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Anomalia congênita da veia hepática
ORPHA:95507CID-10 · Q26.8CID-11 · LB20.0YDOENÇA RARA

O coração é um órgão muscular presente nos humanos e em outros animais que bombeia o sangue através dos vasos sanguíneos do sistema circulatório. O sangue fornece ao corpo oxigénio e nutrientes e ajuda a eliminar resíduos metabólicos. Nos humanos, o coração situa-se na cavidade torácica entre os pulmões, num espaço denominado mediastino.

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Introdução

O que você precisa saber de cara

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Anomalia congênita da veia hepática é uma malformação rara das veias que drenam o fígado. Pode variar de ausência parcial a completa, impactando o fluxo sanguíneo hepático e a circulação sistêmica.

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SUS: Cobertura mínimaScore: 35%
Centros em: PA, PE, BA, CE, PB +10CID-10: Q26.8
🇧🇷Dados SUS / DATASUS
PROCEDIMENTOS SIGTAP (5)
0202010503
Cariótipo — bandas G, Q ou Rgenetic_test
0202010600
Pesquisa de microdeleções/microduplicações por FISHlab_test
0202010694
Sequenciamento completo do exoma (WES)rehabilitation
0202010260
Dosagem de alfa-fetoproteína
0301070040
Atendimento em reabilitação — doenças raras
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Sinais e sintomas

O que aparece no corpo e com que frequência cada sintoma acontece

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Anos de pesquisa5
Últimos 10 anos200publicações
Pico202554 papers
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2021Hoje · 2026🧪 2008Primeiro ensaio clínico📈 2025Ano de pico
Publicações por ano (últimos 10 anos)

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Genética e causas

O que está alterado no DNA e como passa nas famílias

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Diagnóstico

Os sinais que médicos procuram e os exames que confirmam

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Tratamento e manejo

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Onde tratar no SUS

Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)

🇧🇷 Atendimento SUS — Anomalia congênita da veia hepática

Centros de Referência SUS

24 centros habilitados pelo SUS para Anomalia congênita da veia hepática

Centros para Anomalia congênita da veia hepática

Detalhes dos centros

Hospital Universitário Prof. Edgard Santos (HUPES)

R. Dr. Augusto Viana, s/n - Canela, Salvador - BA, 40110-060 · CNES 0003808

Serviço de Referência

Rota
Anomalias CongênitasErros Inatos do Metabolismo

Hospital Infantil Albert Sabin

R. Tertuliano Sales, 544 - Vila União, Fortaleza - CE, 60410-794 · CNES 2407876

Serviço de Referência

Rota
Anomalias CongênitasDeficiência Intelectual

Hospital de Apoio de Brasília (HAB)

AENW 3 Lote A Setor Noroeste - Plano Piloto, Brasília - DF, 70684-831 · CNES 0010456

Serviço de Referência

Rota
Anomalias CongênitasErros Inatos do MetabolismoDeficiência Intelectual

Hospital Estadual Infantil e Maternidade Alzir Bernardino Alves (HIABA)

Av. Min. Salgado Filho, 918 - Soteco, Vila Velha - ES, 29106-010 · CNES 6631207

Serviço de Referência

Rota
Anomalias CongênitasErros Inatos do MetabolismoDeficiência Intelectual

Hospital das Clínicas da UFG

Rua 235 QD. 68 Lote Área, Nº 285, s/nº - Setor Leste Universitário, Goiânia - GO, 74605-050 · CNES 2338424

Serviço de Referência

Rota
Anomalias CongênitasErros Inatos do Metabolismo

Hospital Universitário da UFJF

R. Catulo Breviglieri, Bairro - s/n - Santa Catarina, Juiz de Fora - MG, 36036-110 · CNES 2297442

Atenção Especializada

Rota
Anomalias Congênitas

Hospital das Clínicas da UFMG

Av. Prof. Alfredo Balena, 110 - Santa Efigênia, Belo Horizonte - MG, 30130-100 · CNES 2280167

Serviço de Referência

Rota
Anomalias CongênitasErros Inatos do MetabolismoDeficiência Intelectual

Hospital Universitário Julio Müller (HUJM)

R. Luis Philippe Pereira Leite, s/n - Alvorada, Cuiabá - MT, 78048-902 · CNES 2726092

Atenção Especializada

Rota
Anomalias Congênitas

Hospital Universitário João de Barros Barreto

R. dos Mundurucus, 4487 - Guamá, Belém - PA, 66073-000 · CNES 2337878

Serviço de Referência

Rota
Anomalias CongênitasErros Inatos do MetabolismoDeficiência Intelectual

Hospital Universitário Lauro Wanderley (HULW)

R. Tabeliao Estanislau Eloy, 585 - Castelo Branco, João Pessoa - PB, 58050-585 · CNES 0002470

Atenção Especializada

Rota
Anomalias Congênitas

Instituto de Medicina Integral Prof. Fernando Figueira (IMIP)

R. dos Coelhos, 300 - Boa Vista, Recife - PE, 50070-902 · CNES 0000647

Serviço de Referência

Rota
Anomalias CongênitasErros Inatos do MetabolismoDeficiência Intelectual

Hospital Pequeno Príncipe

R. Des. Motta, 1070 - Água Verde, Curitiba - PR, 80250-060 · CNES 3143805

Serviço de Referência

Rota
Anomalias CongênitasDeficiência Intelectual

Hospital Universitário Regional de Maringá (HUM)

Av. Mandacaru, 1590 - Parque das Laranjeiras, Maringá - PR, 87083-240 · CNES 2216108

Atenção Especializada

Rota
Anomalias Congênitas

Hospital de Clínicas da UFPR

R. Gen. Carneiro, 181 - Alto da Glória, Curitiba - PR, 80060-900 · CNES 2364980

Serviço de Referência

Rota
Anomalias CongênitasErros Inatos do MetabolismoDeficiência Intelectual

Hospital Universitário Pedro Ernesto (HUPE-UERJ)

Blvd. 28 de Setembro, 77 - Vila Isabel, Rio de Janeiro - RJ, 20551-030 · CNES 2280221

Serviço de Referência

Rota
Anomalias CongênitasErros Inatos do Metabolismo

Instituto Nacional de Saúde da Mulher, da Criança e do Adolescente Fernandes Figueira (IFF/Fiocruz)

