Images of Pediatric and Congenital Heart Disease
Infracardiac Total Anomalous Pulmonary Venous Return: An Unusual Cause of Neonatal Portal Vein Enlargement
World Journal for Pediatric and Congenital Heart Surgery 2014, Vol 5(1) 131-132 ª The Author(s) 2014 Reprints and permission: sagepub.com/journalsPermissions.nav DOI: 10.1177/2150135113504307 pch.sagepub.com
Anmol Bhatia, MD1, Kushaljit S. Sodhi, MD1, Akshay K. Saxena, MD1, Manphool Singhal, MD1, and Niranjan Khandelwal, MD1
Abstract We present an unusual cause of aneurysmal dilatation of portal vein in a neonate in a case of infracardiac total anomalous pulmonary venous return. The common descending pulmonary vein was seen joining below the diaphragm with the left branch of portal vein, which was terminating as a blind-ending, aneurysmally dilated vascular channel. Keywords portal vein, neonatal, aneurysm, infracardiac, TAPVR Submitted May 29, 2013; Accepted August 14, 2013.
Introduction Aneurysms of portal venous system are rare and defined as focal fusiform or saccular dilatation of the involved vessel.1,2 We present a rare case of neonatal portal vein dilatation due to infracardiac type of total anomalous pulmonary venous return (TAPVR) detected in computed tomographic angiography (CTA).
Case Report A three-week male child was admitted to pediatric emergency with complaints of recurrent cyanotic spells since birth. Echocardiography findings were suggestive of infracardiac type of TAPVR. The CTA was planned to delineate the vascular anatomy in detail prior to surgery. The CTA showed anomalous pulmonary venous drainage with a common descending pulmonary vein joining inferiorly below the diaphragm with the left branch of portal vein. The left branch of portal vein was seen terminating as a blind-ending, aneurysmally dilated vascular channel giving venous branches to left lobe as well as to right lobe (Figure 1). Superiorly, none of the pulmonary veins (either right or left) were connecting to left atrium (which was hypoplastic). In addition, patent ductus arteriosus was also seen. The child was planned for surgery; however, he had a cardiac arrest during admission and could not be resuscitated.
Discussion Total anomalous pulmonary venous return results from persistence of primitive splanchnic connections of the pulmonary
veins to the cardinal systemic veins and thence to the right atrium, due to failure of common pulmonary vein to connect to left atrium during embryological development.3 Supracardiac TAPVR is the most common type among the various categories of TAPVR which include supracardiac, cardiac, infracardiac, and mixed types. The descending vein in the infracardiac variety passes through the esophageal hiatus in the abdomen and connects most commonly with the portal vein at the confluence of splenic and superior mesenteric veins. However, communication with hepatic veins, ductus venosus as well as inferior vena cava can also be seen.3 The indexed case was infracardiac type of TAPVR, in which the descending vein was seen communicating with the blind-ending, aneurysmally dilated left branch of portal vein. Aneurysms of portal venous system represent 3% of all visceral aneurysms and are defined as focal fusiform or saccular dilatation of the involved vessel.1,2 Congenital and acquired causes have been proposed for their development. Increase in portal venous pressure can cause dilatation of the
1 Department of Radiodiagnosis and Imaging, Post Graduate Institute of Medical Education and Research, Chandigarh, India
Corresponding Author: Kushaljit Singh Sodhi, Department of Radiodiagnosis and Imaging, Post Graduate Institute of Medical Education and Research, Sector-12, Chandigarh-160012, India. Email: [email protected]
Downloaded from pch.sagepub.com at Universiti Teknologi MARA (UiTM) on January 29, 2015
132
World Journal for Pediatric and Congenital Heart Surgery 5(1)
Figure 1. Coronal (A and B) and axial (C) reformatted CTA images showing common descending pulmonary vein (arrow in A) joining with left portal vein (black arrow in B) which is seen as blind-ending dilated vascular channel (white arrows in B and C) giving branches to right lobe as well as to left lobe. CTA indicates Computed tomographic angiography.
congenital or developmentally defective segment of portal venous system.4 In the indexed case, whole of the pulmonary venous blood was being drained to the left branch of the portal vein by the common descending pulmonary vein. Postnatal closure of ductus venosus of Arantius and the obligation of the venous pulmonary blood flow to cross the barrier of the intrahepatic sinusoids (‘‘rete mirabilia’’), in the absence of the bypass of the liver, result in the increase in portal venous pressure. This was the likely cause of the dilatation of portal vein in our case. To the best of our knowledge, this unusual cause of aneurysm of portal venous system in a neonate has not been described previously in the literature on CTA. Declaration of Conflicting Interests The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.
Funding The author(s) received no financial support for the research, authorship, and/or publication of this article
References 1. Gallego C, Velasco M, Marcuello P, Tejedor M, Campo LD, Friera A. Congenital and acquired anomalies of the portal venous system. Radiographics. 2002;22(1): 141-159. 2. Koc Z, Oguzkurt L, Ulusan S. Portal venous system aneurysms: imaging, clinical findings, and a possible new etiologic factor. Am J Roentgenol. 2007;189(5): 1023-1030. 3. Vyas HV, Greenberg SB, Krishnamurthy R. MR imaging and CT evaluation of congenital pulmonary vein abnormalities in neonates and infants. Radiographics. 2012;32(1): 87-98. 4. Tolgonay G, Ozbek SS, Oniz H, Suzer E, Yur-dakul LO. Regression of splenic vein aneurysm following resolution of splenomegaly. J Clin Ultrasound. 1998;26(2): 98-102.
