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Ann Thorac Surg 2001;72:909-911
© 2001 The Society of Thoracic Surgeons

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Case report

Regression of pulmonary fistulas in congenital heart disease after redirection of hepatic venous flow to the lungs

^a Division of Cardiology, Ospedali Riuniti, Bergamo, Italy
^b Division of Cardiac Surgery, Ospedali Riuniti, Bergamo, Italy

Accepted for publication October 18, 2000.

Address reprint requests to Dr Agnoletti, Via C. Battisti, 43C, 25073 Bovezzo (BS), Italy
e-mail: gabriellaagnoletti{at}libero.it

	Abstract

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Although the etiology of pulmonary fistulas in congenital heart disease remains unknown, several hemodynamic factors are thought to play a role. Recently it was postulated that hepatic venous effluent might protect the pulmonary bed from development of pulmonary fistulas. However, there are no experimental data supporting this hypothesis. Here, we present the cases of 2 patients in whom pulmonary fistulas developed after unintentional surgical exclusion of the hepatic venous flow from the pulmonary circulation. In both patients, symptoms resolved after rerouting of the hepatic venous blood to the lungs.

	Introduction

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The development of pulmonary fistulas has been described in patients who have undergone a Glenn anastomosis [1] or a Kawashima operation [2], or who have cirrhosis [3]. The ultimate cause of the development of these fistulas remains unknown, although the maldistribution of pulmonary blood flow to the upper and lower lobes and nonpulsatile blood flow were considered to play a role after the Glenn anastomosis [4]. Inappropriate vasodilation of existing precapillary and capillary alveolar vessels was postulated in patients with cirrhosis [5].

In 1995, it was suggested that hepatic venous effluent might protect the pulmonary bed from the development of pulmonary fistulas [2]. However, no experimental data confirming this hypothesis have been reported. Here, we present the cases of 2 patients in whom pulmonary fistulas developed after unintentional surgical exclusion of the hepatic venous flow from the pulmonary circulation. In both patients, symptoms resolved after the rerouting of the hepatic venous blood to the pulmonary arteries.

	Case reports

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Patient 1
A female baby was born on June 13, 1989, with the diagnosis of cor triatriatum and sinus venosus/atrial septal defect. The patient underwent closure of the atrial septal defect and resection of the supramitral diaphragm at the age of 3 months. She was lost to follow-up until 1999, when she was referred because of dyspnea on effort.

At clinical examination, we observed severe cyanosis (oxygen saturation of 70%) but no murmurs. Hemoglobin level was 21 g/dL. Chest roentgenogram showed a small heart and a reduction in pulmonary flow. Echocardiographic examination indicated a small right ventricle and poor pulmonary flow. Contrast echocardiography showed early opacification of the left atrium, and a radioisotope lung scan revealed intrapulmonary arteriovenous communications. Cardiac catheterization demonstrated the connection between the inferior vena cava and the left atrium and bilateral diffuse pulmonary fistulas (Fig 1).

View larger version (99K): [in this window] [in a new window]   Fig 1. (Patient 1.) Angiograms showing (A) connection between inferior vena cava (ivc) and left atrium (la) and (B) diffuse bilateral pulmonary fistulas. (lv = left ventricle.)

Patient 2
A female baby was born on October 22, 1997, with the diagnosis of biliary atresia, single atrium, interrupted inferior vena cava, and double-outlet right ventricle. Pulmonary artery banding was performed when she was 1 month old and portoenterostomy, when she was 2 months of age. At that time, peripheral oxygen saturation and results of pulmonary scintigraphy were normal. Atrial septation and rerouting of the left ventricle to the aorta were performed when she was 6 months old and orthotopic liver transplantation, 3 months later.

From the time she was 10 months old, clinical findings indicated cyanosis (oxygen saturation of 68% and hemoglobin level of 16.7 g/dL). Echocardiographic examination showed drainage of the hepatic veins to the left atrium, contrast echocardiography revealed early opacification of the left atrium, and pulmonary scintigraphy demonstrated an intrapulmonary shunt. Cardiac catheterization showed the connection of the hepatic veins to the left atrium as well as early opacification of the left atrium after an injection into the pulmonary trunk.

Rerouting of the hepatic veins to the right atrium was performed on May 10, 1999. Severe cyanosis was still present after the intervention. Echocardiographic examination showed drainage of the hepatic veins to the right atrium. Two months later, regression of cyanosis (97% oxygen saturation) and normalization of both contrast echocardiography and pulmonary scintigraphy were observed.

	Comment

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It is well known that pulmonary fistulas are frequently observed in patients with a classic Glenn anastomosis [1] and in patients with liver diseases [3]. The etiology of pulmonary fistulas is still debated. The development of anomalous arteriovenous communications bypassing the alveolus was demonstrated in patients with congenital heart disease [6]; conversely, abnormal dilatation of the existing precapillary and capillary vessels, with a diffusion/perfusion defect, was thought to play a role in patients with liver disease [5].

