Baveno VII - Renewing consensus in portal hypertension: personalized care for portal hypertension / 中华肝脏病杂志

The Baveno VII workshop held in October 2021 was featured by the subject of personalized care in portal hypertension. The workshop focused on the following 9 topics including: the relevance and indications for measuring the hepatic venous pressure gradient as a gold standard; the use of non-invasive tools for the diagnosis of compensated advanced chronic liver disease and clinically significant portal hypertension; the impact of etiological and of non-etiological therapies in the course of cirrhosis; the prevention of the first episode of decompensation; the management of the acute bleeding episode; the prevention of further decompensation; as well as the diagnosis and management of splanchnic vein thrombosis and other vascular disorders of the liver. This essay provides a compilation and summary of recommendations regarding the abovementioned topics, and presents the most recent research proceedings and the corresponding consensus to our readers.

Expert consensus on clinical diagnosis and treatment of portal hypertension with hepatocellular carcinoma (2022) / 中华外科杂志

At present, there is no uniform standard for diagnosis and treatment of portal hypertension complicated with hepatocellular carcinoma internationally. Although in recent years, with the significant advances of surgical technique and the positive progress of targeted and immunotherapy in the field of hepatocellular carcinoma, the survival of hepatocellular carcinoma patients has improved, but the risk of surgery in patients with portal hypertension complicated with hepatocellular carcinoma remains high, and surgical treatment is still controversial. Therefore, based on the existing evidence, the Chinese Society of Spleen and Portal Hypertension Surgery, Chinese Society of Surgery, Chinese Medical Association has organized relevant experts to develop the consensus on clinical diagnosis and treatment of portal hypertension with hepatocellular carcinoma (2022) after full discussion. This consensus aims to provide the latest guidance for the standardized diagnosis and treatment of portal hypertension with hepatocellular carcinoma in China. Given that most portal hypertension originates from cirrhosis, this consensus only addresses the diagnosis and treatment of cirrhosis-related portal hypertension with hepatocellular carcinoma.

Cavernomatosis de la porta: presentación de un caso / Portal cavernomatosis: a case report

Fundamento: La cavernomatosis portal es una enfermedad poco frecuente causada por la trombosis de la vena porta, que provoca hipertensión portal (HP). Se ha relacionado con la realización de cateterismo umbilical, traumatismos abdominales e infecciones del período neonatal. La presentación clínica más frecuente es la hemorragia digestiva alta, con o sin melena, esplenomegalia, red venosa colateral y en etapas tardías puede observarse pancitopenia. Los métodos diagnósticos son ecografía abdominal, endoscopía digestiva y la angiotomografía. El diagnóstico definitivo es anatomopatológico. La literatura internacional y nacional es escasa para esta enfermedad, predominando el reporte de casos referidos a la edad pediátrica. Objetivo: presentar las características que definen esta enfermedad, en ocasión de darle seguimiento terapéutico a un paciente. Presentación de caso: se presenta un paciente de 20 años de edad, cuyo diagnóstico fue eventual por hallazgo ultrasonográfico en el periodo neonatal, con retraso madurativo y malnutrición proteico-energética. Conclusiones: la cavernomatosis portal o transformación cavernomatosa de la porta se define como la dilatación de las venas paracoledocianas y epicoledocianas generalmente secundaria a una trombosis portal, con una escasa prevalencia, fundamentalmente en edades pediátricas, que constituye la primera causa de hipertensión portal en este grupo etario. Provoca retardo del desarrollo pondoestatural, malnutrición proteicoenergética y sangramientos digestivos.

Background: Portal cavernomatosis is a rare disease caused by portal vein thrombosis, causing portal hypertension. It has been associated with performing umbilical catheterization, abdominal trauma and infections in the neonatal period. The most frequent clinical presentation is bleeding upper digestive, with or without melena, splenomegaly, collateral venous network and pancytopenia can be observed in late stages. Diagnostic methods are abdominal ultrasound, digestive endoscopy, and angiotomography. The definitive diagnosis is pathological. The international and national literature is scarce for this disease, with the predominant reporting of cases referring to pediatric age. Objective: to present the characteristics that define this disease, on the occasion of giving therapeutic follow-up to a patient. Case presentation: a 20-year-old patient is presented, whose diagnosis was eventual by ultrasound finding in the neonatal period, with maturational delay and protein-energy malnutrition. Conclusions: portal cavernomatosis or cavernomatous transformation of the Porta is defined as the dilation of the paracholedocian and epicoledocian veins generally secondary to portal thrombosis, with a low prevalence, mainly at pediatric ages, which is the leading cause of portal hypertension in this group. etareo. It causes delayed development of the body, protein-energy malnutrition and digestive bleeding.

