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.

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.

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.

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.

What's new in portal hypertension?

Portal hypertension is defined as increased pressure in the portal venous system. The most common cause of portal hypertension is cirrhosis. In this setting, there is an increase in intrahepatic resistance leading to an increase in portal pressure. By increasing portal blood flow, splanchnic vasodilation further aggravates portal hypertension. New pathogenic pathways are being established which might result in new therapeutic strategies. The presence of varices at endoscopy and/or other abdominal portosystemic collaterals confirms the diagnosis of portal hypertension. The role of non-invasive and imaging tests in the diagnosis and prognosis of portal hypertension has been clarified. Non-selective beta-blockers decrease both the risk of variceal haemorrhage and hepatic decompensation. Terlipressin, somatostatin or octreotide, in combination with early endoscopic therapy, are recommended for the treatment of acute variceal haemorrhage. Early Transjugular intrahepatic portosystemic shunt (TIPS) is effective as salvage therapy in acute variceal bleeding in selected patients and prevents rebleeding more effectively than endoscopic and medical therapy resulting in an increased survival.

Liver disease in pregnancy: Medical aspects and their implications for mother and child.

Liver disease during pregnancy is more common than expected and may require specialized intervention. It is important to determine if changes in liver physiology may develop into liver disease, to assure early diagnosis. For adequate surveillance of mother-fetus health outcome, liver disease during pregnancy might require intervention from a hepatologist. Liver diseases have a prevalence of at least 3% of all pregnancies in developed countries, and they are classified into two main categories: related to pregnancy; and those non- related that are present de novo or are preexisting chronic liver diseases. In this review we describe and discuss the main characteristics of those liver diseases associated with pregnancy and only some frequent pre-existing and co-incidental in pregnancy are considered. In addition to the literature review, we compiled the data of liver disease occurring during pregnancies attended at the National Institute of Perinatology in Mexico City in a three-year period. In our tertiary referral women hospital, liver disease was present in 11.24 % of all pregnancies. Associated liver disease was found in 10.8% of all pregnancies, mainly those related to pre-eclampsia (9.9% of pregnancies). Only 0.56% was due to liver disease that was co-incidental or preexisting; the acute or chronic hepatitis C virus was the most frequent in this group (0.12%). When managing pregnancy in referral hospitals in Latin America, it is important to discard liver alterations early for adequate follow up of the disease and to prevent adverse consequences for the mother and child.

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/).

Hepatic Hydrothorax.

Hepatic hydrothorax (HH) is a pleural effusion that develops in a patient with cirrhosis and portal hypertension in the absence of cardiopulmonary disease. Although the development of HH remains incompletely understood, the most acceptable explanation is that the pleural effusion is a result of a direct passage of ascitic fluid into the pleural cavity through a defect in the diaphragm due to the raised abdominal pressure and the negative pressure within the pleural space. Patients with HH can be asymptomatic or present with pulmonary symptoms such as shortness of breath, cough, hypoxemia, or respiratory failure associated with large pleural effusions. The diagnosis is established clinically by finding a serous transudate after exclusion of cardiopulmonary disease and is confirmed by radionuclide imaging demonstrating communication between the peritoneal and pleural spaces when necessary. Spontaneous bacterial empyema is serious complication of HH, which manifest by increased pleural fluid neutrophils or a positive bacterial culture and will require antibiotic therapy. The mainstay of therapy of HH is sodium restriction and administration of diuretics. When medical therapy fails, the only definitive treatment is liver transplantation. Therapeutic thoracentesis, indwelling tunneled pleural catheters, transjugular intrahepatic portosystemic shunt and thoracoscopic repair of diaphragmatic defects with pleural sclerosis can provide symptomatic relief, but the morbidity and mortality is high in these extremely ill patients.

CONGENITAL HEPATIC FIBROSIS AND OBLITERATIVE PORTAL VENOPATHY WITHOUT PORTAL HYPERTENSION - A REVIEW OF LITERATURE BASED ON AN ASYMPTOMATIC CASE.

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.

[Portopulmonary hypertension: Updated review]. / Hipertensión portopulmonar: Revisión actualizada.

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.

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

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.

Schistosomal portal hypertension: Randomized trial comparing endoscopic therapy alone or preceded by esophagogastric devascularization and splenectomy.

