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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O que você precisa saber de cara
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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Linha do tempo da pesquisa
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O que está alterado no DNA e como passa nas famílias
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Os sinais que médicos procuram e os exames que confirmam
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🇧🇷 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
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Hospital Universitário Prof. Edgard Santos (HUPES)
R. Dr. Augusto Viana, s/n - Canela, Salvador - BA, 40110-060 · CNES 0003808
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Hospital Infantil Albert Sabin
R. Tertuliano Sales, 544 - Vila União, Fortaleza - CE, 60410-794 · CNES 2407876
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Hospital de Apoio de Brasília (HAB)
AENW 3 Lote A Setor Noroeste - Plano Piloto, Brasília - DF, 70684-831 · CNES 0010456
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Hospital Estadual Infantil e Maternidade Alzir Bernardino Alves (HIABA)
Av. Min. Salgado Filho, 918 - Soteco, Vila Velha - ES, 29106-010 · CNES 6631207
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Hospital das Clínicas da UFG
Rua 235 QD. 68 Lote Área, Nº 285, s/nº - Setor Leste Universitário, Goiânia - GO, 74605-050 · CNES 2338424
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Hospital Universitário da UFJF
R. Catulo Breviglieri, Bairro - s/n - Santa Catarina, Juiz de Fora - MG, 36036-110 · CNES 2297442
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Hospital das Clínicas da UFMG
Av. Prof. Alfredo Balena, 110 - Santa Efigênia, Belo Horizonte - MG, 30130-100 · CNES 2280167
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Hospital Universitário Julio Müller (HUJM)
R. Luis Philippe Pereira Leite, s/n - Alvorada, Cuiabá - MT, 78048-902 · CNES 2726092
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Hospital Universitário João de Barros Barreto
R. dos Mundurucus, 4487 - Guamá, Belém - PA, 66073-000 · CNES 2337878
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Hospital Universitário Lauro Wanderley (HULW)
R. Tabeliao Estanislau Eloy, 585 - Castelo Branco, João Pessoa - PB, 58050-585 · CNES 0002470
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Instituto de Medicina Integral Prof. Fernando Figueira (IMIP)
R. dos Coelhos, 300 - Boa Vista, Recife - PE, 50070-902 · CNES 0000647
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Hospital Pequeno Príncipe
R. Des. Motta, 1070 - Água Verde, Curitiba - PR, 80250-060 · CNES 3143805
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Hospital Universitário Regional de Maringá (HUM)
Av. Mandacaru, 1590 - Parque das Laranjeiras, Maringá - PR, 87083-240 · CNES 2216108
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Hospital de Clínicas da UFPR
R. Gen. Carneiro, 181 - Alto da Glória, Curitiba - PR, 80060-900 · CNES 2364980
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Hospital Universitário Pedro Ernesto (HUPE-UERJ)
Blvd. 28 de Setembro, 77 - Vila Isabel, Rio de Janeiro - RJ, 20551-030 · CNES 2280221
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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
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Hospital São Lucas da PUCRS
Av. Ipiranga, 6690 - Jardim Botânico, Porto Alegre - RS, 90610-000 · CNES 2232928
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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
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Hospital Universitário da UFSC (HU-UFSC)
R. Profa. Maria Flora Pausewang - Trindade, Florianópolis - SC, 88036-800 · CNES 2560356
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Hospital das Clínicas da FMUSP
R. Dr. Ovídio Pires de Campos, 225 - Cerqueira César, São Paulo - SP, 05403-010 · CNES 2077485
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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
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Hospital de Clínicas da UNICAMP
R. Vital Brasil, 251 - Cidade Universitária, Campinas - SP, 13083-888 · CNES 2748223
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Hospital de Clínicas de Ribeirão Preto (HCRP-USP)
R. Ten. Catão Roxo, 3900 - Vila Monte Alegre, Ribeirão Preto - SP, 14015-010 · CNES 2082187
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UNIFESP / Hospital São Paulo
R. Napoleão de Barros, 715 - Vila Clementino, São Paulo - SP, 04024-002 · CNES 2688689
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Publicações mais relevantes
Noncirrhotic Portopulmonary Hypertension Due to Hepatoportal Sclerosis in Adams-Oliver Syndrome.
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.
Terlipressin Therapy for Portal Hyperperfusion Secondary to Portal Vein Size Discrepancy After Pediatric Liver Transplant.
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.
Sinus venosus atrial septal defect with partial anomalous pulmonary and hepatic venous drainage: a case report.
