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Correction of the scimitar syndrome, a rare cardiac venous anomaly, leading to Budd–Chiari syndrome: a case report
INTRODUCTION: Scimitar syndrome is a congenital heart disease characterized by an abnormal drainage of the right lung into the inferior vena cava, the right atrium or a variety of venous connections from the anomalous pulmonary vein to a systemic vein. This left-to-right shunt induces pulmonary hype...
Autores principales: | , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
BioMed Central
2014
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4137726/ https://www.ncbi.nlm.nih.gov/pubmed/25113120 http://dx.doi.org/10.1186/1752-1947-8-273 |
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author | Assoignon, Marie-Pia Christiaens, Paul Laleman, Wim |
author_facet | Assoignon, Marie-Pia Christiaens, Paul Laleman, Wim |
author_sort | Assoignon, Marie-Pia |
collection | PubMed |
description | INTRODUCTION: Scimitar syndrome is a congenital heart disease characterized by an abnormal drainage of the right lung into the inferior vena cava, the right atrium or a variety of venous connections from the anomalous pulmonary vein to a systemic vein. This left-to-right shunt induces pulmonary hypertension and is an indication for surgical repair in cases of a history of recurrent pneumonia or significant left-to-right shunting. A corrective approach, which consists of rerouting the anomalous pulmonary flow to the left atrium, is usually performed. Complications of scimitar repair are stenosis, thrombosis and occlusion of the scimitar vein and its deviation. CASE PRESENTATION: This case report describes a 53-year-old Caucasian woman with known scimitar syndrome, undergoing surgical repair due to invaliding symptoms of dyspnoea, and presenting with postoperative Budd–Chiari syndrome due to anomalous drainage of her right hepatic vein into the left atrium. It is an interesting cause of liver pathology caused by Budd–Chiari that never has been described before. CONCLUSIONS: This case report emphasizes the importance of a thorough preoperative evaluation, and the importance of antecedents in newly presenting pathology. It is an interesting cause of a known hepatic syndrome, the Budd–Chiari syndrome. This case report is of interest to many specialties, including Hepatology, Cardiology, Radiology and Cardiovascular Surgery. It exposes a new interesting anatomic variation of the scimitar syndrome with significant postoperative implications. |
format | Online Article Text |
id | pubmed-4137726 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2014 |
publisher | BioMed Central |
record_format | MEDLINE/PubMed |
spelling | pubmed-41377262014-08-20 Correction of the scimitar syndrome, a rare cardiac venous anomaly, leading to Budd–Chiari syndrome: a case report Assoignon, Marie-Pia Christiaens, Paul Laleman, Wim J Med Case Rep Case Report INTRODUCTION: Scimitar syndrome is a congenital heart disease characterized by an abnormal drainage of the right lung into the inferior vena cava, the right atrium or a variety of venous connections from the anomalous pulmonary vein to a systemic vein. This left-to-right shunt induces pulmonary hypertension and is an indication for surgical repair in cases of a history of recurrent pneumonia or significant left-to-right shunting. A corrective approach, which consists of rerouting the anomalous pulmonary flow to the left atrium, is usually performed. Complications of scimitar repair are stenosis, thrombosis and occlusion of the scimitar vein and its deviation. CASE PRESENTATION: This case report describes a 53-year-old Caucasian woman with known scimitar syndrome, undergoing surgical repair due to invaliding symptoms of dyspnoea, and presenting with postoperative Budd–Chiari syndrome due to anomalous drainage of her right hepatic vein into the left atrium. It is an interesting cause of liver pathology caused by Budd–Chiari that never has been described before. CONCLUSIONS: This case report emphasizes the importance of a thorough preoperative evaluation, and the importance of antecedents in newly presenting pathology. It is an interesting cause of a known hepatic syndrome, the Budd–Chiari syndrome. This case report is of interest to many specialties, including Hepatology, Cardiology, Radiology and Cardiovascular Surgery. It exposes a new interesting anatomic variation of the scimitar syndrome with significant postoperative implications. BioMed Central 2014-08-12 /pmc/articles/PMC4137726/ /pubmed/25113120 http://dx.doi.org/10.1186/1752-1947-8-273 Text en Copyright © 2014 Assoignon et al.; licensee BioMed Central Ltd. http://creativecommons.org/licenses/by/4.0 This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly credited. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated. |
spellingShingle | Case Report Assoignon, Marie-Pia Christiaens, Paul Laleman, Wim Correction of the scimitar syndrome, a rare cardiac venous anomaly, leading to Budd–Chiari syndrome: a case report |
title | Correction of the scimitar syndrome, a rare cardiac venous anomaly, leading to Budd–Chiari syndrome: a case report |
title_full | Correction of the scimitar syndrome, a rare cardiac venous anomaly, leading to Budd–Chiari syndrome: a case report |
title_fullStr | Correction of the scimitar syndrome, a rare cardiac venous anomaly, leading to Budd–Chiari syndrome: a case report |
title_full_unstemmed | Correction of the scimitar syndrome, a rare cardiac venous anomaly, leading to Budd–Chiari syndrome: a case report |
title_short | Correction of the scimitar syndrome, a rare cardiac venous anomaly, leading to Budd–Chiari syndrome: a case report |
title_sort | correction of the scimitar syndrome, a rare cardiac venous anomaly, leading to budd–chiari syndrome: a case report |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4137726/ https://www.ncbi.nlm.nih.gov/pubmed/25113120 http://dx.doi.org/10.1186/1752-1947-8-273 |
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