Av. Rui Barbosa, 716 - Flamengo, Rio de Janeiro - RJ, 22250-020 · CNES 2269988

Serviço de Referência

Rota
Anomalias CongênitasErros Inatos do MetabolismoDeficiência Intelectual

Hospital São Lucas da PUCRS

Av. Ipiranga, 6690 - Jardim Botânico, Porto Alegre - RS, 90610-000 · CNES 2232928

Serviço de Referência

Rota
Anomalias CongênitasErros Inatos do Metabolismo

Hospital de Clínicas de Porto Alegre (HCPA)

Rua Ramiro Barcelos, 2350 Bloco A - Av. Protásio Alves, 211 - Bloco B e C - Santa Cecília, Porto Alegre - RS, 90035-903 · CNES 2237601

Serviço de Referência

Rota
Anomalias CongênitasErros Inatos do MetabolismoDeficiência Intelectual

Hospital Universitário da UFSC (HU-UFSC)

R. Profa. Maria Flora Pausewang - Trindade, Florianópolis - SC, 88036-800 · CNES 2560356

Serviço de Referência

Rota
Anomalias CongênitasErros Inatos do Metabolismo

Hospital das Clínicas da FMUSP

R. Dr. Ovídio Pires de Campos, 225 - Cerqueira César, São Paulo - SP, 05403-010 · CNES 2077485

Serviço de Referência

Rota
Anomalias CongênitasErros Inatos do MetabolismoDeficiência Intelectual

Hospital de Base de São José do Rio Preto

Av. Brg. Faria Lima, 5544 - Vila Sao Jose, São José do Rio Preto - SP, 15090-000 · CNES 2079798

Atenção Especializada

Rota
Anomalias Congênitas

Hospital de Clínicas da UNICAMP

R. Vital Brasil, 251 - Cidade Universitária, Campinas - SP, 13083-888 · CNES 2748223

Serviço de Referência

Rota
Anomalias CongênitasErros Inatos do MetabolismoDeficiência Intelectual

Hospital de Clínicas de Ribeirão Preto (HCRP-USP)

R. Ten. Catão Roxo, 3900 - Vila Monte Alegre, Ribeirão Preto - SP, 14015-010 · CNES 2082187

Serviço de Referência

Rota
Anomalias CongênitasErros Inatos do MetabolismoDeficiência Intelectual

UNIFESP / Hospital São Paulo

R. Napoleão de Barros, 715 - Vila Clementino, São Paulo - SP, 04024-002 · CNES 2688689

Serviço de Referência

Rota
Anomalias CongênitasErros Inatos do Metabolismo
Sobre os centros SUS: Estes centros são habilitados pelo Ministério da Saúde como Serviços de Referência em Doenças Raras ou Serviços de Atenção Especializada. O atendimento é pelo SUS, com encaminhamento da rede de atenção básica.

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Publicações mais relevantes

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0 papers (10 anos)
#1

Noncirrhotic Portopulmonary Hypertension Due to Hepatoportal Sclerosis in Adams-Oliver Syndrome.

Pediatrics2026 Jan 01

A 13-year-old girl presented for evaluation of pulmonary hypertension after symptoms of dyspnea and exercise intolerance. Full evaluation was negative except for abdominal ultrasonography with splenomegaly and esophageal varices suggestive of portal hypertension. Cardiac and hepatic vein catheterization confirmed portopulmonary hypertension. Liver biopsy demonstrated sinusoidal dilatation, nodularity, and minimal fibrosis, which was interpreted as possible nodular regenerative hyperplasia but not cirrhosis. Radiographic imaging, including computed tomography venography, demonstrated an elongated and severely stenotic extrahepatic portal vein, and portal hypertension was presumed to be secondary to congenital portal vein hypoplasia. She was treated with ambrisentan with initial improvement in symptoms and estimated pulmonary pressure. Whole-exome sequencing revealed a likely pathogenic missense mutation in Delta-like canonical Notch ligand 4 associated with Adams-Oliver syndrome. After 2 years, pulmonary hypertension and right heart failure symptoms worsened, along with liver failure. She then had fulminant liver failure and cardiorespiratory arrest. Resuscitative efforts included extracorporeal membranous oxygenation (ECMO), but because of hypoxic brain injury, care was compassionately withdrawn. Autopsy limited to the thorax and abdomen revealed high-grade pulmonary plexiform arteriopathy, splenomegaly, esophageal varices, and large splenorenal shunt. The liver was small with a nodular surface but not fibrotic. The entire length of the extrahepatic portal vein was severely stenotic, and intrahepatic portal veins were missing or diminutive-findings diagnostic of hepatoportal sclerosis. Noncirrhotic portopulmonary hypertension is rare and should include evaluation of immunologic, infectious, toxic, thrombotic, and genetic etiologies. Unfortunately, there is no known treatment of hepatoportal sclerosis.

#2

Terlipressin Therapy for Portal Hyperperfusion Secondary to Portal Vein Size Discrepancy After Pediatric Liver Transplant.

Pediatric transplantation2026 Mar

Congenital portosystemic shunts (CPSS) are rare vascular malformations in which portal venous blood bypasses the liver and drains directly into systemic circulation. Type I Abernethy malformations, characterized by absent intrahepatic portal veins, rarely close spontaneously and often require liver transplantation. Small-for-size syndrome (SFSS), typically described in adult living donor liver transplantation, is increasingly recognized in pediatric recipients, particularly with graft-to-recipient weight ratio (GRWR) < 1.5%. We report the case of a 5-year-old female with Type I Abernethy malformation who underwent deceased donor whole graft liver transplantation. Intraoperative findings demonstrated marked size discrepancy between the donor portal vein and the patient's large portosystemic shunt. Postoperatively, she developed acute liver dysfunction concerning for relative hyperperfusion and SFSS physiology. Despite a GRWR of 1.78%, the patient exhibited rapid elevations in transaminases, INR, and bilirubin postoperatively, raising concern for SFSS physiology. Octreotide therapy resulted in partial biochemical improvement. The subsequent initiation of terlipressin resulted in a marked and sustained decline in liver enzymes and normalization of coagulation parameters. At 18 months post-transplant, the patient remains clinically well without hepatic dysfunction. This is the first reported pediatric case of successful use of terlipressin, bridged by octreotide, for management of suspected SFSS physiology following liver transplantation. Terlipressin may represent a safe and effective therapeutic option to modulate portal pressures in pediatric patients with graft hyperperfusion.

