Ann Thorac Surg 2005;80:1113-1115
© 2005 The Society of Thoracic Surgeons
Case report
Anomalous Hepatic Venous Drainage
Georgios P. Georghiou, MD
*
,
Eldad Erez, MD,
Elchanan Bruckheimer, MBBS,
Ovadia Dagan, MD,
Bernardo A. Vidne, MD,
Einat Birk, MD
Department of Cardiothoracic Surgery, Rabin Medical Center, Beilinson Campus and Heart Institute, Schneider Children’s Medical Center of Israel, Petah Tikva, Israel both affiliated with the Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel
Accepted for publication March 2, 2004.
* Address reprint requests to Dr Georghiou, Department of Cardiothoracic Surgery, Rabin Medical Center, Beilinson Campus, Petah Tikva49 100, Israel; (Email: georgios{at}clalit.org.il).
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Abstract
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We present a 3-year-old boy born with anomalous hepatic venous drainage into the left atrium and a small sinus venosus atrial septum defect, in whom pulmonary arteriovenous malformations developed with progressive cyanosis. Surgical redirection of the hepatic venous drainage to the right atrium and closure of the atrial septal defect led to regression of the pulmonary arteriovenous malformations. However, in contrast to other reports, progressive pulmonary hypertension developed postoperatively.
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Introduction
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Pulmonary arteriovenous malformations (PAVMs) may develop after a Glenn shunt in up to 25% of cases [1]. The cause is unknown, although maldistribution of the pulmonary blood flow to the upper and lower lobes, and the nonpulsatile blood flow, have been implicated [1]. The development of PAVMs in patients with congenital heart disease may be related to the diversion of the normal hepatic venous flow from the pulmonary circulation and the subsequent lack of hepatic factor (effluent) into the pulmonary vascular bed [2]. Case reports have shown that the redirection of hepatic flow to the pulmonary bed may lead to reversal of PAVMs [[3, 4]. We present a patient with anomalous drainage of all hepatic veins into the left atrium and a small sinus venosus atrial septal defect who developed clinically significant PAVMs. The PAVMs resolved with surgical redirection of the hepatic venous return to the pulmonary arterial system and closure of the atrial septal defect (ASD). However, in contrast to other reports, progressive pulmonary hypertension developed postoperatively.
Our patient presented at 3 years of age with progressive cyanosis of 1-year duration accompanied by easy fatigue on exercise. There were no indications of hepatic disease. He had been completely asymptomatic up to 2 years of age, with no evidence of cyanosis or congestive heart failure. On physical examination, clubbing was noted. Oxygen saturation was 74% in room air, and the hemoglobin level was 21 g/dL. The electrocardiogram was normal. A chest roentgenogram revealed nonspecific lung markings. Echocardiography demonstrated an interrupted inferior vena cava, with azygous continuation to a right superior vena cava and a small sinus venosus ASD. A heart catheterization revealed anomalous drainage of all hepatic veins into the left atrium and a small sinus venosus ASD with no measurable cross shunting. Pulmonary venous anatomy was normal. Hemodynamic findings were an elevated pulmonary pressure to 50% of the systemic pressure and a calculated pulmonary vascular resistance of 2.4 Wood units, indicating the presence of mild-to-moderate pulmonary hypertension. Contrast echocardiography confirmed the presence of PAVMs.
During the operation, a small sinus venosus ASD measuring about 5 mm was found in the area posterior to the eustachian valve. An incision of the atrial septum revealed a single, large hepatic vein and a coronary sinus entering the left atrium. Treatment consisted of corrective diversion of the hepatic flow and coronary sinus from the left to the right atrium with an autologous pericardial patch, followed by primary closure of the ASD. There were no intraoperative complications. Postoperatively, oxygen saturation improved to 83%. Pulmonary pressure at discharge, as estimated by Doppler gradient across the tricuspid valve, was less than 50% of the systemic pressure.
Five months after surgery, an improved level of activity was noted with no further signs of cyanosis. Oxygen saturation gradually increased to 96% in room air. Echocardiography 6 months after surgery demonstrated increasing right ventricular and pulmonary pressure. A repeated cardiac catheterization showed good functioning of the surgical baffle and no evidence of intracardiac shunting. Contrast echocardiography of both pulmonary arteries showed only minimal shunting through residual PAVMs on the right side, with complete resolution in the left lung. However, pulmonary artery pressure was elevated to 90% of the systemic pressure, and the calculated pulmonary vascular resistance measured 8.2 Wood units, with only minimal response to the administration of inhaled 100% oxygen and nitric oxide.