Physiopathological and diagnostic aspects of cirrhotic cardiomyopathy / Aspectos fisiopatológicos y diagnósticos de la miocardiopatía cirrótica

Abstract Cirrhotic cardiomyopathy is characterized by the presence of structural and functional cardiac alterations in patients suffering from hepatic cirrhosis, without previously known cardiac causes that may explain it. Clinically, it is characterized by the presence of variable grades of diastolic and systolic dysfunction (SD), alterations in the electric conductance (elongation of corrected QT interval) and inadequate chronotropic response. This pathology has been related to substandard response in the management of patients with portal hypertension and poor outcome after transplant. Even when the first description of this pathology dates back from 1953, it remains a poorly studied and frequently underdiagnosed entity. Echocardiography prevails as a practical diagnostic tool for this pathology since simple measurements as the E/A index can show diastolic dysfunction. SD discloses as a diminished ejection fraction of the left ventricle and the latent forms are detected by echocardiography studies with pharmacological stress. In recent years, new techniques such as the longitudinal strain have been studied and they seem promising for the detection of early alterations.

Resumen La miocardiopatía cirrótica se caracteriza por la presencia de alteraciones cardiacas estructurales y funcionales en pacientes con cirrosis hepática, sin que existan otras causas de enfermedad cardiaca. Clínicamente se caracteriza por la presencia de grados variables de disfunción diastólica y sistólica, alteraciones de la conducción eléctrica (prolongación del intervalo QT) y respuesta cronotrópica inapropiada. Esta patología se ha relacionado con desenlaces clínicos adversos, mala respuesta en el manejo de la hipertensión portal y resultados desfavorables posterior a trasplante hepático ortotópico. A pesar de que las primeras descripciones datan de 1953, es una entidad poco estudiada y frecuentemente subdiagnosticada. El ecocardiograma es una herramienta de diagnóstico importante en esta entidad. Mediciones simples como el índice E/A pueden traducir disfunción diastólica. La disfunción sistólica se manifiesta con disminución de la fracción de eyección del ventrículo izquierdo y las formas latentes se detectan mediante estudios de ecocardiografía con estrés farmacológico; en los últimos años se han estudiado otras técnicas como el strain longitudinal, que parecen prometedoras en la detección de alteraciones tempranas.

CCI clinical practice guidelines: management of TIPS for portal hypertension (2019 edition) / 中华肝脏病杂志

Portal hypertension(PH) is one of the main complications of cirrhosis.Transjugular intrahepatic portosystemic shunt(TIPS) is the percutaneous creation of a conduit from the hepatic vein to the portal vein that is used to manage consequences of PH (i.e., variceal bleeding and refractory ascites) and used as a bridging therapy to liver transplant for decompensated cirrhosis. The following Clinical Practice Guidelines (CPGs) presents profession associational recommendations of the Chinese College of Interventionalists(CCI) on TIPS for PH. The CPGs was written by more than 30 experts in the field of TIPS in China (including interventional radiologists, liver surgeons, hepatologists and gastroenterologist, et al.). The panel of experts, produced these CPGs using evidence from PubMed and Cochrane database searches and combined with relevant expert consensuses and high quality clinical researches in China providing up to date guidance on TIPS for PH with the only purpose of improving clinical practice.

Fibrose hepática congênita e venopatia portal obliterativa sem hipertensão portal - revisão da literatura com base em um caso assintomático / Congenital hepatic fibrosis and obliterative portal venopathy without portal hypertension - a review of literature based on an asymptomatic case

ABSTRACT The disease and the case reported here are relevant especially because of their varied clinical presentation, possibility of being associated with other disorders affecting several organs and possible differential diagnoses. Congenital Hepatic Fibrosis is an autosomal recessive disease due to mutation in the PKHD1 gene, which encodes the fibrocystin/polyductine protein. It is a cholangiopathy, characterized by varying degrees of periportal fibrosis and irregular proliferation of bile ducts. Affected patients are typically diagnosed in childhood, but in some cases the disease may remain asymptomatic for many years. The exact prevalence and incidence of the disease are not known, but it is consider a rare disease, with a few hundred cases described worldwide. It can affect all ethnic groups and occur associated with various hereditary and non-hereditary disorders. The clinical presentation is quite variable, with melena and hematemesis being initial symptoms in 30%-70% of the cases. More rarely, they may present episodes of cholangitis. The disease has been classified into four types: portal hypertension, cholestasis / cholangitis, mixed and latent. Diagnosis begins with imaging tests, but the definition is made by the histopathological sample. So far, there is no specific therapy that can stop or reverse the pathological process. Currently, the therapeutic strategy is to treat the complications of the disease.

