Ann Thorac Surg 2004;77:1825-1827
© 2004 The Society of Thoracic Surgeons
Case report
Resolution of pulmonary arteriovenous fistula by redirection of hepatic venous blood
Hajime Ichikawa, MDa*,
Norihide Fukushima, MDa,
Masamichi Ono, MDa,
Tomoko Kita, MDa,
Toru Matsushita, MDa,
Yuji Miyamoto, MDa,
Hikaru Matsuda, MDa
a Department of Surgery, Osaka University, Osaka, Japan
Accepted for publication June 3, 2003.
* Address reprint requests to Dr Ichikawa, Department of Surgery, Osaka University, 2-2 Yamadaoka, Suita, Osaka 565-0871, Japan
e-mail: ichikawa{at}surg1.med.osaka-u.ac.jp
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Abstract
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We report an 8-year-old girl whose unilateral pulmonary arteriovenous fistula (PAVF) disappeared after we redirected hepatic venous blood to the affected lung. This case provides strong evidence that formation of a PAVF depends only on the presence or absence of hepatic venous blood in the pulmonary circulation.
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Introduction
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Pulmonary arteriovenous fistula (PAVF) formation in a patient with left isomerism is still a major cause of morbidity after a total cavopulmonary shunt (TCPS) operation [1]. Currently, an absence of hepatic venous blood from the pulmonary circulation is thought to cause the development of PAVF. This theory is supported by the finding that, in liver cirrhosis patients, PAVF disappears after liver transplantation [2] or heart transplantation. Conversion from TCPS to Fontan, by redirecting blood from the hepatic vein to the pulmonary circulation, has been reported to be effective for the treatment of PAVF [3]. However, a beneficial effect is not always obtained. Here, we report an 8-year-old girl whose unilateral PAVF disappeared after redirection of the hepatic venous blood to the affected lung.
A 4-year-old girl was referred to our hospital with a diagnosis of left isomerism, dextrocardia, inferior vena cava absence, hemiazygos continuation, unbalanced atrioventricular septal defect, hypoplastic left ventricle, d-transposition of great arteries, s/p coarctation repair, and pulmonary artery banding. Since this patient was considered to be a marginal candidate for a Fontan-type operation (because of the high pulmonary vascular resistance), a TCPS operation was performed when she was 4-years old. Her arterial oxygen saturation (SaO2) was improved after the surgery from the mid 70s to the mid 80s (%). A week later, however, her SaO2 decreased to the low 80 seconds. Cardiac catheterization and contrast echo one month after the operation revealed the development of a PAVF. Her SaO2 decreased further to the mid 70s at that time. During this postoperative period, she had been on home oxygen therapy (HOT). Cardiac catheterization was repeated the next year, and revealed an increase in the hemiazygos-to-portal-hepatic shunt. Although her pulmonary arterial pressure (PAP) was not low (mean PAP = 14 mm Hg), it was decided to carry out a conversion to a fenestrated Fontan circulation by connecting the hepatic vein to the pulmonary circulation. She was admitted to our hospital, and the operation was performed using an extracardiac conduit with a relatively large (5-mm Gore-Tex tube) fenestration. After this operation, her SaO2 improved from the mid 70s to the high 80s. Cardiac catheterization after the operation showed that there were still shunts from the hemiazygos to the single atrium, but not to the portal-hepatic vein.
One year after the Fontan operation, her oxygen saturation once again started to decrease. Cardiac catheterization revealed that the hepatic venous blood was being directed only to the right lung (Fig 1A).
PA graphy (Fig 1B) and a simultaneous contrast echo revealed a highly developed PAVF only in the left lung. The hemiazygos-to-systemic shunts were still present (Fig 2). Use of prostacyclin or HOT showed no effect. Therefore, it was decided to revise the hepatic vein to pulmonary artery (HV-PA) conduit so as to supply hepatic venous blood to the left lung, in the hope that the PAVF would decrease.