In patients with a Glenn anastomosis, pulmonary fistulas were suggested to result from maldistribution of flow with a decreased upper lobe to lower lobe perfusion ratio [1], low pulsatile pulmonary blood flow [4], pulmonary hypertension in the nonshunted lung [1], and increased perfusion and pressure in the lower lung fields [4]. The development of pulmonary fistulas was shown to be related to the time interval since the Glenn shunt, the presence of pulmonary hypertension in the contralateral lung, and age at the time of Glenn anastomosis [1].

Although the distribution of pulmonary blood flow and the lack of pulsatile blood flow after the Fontan operation are similar to those observed after the Glenn anastomosis, pulmonary fistulas were not noted after the Fontan operation [4]. In 1995, Srivastava and colleagues [6] reported their experience with 10 patients with pulmonary fistulas. All 10 patients had exclusion of normal hepatic venous flow from the affected pulmonary circulation, and bilateral pulmonary fistulas were observed when both lungs were deprived of hepatic venous flow. The authors postulated that occurrence of pulmonary fistulas in patients with congenital heart disease could be related to the diversion of normal hepatic venous flow from the pulmonary circulation and that pulmonary fistulas share a common pathogenesis with the vascular malformations observed in cirrhotic patients [3].

On the basis of these findings, it was hypothesized that hepatic venous effluent contains a substance that, by inhibiting endogenous vasodilation or promoting vasoconstriction, maintains the functional and anatomic integrity of the pulmonary capillaries [2]. Conversely, in patients with liver disease, the release of a substance stimulating the formation of fistulas or the inability to metabolize vasoactive substances present in portal venous blood [5] could be postulated. It was shown that pulmonary fistulas can regress once blood containing an adequate amount of this substance is supplied to the lungs. In patients with cirrhosis, orthotopic liver transplantation was shown to reverse cyanosis, whereas cyanosis can recur in the case of graft failure [3]. In patients with a Glenn anastomosis, an auxiliary arteriovenous fistula can prevent the development of the fistulas [7].

Our data strongly support the hypothesis that hepatic venous flow plays a role in protecting the pulmonary bed from the development of pulmonary fistulas. In our patients, a previous surgical error had caused the exclusion of hepatic venous flow from the pulmonary bed, thus giving us the opportunity to demonstrate that pulmonary fistulas develop after such exclusion. Regression of the fistulas occurs when hepatic flow is redirected to the right atrium.

Both patients had pulsatile pulmonary blood flow; neither of them had maldistribution of flow to upper or lower lung fields or pulmonary hypertension. Both children were pink before correction of the cardiac defect, but still manifested definite cyanosis after the rerouting of the inferior vena cava or the hepatic veins to the right atrium, thus ruling out the possibility that cyanosis was due simply to the diversion of unsaturated blood to the systemic arterial bed. A few months after operation, peripheral oxygen saturation returned to normal in both children. Although several endothelial or fibroblastic angiogenic factors are currently under investigation, a labile substance regulating angiogenesis has not yet been isolated.

	References

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1. Kopf G.S., Laks H., Stansel H.C., Hellenbrand W.E., Kleinman C.S., Talner N.S. Thirty-year follow-up of superior vena cava–pulmonary artery (Glenn) shunts. J Thorac Cardiovasc Surg 1990;100:662-671.[Abstract]
2. Knight W.B., Mee R.B.B. A cure for pulmonary arteriovenous fistulas?. Ann Thorac Surg 1995;59:999-1001.[Abstract/Free Full Text]
3. Fewtrell M.S., Nobel-Jamieson G., Revell S., et al. Intrapulmonary shunting in the biliary atresia/polysplenia syndrome: reversal after liver transplantation. Arch Dis Child 1994;70:501-504.[Abstract]
4. Cloutier A., Ash J.M., Smallhorn J.F., et al. Abnormal distribution of pulmonary blood flow after the Glenn shunt or Fontan procedure: risk of development of arteriovenous fistulae. Circulation 1985;72:471-479.[Abstract/Free Full Text]
5. Krowka M.J., Cortese D.A. Hepatopulmonary syndrome: an evolving perspective in the era of liver transplantation. Hepatology 1990;11:138-142.[Medline]
6. 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]
7. Glenn W.W.L. Superior vena cava–pulmonary artery anastomosis. Ann Thorac Surg 1984;37:9-11.[Medline]

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This Article Abstract Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to Personal Folders Download to citation manager Author home page(s): Francesco Paolo Annecchino Giancarlo Crupi Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Agnoletti, G. Articles by Crupi, G. Search for Related Content PubMed PubMed Citation Articles by Agnoletti, G. Articles by Crupi, G. Related Collections Congenital - cyanotic