RESUMO A patologia e o caso aqui reportados são relevantes especialmente devido sua variada apresentação clínica, possibilidade de estar associada com outras desordens acometendo diversos órgãos e pelos possíveis diagnósticos diferenciais. A fibrose hepática congênita é uma doença autossômica recessiva, devido mutação no gene PKHD1, que codifica a proteína fibrocistina/poliductina. É uma colangiopatia, caracterizada por variados graus de fibrose periportal e proliferação irregular de ductos biliares. Os pacientes acometidos são tipicamente diagnosticados na infância, mas em alguns casos a doença pode permanecer assintomática por muitos anos. Exatas prevalência e incidência da doença não são conhecidas, mas sabe-se que é uma doença bastante rara, com algumas centenas de casos descritos no mundo. Pode afetar todos grupos étnicos e ocorrer associada com diversas desordens hereditárias e não-hereditárias. A apresentação clínica é bastante variável, com melena e hematêmese sendo sintomas iniciais em 30%-70% dos casos. Mais raramente, podem apresentar episódios de colangite. A doença tem sido classificada em quatro tipos: hipertensão portal, colestática/colangite, mista e latente. O diagnóstico inicia com exames de imagem, mas a definição é feita pela amostra histopatológica. Até o momento, não há terapia específica que possa parar ou reverter o processo patológico e a estratégia terapêutica atual é tratar as complicações da doença.

Hipertensión portopulmonar: Revisión actualizada / Portopulmonary hypertension: Updated review

Resumen: La hipertensión portopulmonar (HPP) es una entidad poco frecuente a nivel mundial, aunque se desconocen los datos epidemiológicos en México. Sin embargo, las enfermedades crónicas del hígado son muy prevalentes en mexicanos. La HPP es el 4.◦ subtipo en frecuencia del grupo de la hipertensión arterial pulmonar. Su diagnóstico está dentro de 2 escenarios: los pacientes con sospecha de hipertensión pulmonar y los candidatos a trasplante hepático ortotópico (THO). Tanto el ecocardiograma como el cateterismo cardiaco derecho son determinantes para el diagnóstico en ambos escenarios. La HPP es un reto para el THO, pues aumenta la mortalidad perioperatoria de manera importante. El uso de terapia específica es la piedra angular de este padecimiento, como una medida para poder mejorar el desenlace de los que llegan a ser candidatos a un THO con HPP moderada a grave. Es importante reconocer que la HPP puede llegar a ser una contraindicación para el THO. Hasta el momento el papel del trasplante combinado pulmón-hígado o corazón-pulmón-hígado como una medida de curación de la enfermedad vascular pulmonar en pacientes con HPP es incierto. © 2016 Instituto Nacional de Cardiolog´ıa Ignacio Cha´vez. Publicado por Masson Doyma Me´xico S.A. Este es un art´ıculo Open Access bajo la licencia CC BY-NC-ND (https://creativecommons.org/licenses/by-nc-nd/4.0/).

Abstract: Portopulmonary hypertension (PPH) is a rare condition worldwide, although epidemiological data are unknown in Mexico. However, chronic liver diseases are very prevalent in Mexico. PPH is the 4th subtype in frequency in the group of pulmonary arterial hypertension. Its diagnosis is made within 2 scenarios: patients with suspected pulmonary hypertension and candidates for orthotopic liver transplantation (OLT). Both echocardiogram and a right cardiac catheterisation are crucial for diagnosis in both cases. PPH is a challenge for OLT, since it can significantly increase perioperative mortality. The use of specific therapy is the cornerstone of this disease, as a measure to improve the outcome of those who become candidates for OLT with moderate to severe PPH. It is important to recognise that PPH can be a contraindication to OLT. The role of lung-liver transplantation or heart-lung-liver transplantation as a measure to heal pulmonary vascular disease in patients with PPH is still uncertain.© 2016 Instituto Nacional de Cardiolog´ıa Ignacio Cha´vez. Published by Masson Doyma Me´xico S.A. This is an open access article under the CC BY-NC-ND license (http://creativecommons. org/licenses/by-nc-nd/4.0/).

Enfrentamiento de pacientes pediátricos con várices esofágicas / Management of pediatric patients with esophageal varices

Background: There is a paucity of good quality research about the diagnosis of esophageal varices and the prophylaxis and treatment of variceal bleeding in pediatric patients with portal hypertension There is little consensus and practically no evidence-based approach about the management of these patients. Aim: To describe the behavior and preferences of pediatric gastroenterologists in Chile in the management of portal hypertension in children. Material and Methods: An online survey was sent to Chilean pediatric gastroenterologists, with questions evaluating the physicians’ approaches to screening of esophageal varices in children with portal hypertension, and their preferred methods of prophylaxis and initial management of variceal bleeding. Results: Thirty five of 69 contacted physicians answered the survey (51%). Twenty nine pediatric gastroenterologists (83%) screen for esophageal varices in patients with clinical evidence of portal hypertension, and 12 (34%) in every patient with chronic liver disease. Twenty eight respondents (80%) use primary prophylaxis, mainly beta blockers. Octreotide, proton pump inhibitors and endoscopy are the most common practices in the initial management of an esophageal varix bleed. The methods mostly used as secondary prophylaxis are band ligation and beta blockers. In the case of recurrent hemorrhage, besides band ligation, management with Transjugular Intrahepatic Portosystemic Shunt (TIPS) and hepatic transplantation are more likely. Conclusions: Even though most pediatric gastroenterologists in this survey are inclined to offer endoscopic screening of esophageal varices and prophylaxis to patients with portal hypertension, this is not a universal behavior. There are different approaches mainly in the election of secondary prophylaxis and the initial management of variceal bleeding.