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.
Incidence of portosystemic shunts in Finnish miniature schnauzer litters.
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.
Left-Sided Portal Hypertension Induced by Splenic Arteriovenous Malformation: A Case Report.
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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Internal medicine (Tokyo, Japan)Intrahepatic and anterior course of the inferior vena cava: CT image and 3D reconstruction of a rare anatomical variation.
Surgical and radiologic anatomy : SRAAbsence 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 SocietyTrans-catheter closure of an unusual type of Abernethy malformation in a child presenting with severe pulmonary hypertension.
Cardiology in the youngRectal Bleed in a Child With Abernathy Malformation- Endovascular Management.
Journal of clinical and experimental hepatologyEstablishment of Biliary Atresia Prognostic Classification System via Survival-Based Forward Clustering - A New Biliary Atresia Classification.
Indian journal of pediatricsLiver transplantation for pediatric patients with congenital heart disease: A single-center study in mainland China.
Pediatric transplantationA rare combination: a single common pulmonary vein with polysplenia syndrome.
Cardiology in the youngHigher 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'anesthesieAnomalous hepatic drainage and platypnea-orthodeoxia in cyanotic children.
Cardiology in the youngIn vivo hepatic flow distribution by computational fluid dynamics can predict pulmonary flow distribution in patients with Fontan circulation.
Scientific reportsThe Portal Vein: A Comprehensive Review.
Radiographics : a review publication of the Radiological Society of North America, IncComputed tomographic measures of renomegaly vary among dogs with different types of congenital portosystemic shunts.
Veterinary radiology & ultrasound : the official journal of the American College of Veterinary Radiology and the International Veterinary Radiology AssociationImpact of patent foramen ovale on short-term outcomes in children with biliary atresia undergoing living donor liver transplantation: a retrospective cohort study.
BMC anesthesiologyShrinkage of Enlarged Hepatic Nodules by an Embolizing Congenital Extrahepatic Portosystemic Shunt.
Internal medicine (Tokyo, Japan)An Unusual Presentation of SCAD in a Young Male Soldier.
Military medicineCongenital 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.)Outcomes of the Kawashima: A Society of Thoracic Surgeons Congenital Heart Surgery Database Analysis.
The Annals of thoracic surgeryLaparoscopic Kasai Procedure of Treating Biliary Atresia with an Aberrant Right Hepatic Artery.
Journal of laparoendoscopic & advanced surgical techniques. Part AThe so-called "one-and-a-half" ventricular repair: where are we after 40 years?
Cardiology in the youngDiagnostic and monitoring value of ultrasound, computed tomography angiography, and portal venography in surgical ligation of congenital extrahepatic portosystemic shunts.
European journal of radiologyIsolated left hepatic vein draining into the coronary sinus: a rare anomaly encountered during mitral valve surgery.
European heart journal. Case reportsEffective 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 diseasesRadial nerve palsy caused by a rapidly growing intramuscular hematoma in an infant with biliary atresia: a case report.
BMC pediatricsIsolated persistent left superior vena cava associated with anomalous left hepatic vein drainage into the right atrium accidentally discovered after sternotomy.
Kardiologia polskaJejunal arteriovenous malformation and multiple acquired extrahepatic portosystemic shunts in a juvenile dog, presenting with melena.
The Journal of small animal practiceEvaluation of Portal, Splenic, and Hepatic Vein Flows in Children Undergoing Congenital Heart Surgery.
Journal of cardiothoracic and vascular anesthesiaFatal acute portal vein thrombosis associated with hepatic cysts in a patient with autosomal dominant polycystic kidney disease.
CEN case reportsPrepancreatic postduodenal portal vein: a case report and literature review.
Surgical case reportsSuccessful coil embolisation of tortuous portosystemic venous shunts using a steerable microcatheter in a patient with polysplenia after a Fontan-type operation.
Cardiology in the youngPortal-hepatic venous shunt in a patient with trisomy 21.
Clinics and research in hepatology and gastroenterologyNovel Diabolo Configuration of a Large Covered Stent to Treat Cyanosis Related to Pulmonary Arteriovenous Malformations.
World journal for pediatric & congenital heart surgeryUtility of portal vein pulsatility fraction in patients undergoing corrective surgery for tetralogy of Fallot.
Cardiology in the youngAssessment of liver fibrosis using a 3-dimensional high-resolution late gadolinium enhancement sequence in children and adolescents with Fontan circulation.
European radiologyCongenital porto-pulmonary shunt in a dog.
