Abernethy Malformation: Possible Diagnosis for Patients with Congenital Heart Disease and Persistent Cyanosis
ABSTRACT 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 atr...
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| Format: | Article |
| Language: | English |
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Sociedade Brasileira de Cirurgia Cardiovascular
2022-10-01
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| Series: | Brazilian Journal of Cardiovascular Surgery |
| Subjects: | |
| Online Access: | http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0102-76382022005008210&tlng=en |
| _version_ | 1798030497955184640 |
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| author | Marilia Maroneze Brun Mariana Rodero Cardoso Bruna Cury Borim Carlos Henrique De Marchi Ulisses Alexandre Croti |
| author_facet | Marilia Maroneze Brun Mariana Rodero Cardoso Bruna Cury Borim Carlos Henrique De Marchi Ulisses Alexandre Croti |
| author_sort | Marilia Maroneze Brun |
| collection | DOAJ |
| description | ABSTRACT 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 10th 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. |
| first_indexed | 2024-04-11T19:42:18Z |
| format | Article |
| id | doaj.art-f627d6b242c04c24b5cc51f7b6882053 |
| institution | Directory Open Access Journal |
| issn | 1678-9741 |
| language | English |
| last_indexed | 2024-04-11T19:42:18Z |
| publishDate | 2022-10-01 |
| publisher | Sociedade Brasileira de Cirurgia Cardiovascular |
| record_format | Article |
| series | Brazilian Journal of Cardiovascular Surgery |
| spelling | doaj.art-f627d6b242c04c24b5cc51f7b68820532022-12-22T04:06:41ZengSociedade Brasileira de Cirurgia CardiovascularBrazilian Journal of Cardiovascular Surgery1678-97412022-10-0110.21470/1678-9741-2022-0110Abernethy Malformation: Possible Diagnosis for Patients with Congenital Heart Disease and Persistent CyanosisMarilia Maroneze BrunMariana Rodero CardosoBruna Cury BorimCarlos Henrique De MarchiUlisses Alexandre Crotihttps://orcid.org/0000-0002-1127-4782ABSTRACT 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 10th 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.http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0102-76382022005008210&tlng=enCardiomegalyCyanosisCongenital Heart DefectsHepatopulmonary SyndromeComputed Tomography Angiography |
| spellingShingle | Marilia Maroneze Brun Mariana Rodero Cardoso Bruna Cury Borim Carlos Henrique De Marchi Ulisses Alexandre Croti Abernethy Malformation: Possible Diagnosis for Patients with Congenital Heart Disease and Persistent Cyanosis Brazilian Journal of Cardiovascular Surgery Cardiomegaly Cyanosis Congenital Heart Defects Hepatopulmonary Syndrome Computed Tomography Angiography |
| 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 | Cardiomegaly Cyanosis Congenital Heart Defects Hepatopulmonary Syndrome Computed Tomography Angiography |
| url | http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0102-76382022005008210&tlng=en |
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