Hipertensão portopulmonar / Portopulmonary hypertension

A hipertensão portopulmonar (POPH) é definida como hipertensão pulmonar na presença de hipertensão portal. Acomete cerca de 5% dos pacientes com hipertensão portal, cirróticos ou não, em avaliação para transplante hepático. A fisiopatologia dessa doença não está completamente elucidada. O hiperfluxo pulmonar e a formação conexões porto-sistêmicas, que permitem o acesso de bactérias e de substâncias inflamatórias aos pulmões, são mecanismos prováveis. Variações genéticas possivelmente estão implicadas na variabilidade do comprometimento vascular pulmonar dos pacientes com hipertensão portal. O ecocardiograma tem papel importante como rastreamento, sendo o diagnóstico definitivo realizado através do cateterismo cardíaco direito. A POPH pode ser considerada contraindicação ao transplante hepático, nos casos moderados e graves, no entanto, o tratamento com vasodilatadores pulmonares, indicado para esses pacientes, pode ser capaz de melhorar o perfil hemodinâmico e permitir que mais pacientes atinjam os critérios de segurança para o transplante. Com as novas abordagens terapêuticas, observou-se melhora no prognóstico desses pacientes nos últimos anos...

Portopulmonary hypertension (POPH) is defined as pulmonary hypertension in the presence of portal hypertension. It affects about 5% of patients with portal hypertension, cirrhotic or not, under evaluation for liver transplantation. The pathophysiology of this disease is not completely understood, but hyperdynamic state in pulmonary circulation and formation of porto-systemic connections, which allow access of intestinal bacteria and inflammatory substances to the lungs, are probably involved. Genetic variations possibly play a role in the variability of the pulmonary vascular impairment of patients with portal hypertension. Echocardiography is important for screening, but the definitive diagnosis is made by right heart catheterization. The POPH can be considered a contraindication for liver transplantation in moderate to severe cases, however, treatment with pulmonary vasodilators, indicated for these patients, may be able to improve hemodynamic profile and allow more patients meet the criteria for transplantation. With new therapeutic approaches, there was an improvement in the prognosis of these patients in recent years...

Hipertensión portal en el embarazo: presentación de un caso / Portal hypertension in pregnancy: report of a case

La coexistencia de embarazo y enfermedad hepática representa una situación clínica compleja. Durante el embarazo se desarrolla un estado hipervolémico debido a un flujo esplácnico incrementado, que contribuye a mayor presión portal transmitida a las venas colaterales que incrementan el riesgo de hemorragia varicosa en este grupo de pacientes. Se reporta el caso de una paciente de 39 años en el sexto embarazo y sin ningún antecedente médico previo que presenta hipertensión portal pre-sinusoidal y que gracias al manejo multidisciplinario adecuado, tuvo un parto sin complicaciones. Se revisa la literatura pertinente al caso.

The coexistence of pregnancy and liver disease represents a complex clinical situation. Pregnancy develops hypervolemic state due to increased splachnic blood flow, which contributes to increased portal pressure transmitted to collateral veins that increase the risk of variceal bleeding in these patients. We report the case of a 39 years old patient in the sixth pregnancy and without any previous medical history that presented pre-sinusoidal portal hypertension, and thanks to appropriate multidisciplinary management had an uncomplicated delivery. We review the literature relevant to the case.

Perfil evolutivo das varizes esofágicas pós esplenectomia associada à ligadura da veia gástrica esquerda e escleroterapia na hipertensão portal esquistossomótica / Evolutional profile of the esophageal varices after splenectomy associated with ligation of the left gastric vein and sclerotherapy in schistosomal portal hypertension

RACIONAL: A esquistossomose mansônica afeta 200 milhões de pessoas em 70 países do mundo. Estima-se que 10% dos infectados evoluirão para a forma hepatoesplênica e, destes, 30% progredirão para hipertensão portal e varizes esofagogástricas, cuja expressão será através de hemorragia digestiva com mortalidade relevante no primeiro episódio hemorrágico. Múltiplas técnicas cirúrgicas foram desenvolvidas para prevenir o ressangramento. OBJETIVO: Avaliar o perfil evolutivo das varizes esofágicas após esplenectomia + ligadura da veia gástrica esquerda associada à escleroterapia endoscópica na hipertensão portal esquistossomótica. MÉTODO: Estudo prospectivo, observacional, de pacientes esquistossomóticos com antecedentes de hemorragia digestiva alta, submetidos à esplenectomia + ligadura da veia gástrica esquerda e escleroterapia. As variáveis estudadas foram perfil evolutivo das varizes esofágicas antes e após a operação e índice de recidiva hemorrágica. RESULTADOS: Amostra foi constituída por 30 pacientes distribuídos, quanto ao gênero, em 15 doentes para cada sexo. A idade variou de 19 a 74 anos (mediana=43 anos). Houve redução do grau, calibre e red spots em todos os pacientes (p<0,05). A erradicação das varizes com escleroterapia foi alcançada em 86,7% e exclusivamente com a operação em 15,4% dos pacientes.O tempo de seguimento médio foi de 28 meses, variando de dois a 76 meses. Foram realizadas de uma a sete sessões de escleroterapia e média de três por paciente para erradicar as varizes. Quatro pacientes (13,3%) não completaram o seguimento. A recidiva hemorrágica foi de 16,7%. CONCLUSÃO: Houve redução do grau, calibre e dos red spots das varizes esofágicas em todos os pacientes.