UNLABELLED:  Background. Upper gastrointestinal bleeding is a major cause of morbidity and mortality in patients with portal hypertension secondary to schistosomiasis mansoni. AIM: To evaluate the efficacy of combined surgery and sclerotherapy versus endoscopic treatment alone in the prophylaxis of esophageal variceal rebleeding due to portal hypertension in schistosomiasis. MATERIAL AND METHODS: During a two-years period consecutive patients with schistosomiasis and a recent bleeding history were evaluated for prospective randomization. Absolute exclusion criteria were alcoholism or other liver diseases, whereas platelet count < 50,000/mm3, INR > 1.5 or presence of gastric varices were relative exclusion criteria. By random allocation 25 (group A) have received endoscopic sclerotherapy for esophageal varices alone and 22 (group B) combined treatment: esophagogastric devascularization with splenectomy followed by sclerotherapy. Interim analysis at 24 months has shown significant statistical differences between the groups and the randomization was halted. RESULTS: Mean age was 38.9 ± 15.4 years and 58.46% were male. Mean follow-up was 38.6 ± 20.1 months. Endoscopic comparison of the size of esophageal varices before and after treatment did not show significant differences among the two groups. Treatment efficacy was assessed by the rate of recurrent esophageal variceal bleeding, that was more common in group A- 9/25 patients (36.0%) vs. 2/22 (9.0%) in group B (p = 0.029). Other complications were odynophagia, dysphagia and esophageal ulcer in group A and ascites and portal vein thrombosis in the surgical group. CONCLUSION: In portal hypertension due to schistosomiasis, combined surgical and endoscopic treatment was more effective for the prevention of recurrent esophageal variceal bleeding.

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.

Left Ventricular Dilation and Pulmonary Vasodilatation after Surgical Shunt for Treatment of Pre-Sinusoidal Portal Hypertension.

OBJECTIVE: The aim of this study was to prospectively investigate the long-term cardiovascular and pulmonary hemodynamic effects of surgical shunt for treatment of portal hypertension (PH) due to Schistosomiasis mansoni. LOCATION: The University of São Paulo Medical School, Brazil; Public Practice. METHODS: Hemodynamic evaluation was performed with transesophageal Doppler and contrast-enhanced echocardiography (ECHO) on twenty-eight participants with schistosomal portal hypertension. Participants were divided into two groups according to the surgical procedure used to treat their schistosomal portal hypertension within the last two years: group 1-distal splenorenal shunt (DSRS, n = 13) and group 2-esophagogastric devascularization and splenectomy (EGDS, n = 15). RESULTS: The cardiac output (5.08 ± 0.91 L/min) and systolic volume (60.1 ± 5.6 ml) were increased (p = 0.001) in the DSRS group. DSRS participants had a significant increase (p < 0.0001) in their left ventricular end-systolic and end-diastolic diameters as well as in their left ventricular end-diastolic and end-systolic volumes (p < 0.001) compared with the preoperative period. No statistically significant difference was found in the patients who underwent EGDS. ECHO revealed intrapulmonary vasodilatation (IPV) in 18 participants (64%), 9 DSRS and 9 EGDS (p > 0.05). CONCLUSIONS: The late increase in the cardiac output, stroke volume and left ventricular diameters demonstrated left ventricular dilatation after a distal splenorenal shunt. ECHO revealed a greater prevalence for IPV in patients with schistosomiasis than has previously been described in patients with PH from liver cirrhosis.

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...

Clinical, radiologic, and endoscopic characteristics upon diagnosis of patients with prehepatic portal hypertension at the Instituto Nacional de Pediatría from 2001 to 2011.

BACKGROUND: Prehepatic portal hypertension in children can be asymptomatic for many years. Once diagnosed, the therapeutic measures (pharmacologic, endoscopic, and surgical) are conditioned by the specific characteristics of each patient. In Mexico, there are no recorded data on the incidence of the disease and patient characteristics. AIMS: To determine the main clinical, radiologic, and endoscopic characteristics upon diagnosis of these patients at the Instituto Nacional de Pediatría within the time frame of January 2001 and December 2011. METHODS: A cross-sectional, retrolective, descriptive, and observational study was conducted in which all the medical records of the patients with portal hypertension diagnosis were reviewed. RESULTS: There was a greater prevalence of prehepatic etiology (32/52) (61.5%) in the portal hypertension cases reviewed. Males (62.5%) predominated and 11 of the 32 patients were under 4 years of age. The primary reason for medical consultation was upper digestive tract bleeding with anemia (71.9%) and the main pathology was cavernomatous degeneration of the portal vein (65.6%). Splenoportography was carried out on 17 of the 32 patients. A total of 65.5% of the patients received the combination therapy of propranolol and a proton pump inhibitor. Initial endoscopy revealed esophageal varices in 96.9% of the patients, 12 of whom presented with gastroesophageal varices. Congestive gastropathy was found in 75% of the patients. The varices were ligated in 8 cases, sclerotherapy for esophageal varices was carried out in 5 cases (15.6%), and sclerotherapy for gastric varices was performed in 2 patients. Seventeen patients (53.1%) underwent portosystemic diversion: 10 of the procedures employed a mesocaval shunt and 7 a splenorenal shunt. Nine patients (28.1%) underwent total splenectomy. CONCLUSIONS: The primary cause of the disease was cavernomatous degeneration of the portal vein; it was predominant in males and the first symptom was variceal bleeding.

[Portal hypertension in pregnancy: report of a case]. / Hipertensiónportal en el embarazo: presentaciónde un 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.

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.