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Ann Thorac Surg 1998;65:848-849
© 1998 The Society of Thoracic Surgeons

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Case Reports

Reversal of Pulmonary Arteriovenous Malformation After Diversion of Anomalous Hepatic Drainage

Division of Cardiothoracic Surgery, London Health Sciences Centre, Children’s Hospital of Western Ontario, The University of Western Ontario, London, Ontario, Canada
Department of Paediatrics, Children’s Hospital of Western Ontario, The University of Western Ontario, London, Ontario, Canada

Accepted for publication October 24, 1997.

Dr Menkis, London Health Sciences Centre, University Campus, 339 Windermere Rd, London, ON, Canada N6A 5A5.

	Abstract

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Pulmonary arteriovenous malformation can occur in up to 25% of patients after a classic Glenn shunt. Although unproven, exclusion of hepatic venous blood from the lungs has been proposed as a possible cause. We present a patient born with anomalous hepatic venous drainage into the left atrium with an intact atrial septum in whom pulmonary arteriovenous malformation developed in childhood. This was reversed after diversion of the hepatic venous drainage to the right atrium, supporting exclusion of hepatic venous flow as the cause of pulmonary arteriovenous malformation. The association with the hepatopulmonary syndrome is discussed.

	Introduction

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One of the complications after a classic Glenn shunt is the development of pulmonary arteriovenous malformation (PAVM) [1][2]. Possible causes have included the lack of pulsatile flow and the maldistribution of pulmonary circulation [1][2]. Srivastava and associates [2] reviewed their series of PAVM associated with congenital heart disease and determined that the common denominator in the development of PAVM was the diversion of normal hepatic venous blood flow away from the pulmonary circulation. This report presents a case of anomalous hepatic venous drainage into the left atrium with an associated development of PAVM. The PAVM completely regressed after corrective diversion of hepatic venous blood flow to the right atrium.

The patient had been completely asymptomatic with no evidence of cyanosis or congestive heart failure until the age of 6 years. Over the ensuing 3 years, progressive cyanosis developed and the patient became symptomatic with exercise. Clubbing was noted on examination. The oxygen saturation was 76% with a hemoglobin level of 173 g/L. The electrocardiogram was normal. A chest radiograph revealed tortuous but nonspecific lung markings. An initial echocardiogram demonstrated an interrupted inferior vena cava with azygous continuation to a right superior vena cava but no other abnormalities. Heart catheterization was performed, which revealed anomalous drainage of hepatic veins into the left atrium. There was no intracardiac shunt and PAVM was not clearly demonstrated. Subsequently, a bubble echocardiographic study was carried out, which confirmed PAVM. Computed tomography and magnetic resonance imaging did not show major arteriovenous malformations, suggesting that the defects were at the capillary level.

Operative findings included an intact interatrial septum. The atrial septum was incised, revealing both a single large hepatic vein and a coronary sinus entering the left atrium. The coronary sinus was not unroofed. Surgical repair involved the diversion of the hepatic flow from the left to the right atrium using an autologous pericardial baffle. The coronary sinus remained draining into the left atrium. There were no intraoperative complications. The postoperative oxygen saturation improved to 85% but the cyanosis did not completely resolve. The patient was discharged home on day 10. Three weeks after the operation, the patient demonstrated improved activity level with no further signs of cyanosis. The oxygen saturation was 95%. At the 5-month follow-up, the patient had fully recovered with no evidence of cyanosis or clubbing and with an oxygen saturation of 100%. At the 1-year mark, a bubble echocardiographic study was performed. Bubbles were not seen in the left chambers of the heart. We concluded that the PAVM had fully regressed.

	Comment

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Pulmonary arteriovenous malformation can occur in 19% to 25% of patients after a Glenn shunt [1][2]. The pathogenesis of PAVM has remained elusive. Absence of pulsatile flow has been proposed as a cause of PAVM [2]. This is not likely because PAVM is also well recognized in patients with liver disease in the presence of pulsatile pulmonary blood flow [3]. After a Glenn shunt, the pulmonary blood flow favors the lower lobes, and this maldistribution has been implicated as a possible mechanism for PAVM formation [1][2]. Similar maldistribution in pulmonary flow is seen after a Fontan operation but PAVM has not been recognized as a true complication. Moore and colleagues [4] reported 2 cases of PAVM formation after a modified Fontan operation. In their first patient, the hepatic venous blood was allowed to drain into the left pulmonary artery and PAVM developed only in the right lung 5 years later. The second patient had hepatic veins draining into a common atrium. A modified Fontan operation was carried out whereby the hepatic venous blood was allowed to remain as the only right-to-left shunt. Pulmonary arteriovenous malformations occurred in both lungs within 15 months.