BACKGROUND: The schistosomiasis affects 200 million people in 70 countries worldwide. It is estimated that 10% of those infected will develop hepatosplenic status and of these, 30% will progress to portal hypertension and esophagogastric varices, whose expression is through gastrointestinal bleeding with significant mortality in the first bleeding episode. Multiple surgical techniques have been developed to prevent re-bleeding. AIM: To evaluate the evolutional profile of esophageal varices after splenectomy + ligation of the left gastric vein associated with endoscopic sclerotherapy in schistosomal portal hypertension. METHODS: Prospective and observational study including schistosomiasis patients with previous history of upper digestive hemorrhage and underwent to splenectomy + ligation of the left gastric vein and sclerotherapy. The variables were: evolutional profile of esophageal varices before and after surgery and re-bleeding rate. RESULTS: The sample included 30 patients, 15 patients for each gender. The age ranged from 19 to 74 years (median = 43 years). There was a reduction in the degree, caliber and red spots in all patients (p< 0.05). The eradication of varices with sclerotherapy was achieved in 86.7% and with surgery alone in 15.4%. The mean follow-up was 28 months, ranging from two to 76 months. Were carried from one to seven sessions of sclerotherapy and the average was three per patient to eradicate varices. Four (13.3%) did not complete the follow-up. The re-bleeding rate was 16.7%. CONCLUSION: There was a reduction of the degree, caliber and red spots of esophageal varices in all patients.

Fístula arterio-venosa intrahepática como causa de hipertensión portal no cirrótica, en paciente portador de hepatitis autoinmune: diagnóstico y tratamiento: caso clínico / Autoimmune hepatitis patient carrier with non cirrhotic portal hypertension caused by an intrahepatic portal arteriovenous fistula: diagnosis and treatment: clinical case

Portal hypertension (PH) is defined as pathological increase of hydrostatic pressure in the portal venous system, usually related to liver cirrhosis. Among the uncommon causes of PH is the arteriovenous intra or extrahepatic fistula (AVF) of traumatic, iatrogenic or congenital origin. Clinical history and ultrasound findings of AVF are very important for the diagnosis. From a therapeutic point of view, there are three alternatives: clinical/imaging follow-up, surgical repair and transcutaneous catheter embolization. A report of a clinical case and a review of the literature are presented. Patient with portal hypertension as a result of intra hepatic AVF, successfully treated by transcutaneous catheter embolization. A 54 year-old female patient, and cholecystectomized and with history of breast cancer, presented altered liver function tests several months after gallbladder surgery. Once biliary disease was ruled out, liver biopsy was performed, which was compatible with autoimmune hepatitis. During follow-up, intrahepatic AVF was observed by means of ultrasound. Underlying disease was successfully managed with Prednisone and Azathioprine. Nine years later, she experienced an episode of confusion and disorientation compatible with hepatic encephalopathy (HE) and esophageal varices diagnosed by upper endoscopy. Laboratory tests and imaging did not show progression baseline liver disease. Angiographic procedures confirmed an intra hepatic AVF and selective embolization was carried out. There was clinical remission of HE and esophageal varices. We concluded, that transcutaneous catheter embolization is a valid alternative for the treatment of intra hepatic AVF, which accounted for the successful result for this particular patient.

La hipertensión portal (HP) se define como el aumento patológico de la presión hidrostática en el sistema venoso portal, habitualmente relacionada con cirrosis hepática. Entre las causas infrecuentes de HP está la fístula arterio-venosa (FAV) intra o extrahepática de origen traumáticas, iatrogénicas o congénitas entre otras. En el diagnóstico son importantes los antecedentes clínicos y hallazgos ecográficos que demuestran FAV. Desde el punto de vista terapéutico, existen tres alternativas: seguimiento clínico y de imágenes, reparación quirúrgica y embolización con catéter transcutáneo. Se presenta caso clínico de paciente con (HP) a consecuencia de una FAV intrahepática, tratada satisfactoriamente mediante embolización con catéter transcutáneo y revisión de la literatura pertinente. Se trata de una paciente de 54 años con antecedentes de cáncer mamario y colecistectomía en quien se constatan alteraciones de pruebas hepáticas varios meses con posterioridad a cirugía vesicular. Una vez descartada patología biliar, se realizó biopsia hepática la que fue compatible con hepatitis autoinmune. Durante el seguimiento se pesquisó FAV intrahepática como hallazgo ecográfi co. Su enfermedad de base se trató satisfactoriamente con Prednisona y Azatioprina. Nueve años más tarde, consulta por episodio de confusión y desorientación compatible con encefalopatía hepática (EH) y presencia de várices esofágicas a la endoscopia. Tanto el laboratorio como imágenes no mostraron progresión de enfermedad hepática de base. Es sometida a procedimiento angiográfico, que confirmó FAV intrahepática, procediendo a embolización selectiva. Hubo remisión del cuadro clínico de EH y regresión de las várices esofágicas. Se concluye que la embolización con catéter transcutáneo, es una alternativa válida en el tratamiento de FAV intrahepáticas, terapia que constituyó la solución definitiva del cuadro clínico reportado.

