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Abernethy Malformation: Possible Diagnosis for Patients with Congenital Heart Disease and Persistent Cyanosis
CLINICAL DATA: Infant, nine months of age, female, diagnosed with congenital heart disease, with signs of heart failure associated with cyanosis and difficulty in gaining weight. CHEST RADIOGRAPHY: Cardiomegaly with prevalence of pulmonary vascular network. ELECTROCARDIOGRAM: Ectopic atrial rhythm w...
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Formato: | Online Artículo Texto |
Lenguaje: | English |
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Sociedade Brasileira de Cirurgia Cardiovascular
2023
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Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10069258/ https://www.ncbi.nlm.nih.gov/pubmed/36259992 http://dx.doi.org/10.21470/1678-9741-2022-0110 |
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author | Brun, Marilia Maroneze Cardoso, Mariana Rodero Borim, Bruna Cury Marchi, Carlos Henrique De Croti, Ulisses Alexandre |
author_facet | Brun, Marilia Maroneze Cardoso, Mariana Rodero Borim, Bruna Cury Marchi, Carlos Henrique De Croti, Ulisses Alexandre |
author_sort | Brun, Marilia Maroneze |
collection | PubMed |
description | CLINICAL DATA: Infant, nine months of age, female, diagnosed with congenital heart disease, with signs of heart failure associated with cyanosis and difficulty in gaining weight. CHEST RADIOGRAPHY: Cardiomegaly with prevalence of pulmonary vascular network. ELECTROCARDIOGRAM: Ectopic atrial rhythm with right ventricular overload and left anterosuperior divisional block. ECHOCARDIOGRAM: Single atrium with absent interatrial septum, atrioventricular connection with a single valve and two orifices, with increased pulmonary pressure and high Qp/Qs. COMPUTED TOMOGRAPHY: Absence of portal vein and intrahepatic segment of the inferior vena cava. Infrahepatic portion continuing with the azygos system at the level of the thoracic cavity, presence of mesenteric-caval communication associated with signs suggestive of hepatic peribiliary fibrosis. DIAGNOSIS: Abernethy malformation is a rare condition and represents an extrahepatic portosystemic shunt that develops between the mesenteric-portal vasculature and the systemic veins. It may be associated with cardiac malformations and advance with pulmonary hypertension and even the need for liver transplantation. Persistent cyanosis after corrective surgery led to a deeper investigation and correct diagnosis of this malformation. OPERATION: Sternotomy with 68 minutes of cardiopulmonary bypass and nine minutes of total circulatory arrest. In the postoperative period, persistence of cyanosis was evident, even though there were no immediate complications. Patient was discharged on the 10(th) postoperative day. An abdominal computed tomography angiography confirmed the diagnosis of Abernethy type I malformation, and the patient was transferred for liver transplantation after congenital heart disease treatment. |
format | Online Article Text |
id | pubmed-10069258 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2023 |
publisher | Sociedade Brasileira de Cirurgia Cardiovascular |
record_format | MEDLINE/PubMed |
spelling | pubmed-100692582023-04-04 Abernethy Malformation: Possible Diagnosis for Patients with Congenital Heart Disease and Persistent Cyanosis Brun, Marilia Maroneze Cardoso, Mariana Rodero Borim, Bruna Cury Marchi, Carlos Henrique De Croti, Ulisses Alexandre Braz J Cardiovasc Surg Clinical-Surgical Correlation CLINICAL DATA: Infant, nine months of age, female, diagnosed with congenital heart disease, with signs of heart failure associated with cyanosis and difficulty in gaining weight. CHEST RADIOGRAPHY: Cardiomegaly with prevalence of pulmonary vascular network. ELECTROCARDIOGRAM: Ectopic atrial rhythm with right ventricular overload and left anterosuperior divisional block. ECHOCARDIOGRAM: Single atrium with absent interatrial septum, atrioventricular connection with a single valve and two orifices, with increased pulmonary pressure and high Qp/Qs. COMPUTED TOMOGRAPHY: Absence of portal vein and intrahepatic segment of the inferior vena cava. Infrahepatic portion continuing with the azygos system at the level of the thoracic cavity, presence of mesenteric-caval communication associated with signs suggestive of hepatic peribiliary fibrosis. DIAGNOSIS: Abernethy malformation is a rare condition and represents an extrahepatic portosystemic shunt that develops between the mesenteric-portal vasculature and the systemic veins. It may be associated with cardiac malformations and advance with pulmonary hypertension and even the need for liver transplantation. Persistent cyanosis after corrective surgery led to a deeper investigation and correct diagnosis of this malformation. OPERATION: Sternotomy with 68 minutes of cardiopulmonary bypass and nine minutes of total circulatory arrest. In the postoperative period, persistence of cyanosis was evident, even though there were no immediate complications. Patient was discharged on the 10(th) postoperative day. An abdominal computed tomography angiography confirmed the diagnosis of Abernethy type I malformation, and the patient was transferred for liver transplantation after congenital heart disease treatment. Sociedade Brasileira de Cirurgia Cardiovascular 2023 /pmc/articles/PMC10069258/ /pubmed/36259992 http://dx.doi.org/10.21470/1678-9741-2022-0110 Text en https://creativecommons.org/licenses/by/4.0/This is an Open Access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. |
spellingShingle | Clinical-Surgical Correlation Brun, Marilia Maroneze Cardoso, Mariana Rodero Borim, Bruna Cury Marchi, Carlos Henrique De Croti, Ulisses Alexandre Abernethy Malformation: Possible Diagnosis for Patients with Congenital Heart Disease and Persistent Cyanosis |
title | Abernethy Malformation: Possible Diagnosis for Patients with
Congenital Heart Disease and Persistent Cyanosis |
title_full | Abernethy Malformation: Possible Diagnosis for Patients with
Congenital Heart Disease and Persistent Cyanosis |
title_fullStr | Abernethy Malformation: Possible Diagnosis for Patients with
Congenital Heart Disease and Persistent Cyanosis |
title_full_unstemmed | Abernethy Malformation: Possible Diagnosis for Patients with
Congenital Heart Disease and Persistent Cyanosis |
title_short | Abernethy Malformation: Possible Diagnosis for Patients with
Congenital Heart Disease and Persistent Cyanosis |
title_sort | abernethy malformation: possible diagnosis for patients with
congenital heart disease and persistent cyanosis |
topic | Clinical-Surgical Correlation |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10069258/ https://www.ncbi.nlm.nih.gov/pubmed/36259992 http://dx.doi.org/10.21470/1678-9741-2022-0110 |
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