#3

Sinus venosus atrial septal defect with partial anomalous pulmonary and hepatic venous drainage: a case report.

Cardiology in the young2026 Feb 24

Sinus venosus atrial septal defect is an uncommon congenital cardiac anomaly frequently associated with partial anomalous pulmonary venous drainage. The coexistence of this defect with anomalous hepatic venous drainage, cor triatriatum, and two arterial ductal connections is extremely rare and may create diagnostic and surgical challenges. An eleven-month-old female infant with tachypnoea and growth retardation was found to have sinus venosus atrial septal defect with partial anomalous pulmonary venous drainage and anomalous hepatic venous drainage to the coronary sinus on CT angiography. Cardiac catheterisation demonstrated a moderate left-to-right shunt and mildly elevated pulmonary artery pressure. Intraoperative assessment unexpectedly revealed two arterial ductal connections and cor triatriatum. Surgical management consisted of closure of the atrial septal defect, rerouting of pulmonary veins, and ligation of both arterial ductal connections. Hepatic venous drainage was left unaltered due to low flow and lack of hemodynamic effect. Postoperative recovery was uneventful. This rare constellation of anomalies highlights the importance of advanced imaging, careful preoperative planning, and intraoperative vigilance for unanticipated findings in CHD.

#4

Incidence of portosystemic shunts in Finnish miniature schnauzer litters.

Acta veterinaria Scandinavica2026 Feb 22

Congenital portosystemic shunts (CPSSs) are vascular anomalies that permit communication between the portal and systemic circulation, thus allowing venous blood to bypass the liver. Previous studies have reported an estimated CPSS prevalence of 0.18-0.76% in the general dog population, being more commonly diagnosed in purebred breeds such as cairn terriers, Maltese, dachshunds, Yorkshire terriers, Irish wolfhounds, golden retrievers and Labrador retrievers. In addition, miniature schnauzers are known to be predisposed to CPSSs, but the breed-specific incidence has not been systematically investigated. This study was undertaken to evaluate the incidence of CPSSs in Finnish 6-12-week-old miniature schnauzer puppies within a one-year study period. The puppies were screened for the presence of a CPSS by measuring the postprandial serum bile acid (SBA) concentration. When the SBA concentration was above the reference limit, further investigations were performed (i.e., pre- and postprandial bile acid stimulation test, computer tomography (CT) or post-mortem examination). Based on the results, the incidence of CPSSs was calculated. The study included 582 dogs from 121 litters, representing 58% (582/1004) of the miniature schnauzers born and registered in Finland during the 1-year study period. In screening, an elevated postprandial serum SBA concentration was presented in 3.3% (19/582) of the dogs. In retesting with the SBA stimulation test 1-4 weeks later, 11 of these 19 dogs had pre- and postprandial bile acid concentrations within the reference range and were excluded from further investigations. One dog with elevated SBA concentrations was diagnosed with portal vein hypoplasia and seven dogs with a CPSS. In addition, one dog had a normal postprandial SBA concentration at screening but was later diagnosed with a CPSS as an incidental finding by CT. In summary, a CPSS was diagnosed in 8/582 dogs and the incidence was 1.37%. All diagnosed CPSS vessels originated either from the splenic vein (n = 5) or the left gastric vein (n = 3) and inserted into the caudal vena cava at the level of the diaphragm. The incidence of the congenital portosystemic shunts in Finnish miniature schnauzers was elevated when compared to the estimated prevalence in the general dog population. The morphology of the portosystemic shunts could indicate a common genetic background.

#5

Left-Sided Portal Hypertension Induced by Splenic Arteriovenous Malformation: A Case Report.

The American journal of case reports2026 Feb 16

BACKGROUND Left-sided portal hypertension (LSPH) is a rare condition that occurs when the splenic vein pressure elevated, which can lead to gastric varices, without abnormalities of hepatic function or esophageal varices. The main causes of LSPH are splenic vein compression, stenosis, and thrombosis. However, no cases of LSPH resulting from vascular malformation have been reported. In this case report, we describe our experience of a patient with LSPH caused by a splenic vein malformation. CASE REPORT The patient was a 53-year-old man who reported having melena for 2 weeks. Gastroscopy performed at another hospital revealed gastric varices, and abdominal contrast-enhanced computed tomography (CT) showed gastric fundal varices and splenic vascular malformation. The patient denied any history of pancreatitis, abdominal trauma, or related surgeries. After further examination, comprehensive imaging, including contrast-enhanced CT and magnetic resonance imaging (MRI), revealed tortuous vascular cluster between the splenic artery and vein, marked dilatation of the left gastric vein, and numerous collateral vascular branches surrounding the perigastric area, with no signs of cirrhosis and gastro-renal shunt or splenic-renal shunt. Therefore, we suspected he had LSPH caused by splenic arteriovenous malformation. To improve the melena, after analyzing the advantages and disadvantages of the surgical approach, we abandoned the embolization approach and opted for splenectomy and Hassab's operation. After Hassab's operation, LSPH caused by arteriovenous malformation was diagnosed by pathology and immunohistochemistry. CONCLUSIONS We report our experience with a rare case of LSPH caused by arteriovenous malformation. We suggest the importance of early differentiation of vascular malformations and non-invasive vascular testing in patients suspected of having LSPH.

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Terlipressin Therapy for Portal Hyperperfusion Secondary to Portal Vein Size Discrepancy After Pediatric Liver Transplant.

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Sinus venosus atrial septal defect with partial anomalous pulmonary and hepatic venous drainage: a case report.

Cardiology in the young
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Incidence of portosystemic shunts in Finnish miniature schnauzer litters.

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Left-Sided Portal Hypertension Induced by Splenic Arteriovenous Malformation: A Case Report.

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A One-Dimensional (1D) Computational Fluid Dynamics Study of Fontan-Associated Liver Disease (FALD).

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Hepatic venous malformations versus "hemangiomas": a clinical, radiologic, and pathologic analysis.