Open veterinary journalIntra-abdominal haemorrhaging after cardiac catheterisation: the importance of recognising vascular anomalies in heterotaxy syndrome.
Cardiology in the youngCorrelation 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 KinderchirurgieA Rare Case of Extrahepatic Portal Venous Obstruction in a Nine-Year-Old Female and Its Management: A Case Report.
CureusLarge gastroduodenal artery pseudoaneurysm, arterioportal fistula and portal vein stenosis in chronic pancreatitis treated using combined transarterial embolization and transportal stenting: A case report.
MedicineAn Extremely Rare Case of Intestinal Malrotation with an Absent Inferior Vena Cava.
The Kurume medical journalPortosystemic shunt, a rare cause of neonatal cholestatic hepatitis. A case report.
Archivos argentinos de pediatriaLiver 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 & laboratory medicineHaemodynamic 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 SurgeryClinical findings, diagnosis and therapy of patent ductus venosus in children: a case series.
Cardiovascular diagnosis and therapyA Comparison of Laparoscopic-Modified Kasai Versus Conventional Open Kasai for Biliary Atresia in Infants: A Single-Center Experience.
Journal of laparoendoscopic & advanced surgical techniques. Part APrenatal ultrasonographic features and follow-up outcomes of 19 cases of congenital intrahepatic portosystemic venous shunts diagnosed during the foetal period.
Insights into imagingAbernethy Malformation: Possible Diagnosis for Patients with Congenital Heart Disease and Persistent Cyanosis.
Brazilian journal of cardiovascular surgeryTranscatheter closure of congenital portosystemic shunts - A multicenter experience.
Annals of pediatric cardiologyDirect Hepatic Vein Connection to Left Atrium Without Congenital Anomaly in Adult Heart Disease.
Annals of thoracic surgery short reportsExtracardiac Fontan completion after the Kawashima procedure with a custom-made bifurcated graft.
Multimedia manual of cardiothoracic surgery : MMCTSA clinicopathological reappraisal of orbital vascular malformations and distinctive GJA4 mutation in cavernous venous malformations.
Human pathologyA Case of Primary Insular Ovarian Carcinoid Tumor with Hyperandrogenism and Carcinoid Heart Disease.
The American journal of case reportsObstetrical and neonatal complications in hereditary haemorrhagic telangiectasia: A retrospective study.
BJOG : an international journal of obstetrics and gynaecologyIdentifying racial disparities in hereditary hemorrhagic telangiectasia.
Journal of neurointerventional surgery[Robotic Left Hepatectomy Using the Glissonean Pedicle Approach for the Treatment of Caroli's Syndrome].
Zentralblatt fur ChirurgieSuccessful left hepatic trisectionectomy after portal vein embolization for colon cancer liver metastasis in a patient with right-sided ligamentum teres.
Clinical journal of gastroenterology[Osler's disease - a disease with a wide variety of clinical manifestations deserving multidisciplinary competence].
LakartidningenDuodenopancreatectomy for PDAC Associated with MALS: A Case Report.
Chirurgia (Bucharest, Romania : 1990)Fetal intrahepatic Umbilical-Porto-Systemic venous shunts (IHUPSVS): In-utero anatomic classification.
European journal of obstetrics, gynecology, and reproductive biologyTetralogy of Fallot associated with a rare type partially anomalous pulmonary venous connection and levoatrial cardinal vein.
Journal of cardiac surgeryVascular 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 LiverPancreatoduodenectomy with portal vein resection for pancreatic body cancer in a patient with situs inversus totalis.
Surgical oncologyPediatric Hepatic Cystic Lesions: Differential Diagnosis and Multimodality Imaging Approach.
Radiographics : a review publication of the Radiological Society of North America, IncBiliary atresia-An experience from the first pediatric liver transplant center in Pakistan.
Pediatric transplantationPhrenic artery with fistula formation to pulmonary artery and vein: A rare case report.
Asian journal of surgeryComplications of congenital portosystemic shunts: liver tumors are affected by shunt severity, but pulmonary and neurocognitive associations are not.
Hepatology internationalExtrahepatic 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 sciencesFocal Nodular Hyperplasia and Focal Nodular Hyperplasia-like Lesions.
Radiographics : a review publication of the Radiological Society of North America, IncSecundum Atrial Septal Defects Transcatheter Closure: An Updated Reappraisal.
Cardiovascular revascularization medicine : including molecular interventionsLong-term follow-up of biliary atresia using liver transient elastography.
