop in one or both lungs if preferential blood flow is present. Surgical palliation to incorporate the inferior vena cava and hepatic veins into the pulmonary circulation can vary depending on the single ventricle anatomy.
The patient is a 6-year-old girl born with left dominant atrioventricular canal, situs inversus, and inferior vena cava (IVC) interruption with hemiazygous continuation to the left superior vena cava (SVC). The hepatic vein confluence drained into the common atrium. The child underwent pulmonary artery banding as a newborn. At 6 months of age, a left-sided bidirectional Glenn anastomosis was performed, and the pulmonary valve was oversewn. A nonfenestrated extracardiac Fontan procedure was performed at 4 years of age utilizing a 16-mm Gore-Tex conduit (W.L. Gore, Flagstaff, AZ), connecting the hepatic veins to the right pulmonary artery. Bilateral pulmonary AVMs developed between the Glenn and Fontan procedures. After the third palliative stage, the left pulmonary artery was supplied by the left-sided SVC and the hemiazygous system, with central continuity of the pulmonary arteries (Fig 1).
View larger version (31K):
[in this window]
[in a new window]
|
Fig 1. Anterior view of nonfenestrated extracardiac Fontan connecting the hepatic veins to the right pulmonary artery.
|
|
Over the ensuing 2 years, the patient's cyanosis worsened. Serial echocardiograms documented no obstruction of the Fontan circuit. The patient underwent cardiac catheterization, and marked worsening of left pulmonary AVMs was confirmed. The right lung, receiving hepatic venous flow through the extracardiac conduit, was free of pathology. We hypothesized that revision of the Fontan circuit to redirect the hepatic venous flow into the hemiazygous system would hinder the progression of the left lung AVMs. Preoperative oxygen saturations ranged between 68% and 70% on room air. The patient underwent an H-type Fontan revision through a left sixth intercostal space thoracotomy. The previously placed Gore-Tex conduit was dissected free for about 2 cm cephalad to the anastomosis with the hepatic confluence. The hemiazygous vein measured approximately 15 mm in diameter. The Gore-Tex extracardiac conduit was divided and its superior end oversewn. A 14-mm Gore-Tex graft extension was interposed between the conduit stump (end-to-end) and the hemiazygous vein (end-to-side), using a running 5-0 polypropylene suture (Fig 2). Hepatic outflow occlusion time was 38 minutes. The patient was extubated in the operating room on low-dose dopamine infusion, and pain control was provided by a thoracic epidural catheter. The postoperative course was uncomplicated, and the patient was discharged home on the fourth postoperative day, with room air oxygen saturation of 75%.
View larger version (33K):
[in this window]
[in a new window]
|
Fig 2. Anterior view of the extracardiac conduit divided and oversewn. The graft extension is sutured between the conduit stump and the hemiazygous vein. The hepatic veins are rerouted into the hemiazygous vein.
|
|
Systemic oxygen saturation was 85% at 3 weeks and 94% at 7 weeks postoperatively. Follow-up cardiac catheterization at 7 months postoperative showed significant regression of left lung pulmonary AVMs and no right pulmonary pathology (Figs 3 and 4). Oxygen saturations were 93% to 94% at the time of the cardiac catheterization with saturations reported as high as 98% at clinic visits. A contrast echocardiogram in the left pulmonary artery showed mild opacification of the left atrium.
View larger version (128K):
[in this window]
[in a new window]
|
Fig 3. Preoperative angiographic anterior view of right- and left-sided pulmonary arteriovenous malformations.
|
|
View larger version (119K):
[in this window]
[in a new window]
|
Fig 4. Postoperative angiographic anterior view of regression of right and left-sided pulmonary arteriovenous malformations.
|
|
|
Comment
|
|---|
The etiology of pulmonary AVMs after cavopulmonary connection is unknown. Unequal distribution of venous flow in cases with interrupted IVC producing unilateral pulmonary AVMs has been described [2, 6]. The importance of a "hepatic factor" in the prevention of AVMs has been hypothesized [1–4]. Treatment options are limited. Shah and coworkers [5] illustrated the importance of revising the lateral tunnel to include the hepatic veins into the Fontan circuit. Baskett and colleagues [1] described connecting the hepatic veins directly to the azygos vein, eliminating the need for an extracardiac conduit or intraatrial lateral tunnel with artificial material, allowing for potential growth.