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Ultrasonographic characteristics and outcome of fetal intrahepatic umbilical-porto-systemic venous shunts: a single-center study.

The journal of maternal-fetal &amp; neonatal medicine : the official journal of the European Association of Perinatal Medicine, the Federation of Asia and Oceania Perinatal Societies, the International Society of Perinatal Obstetricians
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Noncirrhotic Portopulmonary Hypertension Due to Hepatoportal Sclerosis in Adams-Oliver Syndrome.

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Fatal Hemoperitoneum Due to Rupture of a Hepatic Vascular Malformation With Focal Absence of Hepatic Parenchyma: A Case Report.

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Chronic abdominal pain: Budd-Chiari syndrome with converging pathologies.

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Idiopathic inferior mesenteric arteriovenous fistula with bleeding diarrhea: A case report.

Medicine
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Congenital hepatic arteriovenous fistula: a case report and review of the literature.

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[Mitral Valvuloplasty in a Patient with Left Inferior Vena Cava with Hemiazygos Continuation:Report of a Case].

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Laparoscopic Anatomical Left Hepatectomy Guided by the Dilated Bile Duct and Middle Hepatic Vein for Hepaticolithiasis in a Patient With Situs Inversus Totalis: How I Do It.

Asian journal of endoscopic surgery
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Delayed Diagnosis of Cor Triatriatum Dexter: A Case Report and Comprehensive Review of Embryology, Imaging, and Management.

Cureus
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Congenital extrahepatic portosystemic shunts (Abernethy malformation): A report of two cases.

The Journal of international medical research
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Persistent left superior vena cava discovered during central line insertion in a patient with Joubert syndrome: a case report.

Journal of medical case reports
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Clinical practice variability among paediatric interventional cardiologists assessing pulmonary arteriovenous malformations.

Cardiology in the young
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Type II Abernethy malformation presenting with heart failure: A case report.

Radiology case reports
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Reno-Portal Venous Drainage for a Cadaveric Renal Transplant in the Setting of Complete Absence of the Infrahepatic Inferior Vena Cava.

Pediatric transplantation
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Point-of-Care Ultrasound Evaluation of Biliary Atresia in the Pediatric Emergency Department.

Pediatric emergency care
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Portohepatic fusion mimics biliary aplasia.

BMJ case reports
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Elements of Complexity in the Surgical Anatomy of Laparoscopic Median Arcuate Ligament Release.

The American surgeon
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Preduodenal portal vein: a case series of variable clinical presentations and surgical implications.

BMC pediatrics
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The importance of evaluating anatomy below the diaphragm in staged single-ventricle patients presenting with cyanosis.

Cardiology in the young
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Minimally Invasive Aortic Valve Replacement in a Patient With Interrupted Hepatic Inferior Vena Cava and Duplicated Inferior Vena Cava: A Case Report.

Cureus
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Cross-sectional imaging of congenital and acquired hepatic vascular abnormalities.

European journal of radiology
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First-in-man transcatheter Fontan completion in post Kawashima patient after successful recruiting of separately draining hepatic veins - A case report.

Cardiology in the young
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Short- and long-term outcomes of 14 dogs with single congenital extrahepatic portosystemic shunts attenuated with a percutaneously controlled hydraulic occluder.

New Zealand veterinary journal
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Osler-Weber-Rendu Syndrome: A Case Report and Brief Literature Review.

South Dakota medicine : the journal of the South Dakota State Medical Association
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[Azygos continuation of the inferior vena cava associated with "H syndrome"].

La Revue de medecine interne
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Kawashima procedure in single ventricle palliation: the factors affecting clinical outcomes.

Journal of cardiothoracic surgery
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Lethal Neonatal Pulmonary Hypertension in Trisomy 21 (T21) Likely Due to Congenital Portosystemic Shunts.

Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society
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Challenges in Minimally Invasive Choledochal Cyst Surgery.

Journal of Indian Association of Pediatric Surgeons
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Clinical efficacy of TACE using coil or gelatin sponge combined with targeted therapy in the treatment of giant hepatocellular carcinoma with arterioportal fistulas.

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Prenatal Ultrasound Diagnosis of Fetal Type IV Intrahepatic Portosystemic Shunt: A Rare Case Report.

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Transjugular Intrahepatic Portosystemic Shunt Creation in Isolated Persistent Left Superior Vena Cava and Portal Vein Thrombosis.

Interventional radiology (Higashimatsuyama-shi (Japan)
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Hepatic arterioportal fistula after biopsy conditioning portal hypertension: first case in primary biliary cholangitis and a systematic literature review.

Annals of hepatology
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Congenital Intrahepatic Disease.

The Veterinary clinics of North America. Small animal practice
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Prevalence of portal vein thrombosis in pediatric patients with cirrhosis and intrahepatic non-cirrhotic portal hypertension.

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Alterations in portal vein confluence during gastric cancer surgery: two case reports.

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Evolving hemodynamics in Fontan circulation: age-driven insights in patients with interrupted inferior vena cava and hepatoazygos shunt.

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Duplication of the inferior vena cava: Case report of an incidental radiological finding.

Radiology case reports
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Monosegmental Reduction as Strategy to Simplify Venous Reconstruction During Pediatric Liver Transplantation.

Pediatric transplantation
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Intrahepatic Congenital Portosystemic Shunts.

The Veterinary clinics of North America. Small animal practice
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An Anomalous Connection of the Left Hepatic Vein with the Coronary Sinus in a Selkirk Rex Cat with a Portosystemic Shunt.

Veterinary radiology &amp; ultrasound : the official journal of the American College of Veterinary Radiology and the International Veterinary Radiology Association
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Incidence, gross features, and histologic characterization of feline fibrocystic hepatobiliary lesions and extrahepatic biliary malformations.

Veterinary pathology
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Anaesthetist Preparedness for Transhepatic Approach to Ostium Secundum Atrial Septal Defect Device Closure: A Case Report.

Annals of cardiac anaesthesia
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Perinatal outcomes in cases of umbilical-portal-systemic venous shunts: experience of a tertiary center.

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Decompensated liver failure due to portal hypertension as a result of hepatic arteriovenous malformations.

BMJ case reports
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Frontiers in cardiovascular medicine
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IVCA in a Boy with Multilocular Renal Cyst as a Risk Factor for Deep Vein Thrombosis.