Haga su diagnóstico / Make your diagnosis

El diagnóstico es Fístula arterioportal congénita

Anastomose portossistêmica intra-hepática transjugular (TIPS): análise casuística de 28 casos em um hospital de referência / Transjugular intrahepatic portosystemic shunts transjugular liver (TIPS): analisys of 28 patients in a hospital cases of reference

Introdução: TIPS (anastomose intra-hepática portossistêmica transjugular) surgiu como uma alternativa para as complicações da hipertensão porta não-responsiva ao tratamento convencional, e está cada vez mais sendo utilizada. Objetivos: estudar os resultados da colocação do TIPS em 28 pacientes em um hospital de Porto Alegre e descrevê-los segundo indicações, gênero, idade, classificação de Child-Pugh, etiologia da hipertensão porta e gradiente de pressão porta. Método: estudo descritivo do tipo série de casos realizado na cidade de Porto Alegre, Rio Grande do Sul, envolvendo pacientes que foram submetidos ao implante de TIPS, no período de 1999 a 2009, através da revisão de seus prontuários. Resultados: a indicação mais comum para o implante de TIPS foi ascite refratária (n=19, 67,8%), e a maioria dos pacientes tinha a classe C de Child (n=16, 57,1%) de origem etiológica por hepatite C (n=19, 67,9%). O sucesso da técnica foi alcançado em 26 (92,8%) pacientes, porém, houve complicações imediatas ao procedimento em onze pacientes e foram as seguintes: estenose do stent por trombose (n=4) ou por oclusão (n=5), ressangramento das varizes esofágicas logo após o procedimento (n=1) e encefalopatia portossistêmica (n=1). Houve impossibilidade de implante devido à fibrose hepática em dois pacientes, não sendo considerada como complicação já que o procedimento não foi finalizado. Conclusão: este estudo confirma a eficácia do TIPS no manejo da hipertensão porta, mostrando uma taxa de sucesso da técnica de 92,8%. A indicação mais comum neste estudo foi ascite refratária e a etiologia foi cirrose por hepatite C. A complicação que mais surgiu foi oclusão do stent, sendo, na maioria dos pacientes, resolvida após um ou mais procedimentos de redilatação.

Introduction: TIPS (transjugular intrahepatic portosystemic shunt) has emerged as an alternative to treat the complications of portal hypertension non responsive to the conventional treatment and it has been used more and more often. Objectives: to study the results of TIPS creation in 28 patients in a hospital in Porto Alegre and to describe them according to indication, gender, age, Child-Pugh classification, portal hypertension etiology and portal pressure gradient. Method: descriptive study of case series carried out in Porto Alegre, Rio Grande do Sul, involving patients who had undergone TIPS creation between 1999 and 2009. Results: the most common indication for TIPS creation was refractory ascites (n=19, 67.8%) and most patients were classified as Child-Pugh class C (n=16, 57.1%) originated from hepatitis C (n=19, 67.9%). The technique was successful in 26 (92.8%) patients. However, there were immediate complications to the procedure in eleven patients. The complications were as follows: stenosis of stent by thrombosis (n=4) or by occlusion (n=5), esophageal varices bleeding rig ht after the procedure (n=1) and portosystemic encephalopathy (n=1). It was not possible to create TIPS in two patients due to hepatic fibrosis, and this was not considered a complication since the procedure was not concluded. Conclusion: this study confirms the efficiency of TIPS in the management of portal hypertension, showing a success rate of 92.8%. The most common indication in this study was refractory ascites and the ethiology was cirrhosis caused by hepatitis C. The most frequent complication was occlusion of stent, which was solved, in most cases, by using one or more procedures of repeat dilatation.

Consenso Baveno V: ¿Qué hay de nuevo en hipertensión portal? / Baveno V consensus workshop: what is new in portal hypertension?