In the case presented, in spite of continuous central pulmonary arteries, the rapid progression of left pulmonary AVMs was most likely secondary to preferential right-sided flow of hepatic venous drainage. The regression of unilateral severe pulmonary AVMs after revision of the extracardiac Fontan conduit to an H configuration was evident on follow-up cardiac catheterization, confirming the need for balanced bilateral hepatic venous flow to the pulmonary vasculature to prevent or regress pulmonary AVMs.
We recommend utilizing the extracardiac conduit to redirect the hepatic veins from the common atrium to the azygos vein through a sternotomy incision at the time of the initial Fontan procedure to provide the best distribution of hepatic flow to both lungs.
|
References
|
|---|
- Baskett R.J.F., Ross D.B., Warren A.E., Sharratt G.P., Murphy D.A. Hepatic vein to the azygous vein anastomosis for pulmonary arteriovenous fistulae. Ann Thorac Surg 1999;68:232-233.[Abstract/Free Full Text]
- Knight W.B., Mee R.B. A cure for pulmonary arteriovenous fistulas?. Ann Thorac Surg 1995;59:999-1001.[Abstract/Free Full Text]
- Justino H., Benson L.N., Freedom R.M. Development of unilateral pulmonary arteriovenous malformations due to unequal distribution of hepatic venous flow. Circulation 2001;103:e39-40.[Free Full Text]
- Schneider D.J., Banerjee A., Mendelsohn A.M., Norwood W.I. Hepatic venous malformation after modified Fontan procedure with partial hepatic vein exclusion. Ann Thorac Surg 1997;63:1177-1179.[Abstract/Free Full Text]
- Shah M.J., Rychik J., Fogel M.A., Murphy J.O., Jacobs M.L. Pulmonary AV malformations after superior cavopulmonary connection: resolution after inclusion of hepatic veins in the pulmonary circulation. Ann Thorac Surg 1997;63:960-963.[Abstract/Free Full Text]
- Srivastava D., Preminger T., Lock J.E., et al. Hepatic venous blood and the development of pulmonary arteriovenous malformations in congenital heart disease. Circulation 1995;92:1217-1222.[Abstract/Free Full Text]
This article has been cited by other articles:
|
|
|
|
 
A. L. Marsden, A. J. Bernstein, V. M. Reddy, S. C. Shadden, R. L. Spilker, F. P. Chan, C. A. Taylor, and J. A. Feinstein
Evaluation of a novel Y-shaped extracardiac Fontan baffle using computational fluid dynamics.
J. Thorac. Cardiovasc. Surg.,
February 1, 2009;
137(2):
394 - 403.e2.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
D. D. Soerensen, K. Pekkan, D. de Zelicourt, S. Sharma, K. Kanter, M. Fogel, and A. P. Yoganathan
Introduction of a New Optimized Total Cavopulmonary Connection
Ann. Thorac. Surg.,
June 1, 2007;
83(6):
2182 - 2190.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
D. A. de Zelicourt, K. Pekkan, J. Parks, K. Kanter, M. Fogel, and A. P. Yoganathan
Flow study of an extracardiac connection with persistent left superior vena cava
J. Thorac. Cardiovasc. Surg.,
April 1, 2006;
131(4):
785 - 791.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
J. W. Brown, M. Ruzmetov, P. Vijay, M. D. Rodefeld, and M. W. Turrentine
Pulmonary Arteriovenous Malformations in Children After the Kawashima Operation
Ann. Thorac. Surg.,
November 1, 2005;
80(5):
1592 - 1596.
[Abstract]
[Full Text]
[PDF]
|
|
|
Top
Abstract
Introduction