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Normative Canine Liver Ultrasonography: Papillary Process and Ligamentum Venosum Fissure.

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Clinical outcomes of Kawashima procedure and subsequent hepatic vein incorporation†.

European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery
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Portal vein thrombosis complicating neonatal umbilical vein catheterization in a 3-month-old infant with coincidental extrahepatic biliary atresia: A case report.

Medicine
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Intrahepatic portosystemic shunt: salvage mechanism for oligohydramnios complicating fetal growth restriction.

Ultrasound in obstetrics &amp; gynecology : the official journal of the International Society of Ultrasound in Obstetrics and Gynecology
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The Impact of Inferior Vena Cava Anomalies on Deep Vein Thrombosis: A Case Report.

Cureus
2025

Case Report: Type Ib Abernethy malformation.

Arab journal of gastroenterology : the official publication of the Pan-Arab Association of Gastroenterology
2024

Life-Threatening Esophageal Variceal Hemorrhage in a 7-Year-Old Boy with Massive Portal Vein Enlargement Due to Congenital Arterioportal Fistula.

The American journal of case reports
2024

Morphometry, variations, and histogenesis of umbilical vein, portal sinus, and ductus venosus in human fetal liver: an anatomical study.

Surgical and radiologic anatomy : SRA
2024

Analysis of associated malformations by computed tomography in adults with polysplenia syndrome: A pilot study.

PloS one
2024

Glenn circulation causes early and progressive shunting in a surgical model of pulmonary arteriovenous malformations.

Physiological reports
2024

Modified Kasai operation combined with autologous bone marrow mononuclear cell infusion for biliary atresia.

BMC surgery
2024

A Rare Case of Childhood Malformations, Abernethy Syndrome: Literature Review and Presentation of Two Cases.

Experimental and clinical transplantation : official journal of the Middle East Society for Organ Transplantation
2024

Pseudo hepatic vein thrombosis in a newborn with infracardiac total anomalous pulmonary venous connection.

Pediatric radiology
2024

[Study of related factors to vascular complications after pediatric liver transplantation].

Zhonghua wai ke za zhi [Chinese journal of surgery]
2024

Living Donor Liver Transplantation for Congenital Portosystemic Shunt Presenting With Hyperinsulinemic Hypoglycemia.

Pediatric transplantation
2024

Enhanced lymphangiogenesis in the left lateral segment of a biopsied liver during portoenterostomy for biliary atresia.

Pediatric surgery international
2025

Left-Sided Inferior Vena Cava: A Retrospective Study and Literature Review.

Annals of vascular surgery
2024

Hepatic and splenic venous access tract closure using the VASCADE vascular closure system following percutaneous intervention in patients with CHD.

Cardiology in the young
2024

Laparoscopic right anterior inferior segmentectomy for hepatocellular carcinoma in a patient with congenital absence of the portal vein: intrahepatic artery-guided simulation.

The British journal of surgery
2024

Transjugular intrahepatic portosystemic shunt in patients with splanchnic vein thrombosis: Prevalence and management of patent foramen ovale.

Liver international : official journal of the International Association for the Study of the Liver
2024

Liver and pancreas transplantation in adult donor and recipients with situs inversus totalis: a case series and review of the literature.

Journal of medical case reports
2024

Use of cardiac gated computed tomography in the diagnosis of a rare congenital cardiovascular anomaly in an adult alpaca.

Veterinary radiology &amp; ultrasound : the official journal of the American College of Veterinary Radiology and the International Veterinary Radiology Association
2024

Portal vein hypoplasia is present in patients with biliary atresia at the time of diagnosis.

Journal of pediatric gastroenterology and nutrition
2024

Anatomical classification of canine congenital extrahepatic portosystemic shunts based on CT angiography: A SVSTS and VIRIES multi-institutional study in 1082 dogs.

Veterinary radiology &amp; ultrasound : the official journal of the American College of Veterinary Radiology and the International Veterinary Radiology Association
2024

Quantifying and visualizing abdominal hemodynamics in patients with Fontan circulation by 4D phase-contrast flow magnetic resonance imaging at 1.5 T.

International journal of cardiology
2024

Outcomes of Patients Undergoing the Kawashima Procedure at an Early Age: A Single Center Experience.

World journal for pediatric &amp; congenital heart surgery
2025

Augmented Biphasic Breathing Using Sniff and an Oral Positive Expiratory Pressure Device (Sniff-PEP) in Fontan Patients.

Pediatric cardiology
2024

Contained hepatic vascular injuries following liver trauma: a retrospective monocentric study and review of the literature.

International journal of surgery (London, England)
2024

Membranoproliferative glomerulonephritis in a child with congenital portosystemic shunt.

Pediatric nephrology (Berlin, Germany)
2024

Trans-umbilical Venous Approach for Embolization of Congenital Hepatic Haemangioma via Patent Ductus Arteriosus in a Preterm Neonate.

Cardiovascular and interventional radiology
2024

Primary disease of adipose tissue: When to think about and how to evaluate it in clinical practice?

Annales d'endocrinologie
2024

Infracardiac total anomalous pulmonary venous connection with dual drainage into the hepatic and portal venous system.

Journal of cardiovascular computed tomography
2025

Imaging features, classification and clinical features of intrahepatic congenital portosystemic shunts.

Journal of ultrasound
2024

Utilization of microflow imaging for the diagnosis of an intrahepatic tumor-like portal vein-hepatic vein fistula: A case study.

Asian journal of surgery
2024

A rare case of hepatic vein-portal vein fistula with hepatolithiasis presenting as cholangiocarcinoma.

Hepatobiliary &amp; pancreatic diseases international : HBPD INT
2024

Recurrence of Pulmonary Arteriovenous Malformation after Embolization in Patients with Pulmonary Hypertension.

Journal of vascular and interventional radiology : JVIR
2024

Anatomical classification of feline congenital extrahepatic portosystemic shunts based on CT angiography: A SVSTS and VIRIES multi-institutional study in 231 cats.

Veterinary radiology &amp; ultrasound : the official journal of the American College of Veterinary Radiology and the International Veterinary Radiology Association
2024

Impact of Age-Related Change in Caval Flow Ratio on Hepatic Flow Distribution in the Fontan Circulation.