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Comment
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PAVMs that complicate a classic Glenn shunt operation may be caused by either the lack of an as yet unidentified vasoactive hepatic factor or the absence of pulsatile pulmonary blood flow. PAVMs have not been found after the Fontan operation, even when specifically sought during examination [1]. Two exceptions were 2 patients who also had bidirectional cavopulmonary Glenn anastomoses that included all venous return except hepatic and coronary sinus blood [5]. In addition, in a series of 10 patients with congenital heart disease who developed PAVMs, the clinical description identified a common denominator, namely, the exclusion of normal hepatic venous blood flow from the affected pulmonary circulation [2]. These PAVMs were similar in angiographic and histologic appearance to PAVMs associated with liver disease. These data strongly suggest that normal hepatic venous blood flow has a role in the prevention of PAVMs. Patients with hepatic cirrhosis develop vascular dilatations [6] at the pulmonary precapillary and capillary levels. These are thought to cause diffusion and perfusion defects that result in cyanosis.
The association of PAVM with liver disease is well documented and is termed hepatopulmonary syndrome [7]. Other major features of the syndrome include an increased alveolar and arterial gradient while the patient is breathing room air and evidence of 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, similar to the PAVM associated with the Glenn shunt.
The recent evidence of the reversibility of PAVMs after liver transplantation in patients with cirrhosis raises the possibility that the same effect may be induced in some affected patients with congenital heart disease by redirecting the hepatic venous flow to the pulmonary bed [4]. In our patient, the improved oxygen saturation to 96% in room air at 5 months after the hepatic venous flow was surgically redirected to the right heart and pulmonary arterial system was indicative of a resolving right-to-left shunt.
Our survey of the literature revealed 15 cases of anomalous hepatic venous drainage into the left atrium. In 7 cases the hepatic venous drainage was a result of a cardiac surgical shunt procedure [8]; in 2 cases it was because of unintentional surgical exclusion of the hepatic venous flow from the pulmonary circulation [3]; and in 6 cases it was congenital [4, 8].
Our case is unique because the patient did not have a surgically created cavopulmonary anastomosis. Redirection of the hepatic venous flow led to an ongoing process of resolution of the PAVMs. Our patient’s outcome provides support for the assumption that it is the long-term lack of a hepatic effluent in the pulmonary vasculature tract that causes the progressive development of PAVMs. Pulmonary hypertension developed in our patient after the redirection of an anomalous hepatic drainage.
Mild-to-moderate pulmonary hypertension, with only mildly elevated pulmonary vascular resistance, was documented in our patient before surgery; however, both pulmonary pressure and resistance were markedly elevated 6 months later. It is possible that the patient already had pulmonary vascular disease before surgery that went undetected during the preoperative catheterization because the multiple large PAVMs that were present provided a decompression mechanism to the pulmonary arterial tree. Once the PAVMs resolved (after the hepatic blood flow was redirected to the pulmonary circulation), the pulmonary vascular resistance became manifest.
We suggest that the development of PAVMs should be considered in patients with anomalous hepatic venous return who become cyanotic. PAVMs that are due to pulmonary arterial exclusion of hepatic venous return are potentially reversible once hepatic blood returns to the pulmonary circulation. If pulmonary hypertension is detected before the operation, it is unclear if corrective surgery is necessary.
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References
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- Cloutier A, Ash JM, Smallhorn JF, et al. Abnormal distribution of pulmonary blood flow after Glenn shunt or Fontan procedurerisk of development of arteriovenous fistulae. Circulation 1985;72:471-479.[Abstract/Free Full Text]
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- Agnoletti G, Borghi A, Annecchino FP, Crupi G. Regression of pulmonary fistulas in congenital heart disease after redirection of hepatic venous flow to the lungs Ann Thorac Surg 2001;72:909-911.[Abstract/Free Full Text]
- Lee J, Menkis AH, Rosenberg HC. Reversal of pulmonary arteriovenous malformation after diversion of anomalous hepatic drainage Ann Thorac Surg 1998;65:848-849.[Abstract/Free Full Text]
- 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]
- Krowka MJ, Cortese DA. Pulmonary aspects of liver disease and transplantation Clin Chest Med 1989;10:593-615.[Medline]
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- Stoller JK, Hoffman RM, White RD, Mee RBB. Anomalous hepatic venous drainage into the left atriuman unusual case of hypoxemia. Respir Care 2003;48:58-62.[Medline]
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Abstract
Introduction