Downloaded from pch.sagepub.com at Universiti Teknologi MARA (UiTM) on January 29, 2015
Infracardiac Total Anomalous Pulmonary Venous Return: An Unusual Cause of Neonatal Portal Vein Enlargement
World Journal for Pediatric and Congenital Heart Surgery 2014, Vol 5(1) 131-132 ª The Author(s) 2014 Reprints and permission: sagepub.com/journalsPermissions.nav DOI: 10.1177/2150135113504307 pch.sagepub.com
Anmol Bhatia, MD1, Kushaljit S. Sodhi, MD1, Akshay K. Saxena, MD1, Manphool Singhal, MD1, and Niranjan Khandelwal, MD1
Abstract We present an unusual cause of aneurysmal dilatation of portal vein in a neonate in a case of infracardiac total anomalous pulmonary venous return. The common descending pulmonary vein was seen joining below the diaphragm with the left branch of portal vein, which was terminating as a blind-ending, aneurysmally dilated vascular channel. Keywords portal vein, neonatal, aneurysm, infracardiac, TAPVR Submitted May 29, 2013; Accepted August 14, 2013.
Introduction Aneurysms of portal venous system are rare and defined as focal fusiform or saccular dilatation of the involved vessel.1,2 We present a rare case of neonatal portal vein dilatation due to infracardiac type of total anomalous pulmonary venous return (TAPVR) detected in computed tomographic angiography (CTA).
Case Report A three-week male child was admitted to pediatric emergency with complaints of recurrent cyanotic spells since birth. Echocardiography findings were suggestive of infracardiac type of TAPVR. The CTA was planned to delineate the vascular anatomy in detail prior to surgery. The CTA showed anomalous pulmonary venous drainage with a common descending pulmonary vein joining inferiorly below the diaphragm with the left branch of portal vein. The left branch of portal vein was seen terminating as a blind-ending, aneurysmally dilated vascular channel giving venous branches to left lobe as well as to right lobe (Figure 1). Superiorly, none of the pulmonary veins (either right or left) were connecting to left atrium (which was hypoplastic). In addition, patent ductus arteriosus was also seen. The child was planned for surgery; however, he had a cardiac arrest during admission and could not be resuscitated.
Discussion Total anomalous pulmonary venous return results from persistence of primitive splanchnic connections of the pulmonary
veins to the cardinal systemic veins and thence to the right atrium, due to failure of common pulmonary vein to connect to left atrium during embryological development.3 Supracardiac TAPVR is the most common type among the various categories of TAPVR which include supracardiac, cardiac, infracardiac, and mixed types. The descending vein in the infracardiac variety passes through the esophageal hiatus in the abdomen and connects most commonly with the portal vein at the confluence of splenic and superior mesenteric veins. However, communication with hepatic veins, ductus venosus as well as inferior vena cava can also be seen.3 The indexed case was infracardiac type of TAPVR, in which the descending vein was seen communicating with the blind-ending, aneurysmally dilated left branch of portal vein. Aneurysms of portal venous system represent 3% of all visceral aneurysms and are defined as focal fusiform or saccular dilatation of the involved vessel.1,2 Congenital and acquired causes have been proposed for their development. Increase in portal venous pressure can cause dilatation of the
1 Department of Radiodiagnosis and Imaging, Post Graduate Institute of Medical Education and Research, Chandigarh, India
Corresponding Author: Kushaljit Singh Sodhi, Department of Radiodiagnosis and Imaging, Post Graduate Institute of Medical Education and Research, Sector-12, Chandigarh-160012, India. Email: [email protected]
Downloaded from pch.sagepub.com at Universiti Teknologi MARA (UiTM) on January 29, 2015
132
World Journal for Pediatric and Congenital Heart Surgery 5(1)
Figure 1. Coronal (A and B) and axial (C) reformatted CTA images showing common descending pulmonary vein (arrow in A) joining with left portal vein (black arrow in B) which is seen as blind-ending dilated vascular channel (white arrows in B and C) giving branches to right lobe as well as to left lobe. CTA indicates Computed tomographic angiography.
congenital or developmentally defective segment of portal venous system.4 In the indexed case, whole of the pulmonary venous blood was being drained to the left branch of the portal vein by the common descending pulmonary vein. Postnatal closure of ductus venosus of Arantius and the obligation of the venous pulmonary blood flow to cross the barrier of the intrahepatic sinusoids (‘‘rete mirabilia’’), in the absence of the bypass of the liver, result in the increase in portal venous pressure. This was the likely cause of the dilatation of portal vein in our case. To the best of our knowledge, this unusual cause of aneurysm of portal venous system in a neonate has not been described previously in the literature on CTA. Declaration of Conflicting Interests The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.
Funding The author(s) received no financial support for the research, authorship, and/or publication of this article
References 1. Gallego C, Velasco M, Marcuello P, Tejedor M, Campo LD, Friera A. Congenital and acquired anomalies of the portal venous system. Radiographics. 2002;22(1): 141-159. 2. Koc Z, Oguzkurt L, Ulusan S. Portal venous system aneurysms: imaging, clinical findings, and a possible new etiologic factor. Am J Roentgenol. 2007;189(5): 1023-1030. 3. Vyas HV, Greenberg SB, Krishnamurthy R. MR imaging and CT evaluation of congenital pulmonary vein abnormalities in neonates and infants. Radiographics. 2012;32(1): 87-98. 4. Tolgonay G, Ozbek SS, Oniz H, Suzer E, Yur-dakul LO. Regression of splenic vein aneurysm following resolution of splenomegaly. J Clin Ultrasound. 1998;26(2): 98-102.
Downloaded from pch.sagepub.com at Universiti Teknologi MARA (UiTM) on January 29, 2015