Circulation. Cardiovascular imaging
2024

Regional differences in the umbilical vein and ductus venosus at different stages of normal human development.

Anatomical record (Hoboken, N.J. : 2007)
2024

Hereditary hemorrhagic telangiectasia with hepatic arteriovenous shunt diagnosed due to liver damage.

Clinical journal of gastroenterology
2024

Clinical Implications of Serum Mac-2 Binding Protein in Patients After Living Donor Liver Transplantation for Biliary Atresia.

Transplantation proceedings
2024

Platypnea-orthodeoxia syndrome (POS) in a patient who had undergone partial liver resection.

BMJ case reports
2024

Enlargement of the hepatic artery is present in dogs with a congenital extrahepatic portosystemic shunt and is independent of shunt insertion into the systemic circulation.

Veterinary radiology &amp; ultrasound : the official journal of the American College of Veterinary Radiology and the International Veterinary Radiology Association
2024

Autopsy of a 45-year-old Man with Liver Failure Due to Polycystic Liver Disease.

Internal medicine (Tokyo, Japan)
2024

Intrahepatic and anterior course of the inferior vena cava: CT image and 3D reconstruction of a rare anatomical variation.

Surgical and radiologic anatomy : SRA
2024

Absence of Ductus Venosus: A Comparison of 2 Distinctive Fetal Autopsy Cases and Embryologic Perspectives.

Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society
2024

Trans-catheter closure of an unusual type of Abernethy malformation in a child presenting with severe pulmonary hypertension.

Cardiology in the young
2024

Rectal Bleed in a Child With Abernathy Malformation- Endovascular Management.

Journal of clinical and experimental hepatology
2025

Establishment of Biliary Atresia Prognostic Classification System via Survival-Based Forward Clustering - A New Biliary Atresia Classification.

Indian journal of pediatrics
2024

Liver transplantation for pediatric patients with congenital heart disease: A single-center study in mainland China.

Pediatric transplantation
2024

A rare combination: a single common pulmonary vein with polysplenia syndrome.

Cardiology in the young
2023

Higher portal venous pulsatility is associated with worse clinical outcomes following congenital heart surgery: a single-centre prospective cohort study.

Canadian journal of anaesthesia = Journal canadien d'anesthesie
2023

Anomalous hepatic drainage and platypnea-orthodeoxia in cyanotic children.

Cardiology in the young
2023

In vivo hepatic flow distribution by computational fluid dynamics can predict pulmonary flow distribution in patients with Fontan circulation.

Scientific reports
2023

The Portal Vein: A Comprehensive Review.

Radiographics : a review publication of the Radiological Society of North America, Inc
2023

Computed tomographic measures of renomegaly vary among dogs with different types of congenital portosystemic shunts.

Veterinary radiology &amp; ultrasound : the official journal of the American College of Veterinary Radiology and the International Veterinary Radiology Association
2023

Impact of patent foramen ovale on short-term outcomes in children with biliary atresia undergoing living donor liver transplantation: a retrospective cohort study.

BMC anesthesiology
2024

Shrinkage of Enlarged Hepatic Nodules by an Embolizing Congenital Extrahepatic Portosystemic Shunt.

Internal medicine (Tokyo, Japan)
2024

An Unusual Presentation of SCAD in a Young Male Soldier.

Military medicine
2023

Congenital anomalous drainage of the inferior vena cava and hepatic veins into the left atrium associated with long QT syndrome: Multimodality imaging, pitfalls, and caveats.

Echocardiography (Mount Kisco, N.Y.)
2024

Outcomes of the Kawashima: A Society of Thoracic Surgeons Congenital Heart Surgery Database Analysis.

The Annals of thoracic surgery
2023

Laparoscopic Kasai Procedure of Treating Biliary Atresia with an Aberrant Right Hepatic Artery.

Journal of laparoendoscopic &amp; advanced surgical techniques. Part A
2023

The so-called "one-and-a-half" ventricular repair: where are we after 40 years?

Cardiology in the young
2023

Diagnostic and monitoring value of ultrasound, computed tomography angiography, and portal venography in surgical ligation of congenital extrahepatic portosystemic shunts.

European journal of radiology
2023

Isolated left hepatic vein draining into the coronary sinus: a rare anomaly encountered during mitral valve surgery.

European heart journal. Case reports
2023

Effective long-term sirolimus treatment in hypoxemia mainly due to intrapulmonary right-to-left shunt in a patient with multiple vascular anomalies.

Orphanet journal of rare diseases
2023

Radial nerve palsy caused by a rapidly growing intramuscular hematoma in an infant with biliary atresia: a case report.

BMC pediatrics
2023

Isolated persistent left superior vena cava associated with anomalous left hepatic vein drainage into the right atrium accidentally discovered after sternotomy.

Kardiologia polska
2023

Jejunal arteriovenous malformation and multiple acquired extrahepatic portosystemic shunts in a juvenile dog, presenting with melena.

The Journal of small animal practice
2023

Evaluation of Portal, Splenic, and Hepatic Vein Flows in Children Undergoing Congenital Heart Surgery.

Journal of cardiothoracic and vascular anesthesia
2024

Fatal acute portal vein thrombosis associated with hepatic cysts in a patient with autosomal dominant polycystic kidney disease.

CEN case reports
2023

Prepancreatic postduodenal portal vein: a case report and literature review.

Surgical case reports
2023

Successful coil embolisation of tortuous portosystemic venous shunts using a steerable microcatheter in a patient with polysplenia after a Fontan-type operation.

Cardiology in the young
2023

Portal-hepatic venous shunt in a patient with trisomy 21.

Clinics and research in hepatology and gastroenterology
2023

Novel Diabolo Configuration of a Large Covered Stent to Treat Cyanosis Related to Pulmonary Arteriovenous Malformations.

World journal for pediatric &amp; congenital heart surgery
2023

Utility of portal vein pulsatility fraction in patients undergoing corrective surgery for tetralogy of Fallot.

Cardiology in the young
2023

Assessment of liver fibrosis using a 3-dimensional high-resolution late gadolinium enhancement sequence in children and adolescents with Fontan circulation.

European radiology
2023

Congenital porto-pulmonary shunt in a dog.