In patients with cirrhosis and portal hypertension, variceal bleeding is a severe complication, and still a major cause of death. From 1986 to 2010 (Baveno V), several consensuses related to this topic have been carried out. The main purposes of these meetings were to develop clear definitions and therapeutic recommendations aimed at the standardization and increased ease of interpretation among different studies, to be homogeneous, which is essential for meta-analysis. This applies both to the definition of events related to variceal hemorrhage and therapeutic behaviour. In Baveno V some definitions were modified such as criteria for failure to control bleeding, and failure of secondary prophylaxis, while other definitions were validated. As in Baveno IV, the level of evidence (1 being the highest, 5 being the lowest) and the grades of recommendation (‘A’ the strongest, ‘D’ the weakest) were assessed according to the Oxford System. Particular emphasis was placed on management of non-cirrhotic portal hypertension, especially on the Budd-Chiari syndrome and extra-hepatic portal vein obstruction. Areas requiring further study include mechanism in the development and progression of portal hypertension; the development of non-invasive techniques to identify patients with clinically significant portal hypertension; the assessment of the impact of the management of chronic liver disease in the development of this condition; the role of regular control of portal hypertension in the prevention of the development and progression of gastroesophageal varices. Finally more information about non-cirrhotic portal hypertension is needed, especially on the frequency, primary prophylaxis of variceal bleeding, factors associated with treatment failure, progression and thrombosis recurrence.

La hipertensión portal (HP) constituye un alteración hemodinámica severa, responsable de las principales complicaciones de la cirrosis, entre las cuales está la hemorragia por várices gastroesofágicas. Desde el año 1986 y hasta el 2010 (Baveno V), se han efectuado diferentes reuniones de consensos, cuyos objetivos han sido estandarizar criterios, haciéndolos simples y reproducibles, de tal forma de homogeneizar los resultados de estudios clínicos y meta-análisis. Esto es válido tanto para la definición de eventos relacionados con hemorragia variceal, como de conductas terapéuticas. En Baveno V se han modificado algunas definiciones como los criterios de falla en el control de la hemorragia, y de falla en la profilaxis secundaria, mientra que otras se validaron. Al igual que en Baveno IV, se utilizó el Sistema Oxford, para representar el nivel de evidencia (1 el mayor; 5 el menor) y de recomendación (‘A’ la más fuerte y ‘D’ la de menor peso). Especial hincapié se ha hecho en el manejo de la HP de origen no cirrótica (Síndrome de Budd-Chiari y obstrucción de la vena porta extra-hepática). Finalmente, las áreas con desafíos en investigación clínica incluyen mecanismos en el desarrollo y progresión de la HP; empleo de medidas no invasivas para identificar pacientes con HP de significación; evaluación del impacto que tiene el manejo de la enfermedad hepática de base, en el desarrollo de esta complicación; el papel de la medición de la presión portal en la prevención del desarrollo y progresión de las várices gastroesofágicas. Finalmente, se recomienda realizar estudios prospectivos sobre prevalencia y evolución clínica de la HP no cirrótica y profilaxis primaria de la hemorragia variceal; identificación de la población de riesgo y de factores asociados con falla al tratamiento, progresión de la enfermedad y recurrencia.

Hipertensión portal en pediatría: II: complicaciones hemorrágicas / Portal hypertension in pediatrics: II: hemorrhagic complications

La hemorragia por várices esofágicas es la complicación más grave de la hipertensión portal y su tratamiento debe hacerse en centros con la complejidad y capacitación que requiere. El tratamiento con fármacos vasoactivos, especialmente el octreótido, asociado a la ligadura o esclerosis endoscópica de las várices es eficaz en el 90% de los casos. Los tratamientos de rescate, como TIPS (derivación portosistémica intrahepática transyugular) o cirugía, deben estar disponibles inmediatamente para aquellos pacientes en los que el sangrado no se detiene o presentan várices de difícil manejo.

Bleeding from esophageal varices is the most severe complication of portal hypertension, and should be managed in specially trained centers. Vasoactive drugs, mainly octreotide, plus endoscopic treatment are able to control bleeding in 90% of the cases. Rescue treatments like TIPS and surgery should be immediately available for those who do not stop bleeding or have varicesdifficult to manage.

message from the past: splenectomy and devascularisation with salvage sclerotherapy in the treatment of bleeding schistosomal oesophageal varices

To study the short and long term results of splenectomy and esophagogastric devascularisation in the prevention of bleeding from esophageal varices secondary to schistosomal portal hypertension. This is a retrospective prospective study of patients, who underwent splenectomy and devascularisation for schistosomal bleeding varices during the period 1980 to 1990 at Soba university hospital. They had clinical, laboratory, and endoscopic evaluation. They were followed up for a period of 10 years [mean 4.5 years]. At follow up special emphasis was laid on recurrence of variceal hemorrhage and, or mortality. Patients with recurrent variceal bleeding received emergency treatment in the form of resuscitation, balloon tamponade when necessary, and flexible endoscopic sclerotherapy. Splenectomy and oesophagogastric devascularisation was performed in 185 patients, and devascularisation only in 5 patients. Early postoperative mortality occurred in 8 patients [4.2%]. Early recurrence of variceal bleeding occurred in 4 patients who responded to emergency treatment. Transient of treatable ascitis developed in 21% of patients, transient jaundice in 12.6%, and liver failure in 1.6%. Major postoperative septic complications occurred in 6.4%. The rate of recurrence of variceal bleeding was 18.1% within the first 5 years, and 23.6% by 10 years follow-up. Late mortality occurred in 8 patients [4.4%]. The overall mortality of the procedure was 8.4%. Splenectomy and oesophagogastric devascularisation for the treatment of schistosomal portal hypertension can be associated with a high rate of variceal rebleeding. However, when coupled with sclerotherapy for recurrence, it carries an acceptable mortality rate