Pediatric surgery internationalScimitar syndrome - An incidental diagnosis in a case of fibroadenoma.
Radiology case reportsOutcome of Neonates Presenting With Severe Cardiac Failure due to Cerebral Arteriovenous Fistula.
Pediatric neurology[A Case of Early Gastric Cancer with Adachi Type Ⅵ Vascular Anomaly Treated by Laparoscopic Distal Gastrectomy].
Gan to kagaku ryoho. Cancer & chemotherapyClinical implications of serum autotoxin in regular follow up after pediatric living donor liver transplantation for biliary atresia.
Journal of pediatric surgeryTranscatheter 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 & InterventionsPrenatal diagnosis of hepatic interruption of the inferior vena cava with azygos/hemiazygos continuation without structural heart defects: A case series.
Journal of clinical ultrasound : JCURobot-assisted transhiatal lower esophagectomy and proximal gastrectomy for Siewert type II advanced esophagogastric junction cancer with situs inversus totalis: a case report.
Surgical case reportsTraumatic high flow arterioportal fistula. Correction by a covered stent.
Revista espanola de enfermedades digestivasMultimodality imaging of Abernethy malformation.
Echocardiography (Mount Kisco, N.Y.)[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 hepatologyA novel Fontan Y-graft for interrupted inferior vena cava and azygos continuation.
Interactive cardiovascular and thoracic surgeryA new variant of Abernethy malformation treated by transhepatic interventional closure: a case report.
BMC gastroenterologySuccessful embolization of a congenital intra-hepatic arterioportal fistula in a neonate with the MVP Microvascular Plug system (MVP-3Q).
Radiology case reportsComparable long-term results of the extracardiac conduit total cavopulmonary connection in heterotaxy to non-heterotaxy patients.
Journal of cardiac surgeryPortal 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 SocietyAdult Living Donor Liver Transplantation for the Carryover Patients After Kasai Operation Who Have a History of Multiple Laparotomies.
Transplantation proceedingsRecurrent graft failure secondary to portal vein steal syndrome: a case report with an unusual indication for a third liver transplant.
BMC surgeryEndovascular treatment of a portosystemic shunt presenting with hypoglycemia; case presentation and review of literature.
Clinical imagingLong-term outcomes in pediatric patients who underwent living donor liver transplantation for biliary atresia.
SurgeryStepwise 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 cardiologyA Case of High-Output Heart Failure.
ChestComputed tomographic features of portal vein thrombosis in two cats with splenosystemic shunts.
The Journal of small animal practice[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 surgeryNeonatal Presentation of Congenital Portosystemic Shunt.
The Journal of pediatricsTo the Editor: Pathophysiology of Splenic Arteriovenous Fistula.
Annals of vascular surgerySplenic Arteriovenous Fistula Accompanied by Splenic Artery Aneurysm Associated with Acute-onset Portal Hypertension and Gastrointestinal Bleeding: Case Report and Literature Review.
Annals of vascular surgeryCongenital hepatic fibrosis and its mimics: a clinicopathologic study of 19 cases at a single institution.
Diagnostic pathologyAn Uncommon Variant of Nutcracker Syndrome Secondary to Left Renal Vein Compression Between the Right Renal Artery and The Proper Hepatic Artery.
Annals of vascular surgeryAssociações
Organizações que acompanham esta doença — pra ter apoio e orientação
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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.
- Noncirrhotic Portopulmonary Hypertension Due to Hepatoportal Sclerosis in Adams-Oliver Syndrome.
- Terlipressin Therapy for Portal Hyperperfusion Secondary to Portal Vein Size Discrepancy After Pediatric Liver Transplant.
- Sinus venosus atrial septal defect with partial anomalous pulmonary and hepatic venous drainage: a case report.
- Incidence of portosystemic shunts in Finnish miniature schnauzer litters.
- Left-Sided Portal Hypertension Induced by Splenic Arteriovenous Malformation: A Case Report.
- Hepatic venous malformations versus "hemangiomas": a clinical, radiologic, and pathologic analysis.
- Congenital hepatic arteriovenous fistula: a case report and review of the literature.
- 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.
- Cross-sectional imaging of congenital and acquired hepatic vascular abnormalities.
- First-in-man transcatheter Fontan completion in post Kawashima patient after successful recruiting of separately draining hepatic veins - A case report.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:95507(Orphanet)
- MONDO:0019836(MONDO)
- GARD:19282(GARD (NIH))
- Busca completa no PubMed(PubMed)
- 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