Open veterinary journal
2023

Intra-abdominal haemorrhaging after cardiac catheterisation: the importance of recognising vascular anomalies in heterotaxy syndrome.

Cardiology in the young
2023

Correlation of Prenatal and Postnatal Diagnosis in Umbilical-Portal-Systemic Venous Shunts.

European journal of pediatric surgery : official journal of Austrian Association of Pediatric Surgery ... [et al] = Zeitschrift fur Kinderchirurgie
2022

A Rare Case of Extrahepatic Portal Venous Obstruction in a Nine-Year-Old Female and Its Management: A Case Report.

Cureus
2022

Large gastroduodenal artery pseudoaneurysm, arterioportal fistula and portal vein stenosis in chronic pancreatitis treated using combined transarterial embolization and transportal stenting: A case report.

Medicine
2023

An Extremely Rare Case of Intestinal Malrotation with an Absent Inferior Vena Cava.

The Kurume medical journal
2023

Portosystemic shunt, a rare cause of neonatal cholestatic hepatitis. A case report.

Archivos argentinos de pediatria
2023

Liver Explants of Biliary Atresia Patients Transplanted in Adulthood Show Features of Obliterative Portal Venopathy: Case Series and Guidelines for Pathologic Reporting of Adult Explants.

Archives of pathology &amp; laboratory medicine
2022

Haemodynamic performance of 16-20-mm extracardiac Goretex conduits in adolescent Fontan patients at rest and during simulated exercise.

European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery
2022

Clinical findings, diagnosis and therapy of patent ductus venosus in children: a case series.

Cardiovascular diagnosis and therapy
2023

A Comparison of Laparoscopic-Modified Kasai Versus Conventional Open Kasai for Biliary Atresia in Infants: A Single-Center Experience.

Journal of laparoendoscopic &amp; advanced surgical techniques. Part A
2022

Prenatal ultrasonographic features and follow-up outcomes of 19 cases of congenital intrahepatic portosystemic venous shunts diagnosed during the foetal period.

Insights into imaging
2023

Abernethy Malformation: Possible Diagnosis for Patients with Congenital Heart Disease and Persistent Cyanosis.

Brazilian journal of cardiovascular surgery
2022

Transcatheter closure of congenital portosystemic shunts - A multicenter experience.

Annals of pediatric cardiology
2023

Direct Hepatic Vein Connection to Left Atrium Without Congenital Anomaly in Adult Heart Disease.

Annals of thoracic surgery short reports
2022

Extracardiac Fontan completion after the Kawashima procedure with a custom-made bifurcated graft.

Multimedia manual of cardiothoracic surgery : MMCTS
2022

A clinicopathological reappraisal of orbital vascular malformations and distinctive GJA4 mutation in cavernous venous malformations.

Human pathology
2022

A Case of Primary Insular Ovarian Carcinoid Tumor with Hyperandrogenism and Carcinoid Heart Disease.

The American journal of case reports
2023

Obstetrical and neonatal complications in hereditary haemorrhagic telangiectasia: A retrospective study.

BJOG : an international journal of obstetrics and gynaecology
2023

Identifying racial disparities in hereditary hemorrhagic telangiectasia.

Journal of neurointerventional surgery
2023

[Robotic Left Hepatectomy Using the Glissonean Pedicle Approach for the Treatment of Caroli's Syndrome].

Zentralblatt fur Chirurgie
2022

Successful left hepatic trisectionectomy after portal vein embolization for colon cancer liver metastasis in a patient with right-sided ligamentum teres.

Clinical journal of gastroenterology
2022

[Osler's disease - a disease with a wide variety of clinical manifestations deserving multidisciplinary competence].

Lakartidningen
2022

Duodenopancreatectomy for PDAC Associated with MALS: A Case Report.

Chirurgia (Bucharest, Romania : 1990)
2022

Fetal intrahepatic Umbilical-Porto-Systemic venous shunts (IHUPSVS): In-utero anatomic classification.

European journal of obstetrics, gynecology, and reproductive biology
2022

Tetralogy of Fallot associated with a rare type partially anomalous pulmonary venous connection and levoatrial cardinal vein.

Journal of cardiac surgery
2023

Vascular liver diseases: A sex-oriented analysis of the literature.

Digestive and liver disease : official journal of the Italian Society of Gastroenterology and the Italian Association for the Study of the Liver
2022

Pancreatoduodenectomy with portal vein resection for pancreatic body cancer in a patient with situs inversus totalis.

Surgical oncology
2022

Pediatric Hepatic Cystic Lesions: Differential Diagnosis and Multimodality Imaging Approach.

Radiographics : a review publication of the Radiological Society of North America, Inc
2023

Biliary atresia-An experience from the first pediatric liver transplant center in Pakistan.

Pediatric transplantation
2022

Phrenic artery with fistula formation to pulmonary artery and vein: A rare case report.

Asian journal of surgery
2022

Complications of congenital portosystemic shunts: liver tumors are affected by shunt severity, but pulmonary and neurocognitive associations are not.

Hepatology international
2023

Extrahepatic approach for taping the common trunk of the middle and left hepatic veins or the left hepatic vein alone in laparoscopic hepatectomy (with videos).

Journal of hepato-biliary-pancreatic sciences
2022

Focal Nodular Hyperplasia and Focal Nodular Hyperplasia-like Lesions.

Radiographics : a review publication of the Radiological Society of North America, Inc
2022

Secundum Atrial Septal Defects Transcatheter Closure: An Updated Reappraisal.

Cardiovascular revascularization medicine : including molecular interventions
2022

Long-term follow-up of biliary atresia using liver transient elastography.

Pediatric surgery international
2022

Scimitar syndrome - An incidental diagnosis in a case of fibroadenoma.

Radiology case reports
2022

Outcome of Neonates Presenting With Severe Cardiac Failure due to Cerebral Arteriovenous Fistula.

Pediatric neurology
2022

[A Case of Early Gastric Cancer with Adachi Type Ⅵ Vascular Anomaly Treated by Laparoscopic Distal Gastrectomy].

Gan to kagaku ryoho. Cancer &amp; chemotherapy
2022

Clinical implications of serum autotoxin in regular follow up after pediatric living donor liver transplantation for biliary atresia.