Treatment Update on Portal Hypertension and Complications / 대한소화기학회지

Current understanding of the pathophysiology of portal hypertension has resulted in therapeutic approaches aimed at correcting the increased splanchnic blood flow and some of which have been already used in clinical practice. Recently new perspectives opened and erstwhile paradigm has been changed to focus on increased resistance to portal blood flow and the formation of portosystemic collateralization. Several studies revealed the clear-cut mechanisms of hepatic endothelial dysfunction and abnormal angiogenesis contributing to the development of portal hypertension. Thus the modulations of hyperdynamic circulation or angiogenesis seem to be valuable therapeutic targets. In the current review update, we discuss the multidisciplinary management of modulating hepatic vascular resistance and abnormal angiogenesis associated with portal hypertension. However, these new pharmacological approaches are still under investigation and widescale clinical application are needed to develop effective strategies.

Síndrome hepatopulmonar e hipertensión portopulmonar: dos entidades a diferenciar / Hepatopulmonary syndrome and portopulmonary hypertension: two distinct entities

The clinical course of patients with portal hypertension or liver disease may be complicated by two low prevalence entities with high morbimortality: the hepatopulmonary syndrome (HPS) and portopulmonary hypertension (PPHT). Each one is a consequence ofan impaired hepatic clearance of several vascular mediators, triggering vasodilation of the pulmonary vascular territory in HPS and vasoconstriction with vessel remodelation in PPHT. Both disorders have some similar clinical findings, but useful findings for differential diagnosis are the presence of platypnoea and orthodeoxia in HPS, and echocardiographic extracardiac and intrapulmonary shunt in HPS or pulmonary hypertension in PPHT. Currently, liver transplantation is the only effective treatment for both entities provided that indication and timing must be accurately evaluated. We present a review and three cases of both entities.

El curso clínico de los pacientes con cirrosis y/o hipertensión portal puede verse complicado por dos entidades de baja prevalencia pero de elevada morbimortalidad, que corresponden al síndrome hepatopulmonar (SHP) y la hipertensión portopulmonar (HPP). Ambas se presentan a consecuencia de un déficit en la depuración hepática de diversos mediadores vasculares, provocando en el territorio pulmonar una vasodilatación en el SHP y una vasoconstricción con remodelación vascular en la HPP. Si bien estas entidades comparten algunos aspectos clínicos, resulta útil en su diferenciación la presencia de platipnea y ortodeoxia y el hallazgo ecocardiográfico de un shunt extracardíaco e intrapulmonar en el SHP, o de hipertensión pulmonar en HPP. Hasta el momento la única terapia efectiva para ambas entidades es el trasplante hepático, cuya indicación exige una evaluación rigurosa y oportuna. Se presenta una revisión y tres casos clínicos de ambas entidades.

Hipertensión portal: aspectos fisiopatológicos y clínicos / Portal hypertension: physiopathology and clinical aspects

En cirróticos, la hemorragia por varices (HV) esófagogástricas constituye la etapa final de una serie de eventos que tienen como origen el incremento de la presión portal, seguido del desarrollo y dilatación progresiva de varices hasta finalmente sangrar. La evaluación endoscópica constituye una práctica clínica rutinaria en estos pacientes y la profilaxis de HV debe realizarse en sujetos con varices grandes. Los B-bloqueadores son el tratamiento de elección, mientras que la ligadura elástica de las varices es una alternativa para pacientes intolerantes o con contraindicación a B-bloqueadores. La HV activa puede tratarse con fármacos vasoactivos y/o endoscópicamente. Aquellos que superan el episodio agudo deben recibir tratamiento preventivo del resangrado; los B-bloqueadores (asociados o no a nitratos) y la ligadura elástica (con o sin B-bloqueadores) son los de elección. En los que continúan sangrando, debe consíderarse el TIPS (Shunt porto-sístémico íntrahepático) como tratamiento de rescate.

In patients with cirrhosis, variceal bleeding is the last step of a chain that is initiated by an increased portal pressure, followed by the development and progressíve dilatation of varices until they finally bleed. Endoscopic screening for varices should be part of routine clinical practice and prophylactic treatment should be given to those patients with large varices. Non selective B-blockers remain first-line therapy in primary prophylaxis; endoscopic band ligation can be offered for patients who cannot tolerate or have contraindications to B-blockers. Treatment of acute variceal bleeding include both pharmacologic therapy (vasoactive drugs) and endoscopic treatment (combined or not with drugs). Patients surviving an episode of variceal bleeding should enter to a therapeutic program to prevent rebleeding; B-blockers (with or whithout nitrates) and endoscopic band ligation (with or without B-blockers) are the first-line options. Patients who continue to rebleed should be treated with TIPS (transjugular íntrahepatic portosystemic shunt).