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Fig 1. (A) Hepatic venous blood is only directed to the right lung. (B) There are numerous PAVF in the left lung and no PAVF in the right lung. (PAVF = pulmonary arteriovenous fistula.)
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At the operation, a severe intimal thickening was found in the equine pericardial roll. The fenestration was found also to be completely occluded by a fibrin clot. The fibrous intima inside the graft, which extended to the hepatic vein, was removed. A small equine pericardial patch was applied to reshape the HV-PA conduit so as to introduce balanced blood flow from HV to the both lungs. Her postoperative course was uneventful, and SaO2 was markedly improved. Two months after this operation, cardiac catheterization (Fig 3)
showed an evenly distributed blood flow from HV to PA and almost no PAVF. The clinical course is summarized in Fig 4.
There was also a marked reduction in the amount of bubbles seen in a contrast echo by selective injection of bubbled saline into the left PA. Her oxygen saturation has remained in the low to mid 90s for 6 months after the operation.
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Fig 3. Evenly distributed hepatic venous blood to both lungs. Resolution of pulmonary arteriovenous fistula in the left lung is also seen.
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Fig 4. Schematic summary of the clinical course. (HV-PA = hepatic vein to pulmonary artery; PAVF = pulmonary arteriovenous fistula; TCPS = total cavopulmonary shunt.)
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Comment
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The formation of PAVF after TCPS in patients with left isomerism has been reported to be critical; patients with PAVF usually having a poor prognosis. There is still debate as to whether a PAVF is reversible once it has developed [4]. In a previous case, we noted no improvement in SaO2 following insertion of an HV-to-PA shunt in a 16-year-old left isomerism patient who had developed severe PAVF after a TCPS operation, and that patient died of hypoxia two years after the operation. In the present case, the PAVF was reversible by ensuring that the left lung was perfused with hepatic venous blood. Although several developed in the patient, veno-venous and veno-portal shunts together with her PAVF, the final oxygen saturation was more than 90%. There are several possible candidates for the humoral factor responsible for PAVF formation [5], but which factor actually is responsible remains unclear. As we found no PAVF in the right lung and multiple PAVF in the left lung in the same patient at the same time, it is reasonable to submit this case as strong evidence that the cause of PAVF is exclusion of HV blood from the pulmonary circulation, particularly as the PAVF disappeared almost completely after the hepatic blood had been redirected to the affected lung. In our clinical experience, this patient is the youngest yet to receive this type of conversion, although we have had patients of the same kind whose Fontan completion was achieved at a younger age, and who did not develop PAVF. Thus, age at operation may be a major factor influencing the development and reversibility of PAVF.
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References
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- Stumper O., Wright J.G., Sadiq M., De Giovanni J.V. Late systemic desaturation after total cavopulmonary shunt operations. Br Heart J 1995;74:282-286.[Abstract/Free Full Text]
- Stoller J.K., Moodie D., Schiavone W.A., et al. Reduction of intrapulmonary shunt and resolution of digital clubbing associated with primary biliary cirrhosis after liver transplantation. Hepatology 1990;11:54-58.[Medline]
- Knight W.B., Mee R.B. A cure for pulmonary arteriovenous fistulas?. Ann Thorac Surg 1995;59:999-1001.[Abstract/Free Full Text]
- Uemura H., Yagihara T., Hattori R., Kawahira Y., Tsukano S., Watanabe K. Redirection of hepatic venous drainage after total cavopulmonary shunt in left isomerism. Ann Thorac Surg 1999;68:1731-1735.[Abstract/Free Full Text]
- Starnes S.L., Duncan B.W., Kneebone J.M., et al. Pulmonary microvessel density is a marker of angiogenesis in children after cavopulmonary anastomosis. J Thorac Cardiovasc Surg 2000;120:902-907.[Abstract/Free Full Text]
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Abstract
Introduction