Srivastava and associates [2] reported a series of 10 patients with congenital heart disease in whom PAVM developed after palliative shunts. In 4 of their patients, only one lung received hepatic venous blood. Pulmonary arteriovenous malformation was found only in the lungs that did not receive any hepatic venous blood. In their other 6 patients, PAVM occurred bilaterally and it was noted that both lungs were excluded from exposure to hepatic venous blood. Eight of their patients had heterotaxy syndromes with interrupted inferior vena cava. They determined that inferior vena caval interruption alone did not predispose to PAVM but rather that hepatic venous blood must be excluded from the lungs. Our case report supports the findings of Srivastava and associates and Moore and colleagues. In this case PAVM occurred in a patient with interrupted inferior vena cava and hepatic veins draining into the left atrium and with an intact interatrial septum. The PAVM occurred in the absence of any surgical shunts. The PAVM regressed after corrective diversion of hepatic venous blood to the pulmonary circulation.

The association of PAVM and liver disease is well documented and is termed the hepatopulmonary syndrome [3]. This syndrome is defined as a clinical triad of (1) liver disease, (2) increased alveolar–arterial gradient on room air, and (3) intrapulmonary vascular dilatations. The predominant clinical findings are clubbing, cyanosis, and dyspnea. The PAVM in this syndrome is predominant in the middle and lower lung fields, which is similar to PAVM associated with a Glenn shunt. The formation of these vascular changes may be caused by an imbalance between potential pulmonary vasodilators and vasoconstrictors. An increase in pulmonary vasodilators is favored as the mechanism behind the hepatopulmonary syndrome [3]. Although an exact mediator has not been identified, prostaglandins [5] and nitric oxide [6] are two proposed substances. In an animal model of the hepatopulmonary syndrome [7], it was noted that the hypoxic pulmonary vasoconstriction was severely impaired in the cirrhotic group. This phenomenon, however, was reversible with angiotensin II, a vasoconstrictor. It is conceivable that a similar mechanism is responsible for the formation of PAVM after a classic Glenn shunt. If the liver produces regulatory vasoconstrictors that balance the locally existing pulmonary vasodilators, then liver failure or a diversion of hepatic venous blood away from the lungs would lead to an imbalance favoring vasodilation. The formation of PAVM may then be the response to chronic vasodilatory stimuli. In Srivastava and associates’ series [2], the median duration from the time of cavopulmonary anastamosis to diagnosis of PAVM was 3.5 years. Scott and coworkers [8] described 6 patients in whom cirrhosis-related PAVM was reversed after liver transplantation. In our patient, complete resolution of PAVM occurred after the hepatic venous drainage was restored to the right heart. This case of PAVM reversal in a patient with congenital heart disease provides further evidence that PAVM is potentially reversible once hepatic function returns to normal and its venous blood reaches the lungs.

	References

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1. Cloutier A, Ash JM, Smallhorn JF, 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]
2. Srivastava D, Preminger T, Lock JE, 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]
3. Lange PA, Stoller JK The hepatopulmonary syndrome. Ann Intern Med 1995;122:521-529.[Abstract/Free Full Text]
4. Moore JW, Kirby WC, Madden WA, Gaither NS Development of pulmonary arteriovenous malformations after modified Fontan operations. J Thorac Cardiovasc Surg 1989;98:1045-1050.[Abstract]
5. Shijo H, Sasaki H, Yuh K, et al. Effects of indomethacin on hepatogenic pulmonary angiodysplasia. Chest 1991;99:1027-1029.[Abstract/Free Full Text]
6. Pizcueta P, Pique JM, Fernandez M, et al. Modulation of the hyperdynamic circulation of cirrhotic rats by nitric oxide inhibition. Gastroenterology 1992;103:1909-1915.[Medline]
7. Chang S, O’Hara N Pulmonary circulatory dysfunction in rats with biliary cirrhosis: an animal model of the hepatopulmonary syndrome. Am Rev Respir Dis 1992;145:798-805.[Medline]
8. Scott V, Miro A, Kang Y, et al. Reversibility of the hepatopulmonary syndrome by orthotopic liver transplantation. Transplant Proc 1993;25:1787-1788.[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): John Lee Alan H. Menkis Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Lee, J. Articles by Rosenberg, H. C. Search for Related Content PubMed PubMed Citation Articles by Lee, J. Articles by Rosenberg, H. C.