Ann Thorac Surg 2006;81:1890-1892
© 2006 The Society of Thoracic Surgeons
Case report
Heterotaxy Syndrome with Azygous Continuation-Causing Pseudo Budd-Chiari Syndrome After Cardiopulmonary Bypass
Jain Bhaskara Pillai, FRCS (C-Th) UK
a
,
*
,
Jacques Kpodonu, MD
a
,
Catherine Yu, FRCPC
b
,
Michael A. Borger, MD, PhD
a
a Division of Cardiac Surgery, Toronto General Hospital, Toronto, Ontario, Canada
b Division of Hepatology, Toronto General Hospital, Toronto, Ontario, Canada
Accepted for publication May 24, 2005.
* Address correspondence to Dr Pillai, 17 Paxford Close, Vicars Ln, Benton, Newcastle upon Tyne, NE7 7PA United Kingdom (Email: jain_freeman{at}hotmail.com).
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Abstract
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Heterotaxy syndrome with interruption of the inferior vena cava and direct hepatic vein inflow into the right atrium has important implications for cardiac surgery. We report a case causing pseudo Budd-Chiari syndrome after cardiopulmonary bypass.
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Introduction
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Modern cardiac surgery pivots on the conduct of safe cardiopulmonary bypass. Any condition that compromises the extracorporeal circulation has due importance for the surgeon and the patient. We describe how an unrecognized congenital anomaly had significant implication for safe right atrial venous cannulation.
A 48-year-old woman underwent routine coronary artery bypass surgery for unstable angina. A standard two-stage venous cannula was inserted into the right atrium with some resistance at the inferior caval orifice. Visual inspection of the inferior-cavoatrial region in the pericardial well and the superior vena cava seemed unremarkable. A smaller cannula was successfully inserted. Some resistance was felt during insertion, but there was good venous return and no difficulties thereafter. Her liver function tests showed an acute deterioration with aspartate aminotransferase and alanine aminotransferase peaking at 3,000 IU/L and international normalized ratio of 2.5 on postoperative day 5. All preoperative liver function tests were normal. Her hepatitis B and C results were negative. Apart from moderate abdominal discomfort, the patient remained clinically well. Echocardiography showed normal cardiac function. A computed tomographic scan of the abdomen revealed polysplenia syndrome with azygous continuation (Figs 1 and 2).
The biochemical abnormalities gradually recovered by postoperative day 14.
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Fig 1. (A) An absent intrahepatic inferior vena cava with azygous continuation (AzCont) of the inferior vena cava. (B) Hepatic vein (HV) draining directly into the right atrium.
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Fig 2. (A) Polysplenia with three spleens (Sp). (B) Truncated pancreas (P) with absent body and tail. The (congested and enlarged) liver (L) and stomach (St) were normally literalized. (Other features not shown on the selected computed tomographic imaging frames: malrotation of midgut with duodeno-jejunal junction to the right of the midline and jejunal loops centered in the right upper quadrant of the abdomen.)
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Comment
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A continuum of complex embryologic events molds the mature fetal viscero-vascular anatomy. The three main groups of early embryonic veins are the vitelline, umbilical, and cardinal vein complexes. The inferior vena cava is a composite embryologic structure, mainly formed from the cardinal vein complex. The hepatic veins are formed from the hepatocardiac vitelline vein.
Absence of the intrahepatic and other segments of the inferior vena cava represent an interruption of a right-sided structure. The venous return is restored by the azygous or hemi-azygous continuation into the superior vena cava, or more rarely into the intrahepatic veins [1]. The azygous vein originates from the posterior aspect of the inferior vena cava at the level of the renal veins. Azygous continuation is a hallmark of the polysplenia syndrome, a condition within the spectrum of heterotaxy or situs ambiguous syndromes [2], which includes polysplenia and asplenia. Heterotaxy is defined by a defect of lateralization, indexed by abnormalities of the spleen with accompanying visceral malposition and maldevelopment. Inheritance may be genetic, although most cases are sporadic.
Polysplenia reflects bilateral left-sidedness. Markers include left-lung bi-lobation of both lungs and a midline bridging liver. Asplenia indicates right isomerism. In both, the laterality of the stomach is variable. Congenital cardiac involvement is an important feature and a major cause of mortality. Cardiac anomalies are less common, less severe, or even absent with polysplenia. Hence adult patients commonly present with the polysplenia variant. Our patient gave a long history of recurrent abdominal distension, and diarrhea with a diagnosis of irritable bowel syndrome. In retrospect, this is likely be related to her midgut malrotation, which is one of the more constant features in heterotaxy.
Clinical implications of the anomalous venous drainage include lower limb lymphedema, lower extremity, and pelvic vein thrombosis. Mouton and Zehnder [3] found 19 documented cases of deep vein thrombosis, although other risk factors for thrombosis were often present. Enlarged azygous-hemi-azygous paravertebral plexus with multiple venectasis have been misdiagnosed as a mediastinal mass or abdominal lymphoma with longer interruptions of the inferior vena cava [3]. Enlarged vessels lying parallel to the descending thoracic aorta have also been misdiagnosed on transesophageal echocardiography for aortic dissection, aneurysm, or partial rupture [4]. Azygous continuation is rarely seen in asplenia.
The unsuspected anomaly in our patient led to partial obstruction of venous drainage from the liver by the venous cannula during the 52 minutes of cardiopulmonary bypass. Hepatomegaly, pain, and derangement of liver function due to the iatrogenic hepatic vein obstruction constituted a Budd-Chiari equivalent. Intraoperative transesophageal echocardiography for difficult venous cannulation may have clarified the situation. A single stage cannula should be used with azygous continuation for coronary or aortic surgery. In patients requiring right heart procedure, hypothermia with low flows or circulatory arrest may be required.
In unsuspected polysplenia cases, cardiopulmonary bypass could face significant problems. A high index of suspicion is required to recognize this unusual entity.
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References
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- Balkanci F, Ozmdn MN. Case reportinterruption of the inferior vena cava with anomalous intrahepatic continuation. Br J Radiol 1993;66:457-459.[Abstract/Free Full Text]
- Applegate KE, Goske MJ, Pierce G, Murphy D. Situs revisitedimaging of the heterotaxy syndrome. Radiographics 1999;19:837-852.[Abstract/Free Full Text]
- Mouton WG, Zehnder T. Deep vein thrombosis in azygous continuation Eur J Vasc Endovasc Surg 2003;25:90-92.[Medline]
- Blanchard DG, Sobel JL, Hope J, Raisinghani A, Kermati S, DeMaria AN. Infrahepatic interruption of the inferior vena cava with azygous continuationa potential mimicker of aortic pathology. J Am Soc Echocardiogr 1998;11:1078-1083.[Medline]
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Abstract
Introduction