Journal of pediatric surgery
2022

Transcatheter implantation of covered stents serving as extravascular conduits-Proof of a CT-based approach in three cases.

Catheterization and cardiovascular interventions : official journal of the Society for Cardiac Angiography &amp; Interventions
2022

Prenatal diagnosis of hepatic interruption of the inferior vena cava with azygos/hemiazygos continuation without structural heart defects: A case series.

Journal of clinical ultrasound : JCU
2022

Robot-assisted transhiatal lower esophagectomy and proximal gastrectomy for Siewert type II advanced esophagogastric junction cancer with situs inversus totalis: a case report.

Surgical case reports
2023

Traumatic high flow arterioportal fistula. Correction by a covered stent.

Revista espanola de enfermedades digestivas
2022

Multimodality imaging of Abernethy malformation.

Echocardiography (Mount Kisco, N.Y.)
2022

[Diagnosis and embolization therapy for a patient with portosystemic encephalopathy related to hereditary hemorrhagic telangiectasia].

Zhonghua gan zang bing za zhi = Zhonghua ganzangbing zazhi = Chinese journal of hepatology
2022

A novel Fontan Y-graft for interrupted inferior vena cava and azygos continuation.

Interactive cardiovascular and thoracic surgery
2022

A new variant of Abernethy malformation treated by transhepatic interventional closure: a case report.

BMC gastroenterology
2022

Successful embolization of a congenital intra-hepatic arterioportal fistula in a neonate with the MVP Microvascular Plug system (MVP-3Q).

Radiology case reports
2022

Comparable long-term results of the extracardiac conduit total cavopulmonary connection in heterotaxy to non-heterotaxy patients.

Journal of cardiac surgery
2022

Portal vein complications and outcomes following pediatric liver transplantation: Data from the Society of Pediatric Liver Transplantation.

Liver transplantation : official publication of the American Association for the Study of Liver Diseases and the International Liver Transplantation Society
2022

Adult Living Donor Liver Transplantation for the Carryover Patients After Kasai Operation Who Have a History of Multiple Laparotomies.

Transplantation proceedings
2022

Recurrent graft failure secondary to portal vein steal syndrome: a case report with an unusual indication for a third liver transplant.

BMC surgery
2022

Endovascular treatment of a portosystemic shunt presenting with hypoglycemia; case presentation and review of literature.

Clinical imaging
2022

Long-term outcomes in pediatric patients who underwent living donor liver transplantation for biliary atresia.

Surgery
2022

Stepwise Treatment for Heterotaxy Syndrome and Functional Single Ventricle Complicated by Infra-Cardiac Total Anomalous Pulmonary Venous Connection with Ductus Venosus Stent Placement and Subsequent Occlusion.

Pediatric cardiology
2022

A Case of High-Output Heart Failure.

Chest
2022

Computed tomographic features of portal vein thrombosis in two cats with splenosystemic shunts.

The Journal of small animal practice
2022

[Closure of a large arteriovenous malformation of the left lung in a patient after Fontan procedure (case report)].

Angiologiia i sosudistaia khirurgiia = Angiology and vascular surgery
2022

Neonatal Presentation of Congenital Portosystemic Shunt.

The Journal of pediatrics
2022

To the Editor: Pathophysiology of Splenic Arteriovenous Fistula.

Annals of vascular surgery
2022

Splenic Arteriovenous Fistula Accompanied by Splenic Artery Aneurysm Associated with Acute-onset Portal Hypertension and Gastrointestinal Bleeding: Case Report and Literature Review.

Annals of vascular surgery
2021

Congenital hepatic fibrosis and its mimics: a clinicopathologic study of 19 cases at a single institution.

Diagnostic pathology
2021

An Uncommon Variant of Nutcracker Syndrome Secondary to Left Renal Vein Compression Between the Right Renal Artery and The Proper Hepatic Artery.

Annals of vascular surgery

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Referências e fontes

Bases de dados externas citadas neste artigo

Publicações científicas

Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.

  1. Noncirrhotic Portopulmonary Hypertension Due to Hepatoportal Sclerosis in Adams-Oliver Syndrome.
    Pediatrics· 2026· PMID 41406992mais citado
  2. Terlipressin Therapy for Portal Hyperperfusion Secondary to Portal Vein Size Discrepancy After Pediatric Liver Transplant.
    Pediatric transplantation· 2026· PMID 41856932mais citado
  3. Sinus venosus atrial septal defect with partial anomalous pulmonary and hepatic venous drainage: a case report.
    Cardiology in the young· 2026· PMID 41732880mais citado
  4. Incidence of portosystemic shunts in Finnish miniature schnauzer litters.
    Acta veterinaria Scandinavica· 2026· PMID 41724984mais citado
  5. Left-Sided Portal Hypertension Induced by Splenic Arteriovenous Malformation: A Case Report.
    The American journal of case reports· 2026· PMID 41693181mais citado
  6. Hepatic venous malformations versus "hemangiomas": a clinical, radiologic, and pathologic analysis.
    Acta Radiol· 2026· PMID 41493947recente
  7. Congenital hepatic arteriovenous fistula: a case report and review of the literature.
    J Med Case Rep· 2025· PMID 41310891recente
  8. Laparoscopic Anatomical Left Hepatectomy Guided by the Dilated Bile Duct and Middle Hepatic Vein for Hepaticolithiasis in a Patient With Situs Inversus Totalis: How I Do It.
    Asian J Endosc Surg· 2025· PMID 41242877recente
  9. Cross-sectional imaging of congenital and acquired hepatic vascular abnormalities.
    Eur J Radiol· 2025· PMID 40663981recente
  10. First-in-man transcatheter Fontan completion in post Kawashima patient after successful recruiting of separately draining hepatic veins - A case report.
    Cardiol Young· 2025· PMID 40637518recente

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:95507(Orphanet)
  2. MONDO:0019836(MONDO)
  3. GARD:19282(GARD (NIH))
  4. Busca completa no PubMed(PubMed)
  5. Q55788909(Wikidata)

Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.

Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar

Anomalia congênita da veia hepática
Compêndio · Raras BR

Anomalia congênita da veia hepática

ORPHA:95507 · MONDO:0019836
CID-10
Q26.8 · Outras malformações congênitas das grandes veias
CID-11
MedGen
UMLS
C3163825
Wikidata
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