Budd-Chiari syndrome with hepatopulmonary syndrome: a case report and literature review / 中华儿科杂志

Objective: To summarize the clinical features and prognosis of Budd-Chiari syndrome with hepatopulmonary syndrome (HPS) in children. Methods: The clinical data of a child who had Budd-Chiari syndrome with HPS treated at the Department of Pediatrics of the First Affiliated Hospital of Zhengzhou University in December 2016 was analyzed retrospectively. Taking "Budd-Chiari syndrome" and "hepatopulmonary syndrome" in Chinese or English as the keywords, literature was searched at CNKI, Wanfang, China Biomedical Literature Database and PubMed up to July 2023. Combined with this case, the clinical characteristics, diagnosis, treatment and prognosis of Budd-Chiari syndrome with HPS in children under the age of 18 were summarized. Results: A 13-year-old boy, presented with cyanosis and chest tightness after activities for 6 months, and yellow staining of the skin for 1 week. Physical examination at admission not only found mild yellow staining of the skin and sclera, but also found cyanosis of the lips, periocular skin, and extremities. Laboratory examination showed abnormal liver function with total bilirubin 53 μmol/L, direct bilirubin 14 μmol/L, and indirect bilirubin 39 μmol/L, and abnormal blood gas analysis with the partial pressure of oxygen of 54 mmHg (1 mmHg=0.133 kPa), the partial pressure of carbon dioxide of 31 mmHg, and the alveolar-arterial oxygen gradient of 57 mmHg. Hepatic vein-type Budd-Chiari syndrome, cirrhosis, and portal hypertension were indicated by abdominal CT venography. Contrast-enhanced transthoracic echocardiography (CE-TTE) was positive. After symptomatic and supportive treatment, this patient was discharged and received oxygen therapy outside the hospital. At follow-up until March 2023, there was no significant improvement in hypoxemia, accompanied by limited daily activities. Based on the literature, there were 3 reports in English while none in Chinese, 3 cases were reported. Among a total of 4 children, the chief complaints were dyspnea, cyanosis, or hypoxemia in 3 cases, and unknown in 1 case. There were 2 cases diagnosed with Budd-Chiari syndrome with HPS at the same time due to respiratory symptoms, and 2 cases developed HPS 1.5 years and 8.0 years after the diagnosis of Budd-Chiari syndrome respectively. CE-TTE was positive in 2 cases and pulmonary perfusion imaging was positive in 2 cases. Liver transplantation was performed in 2 cases and their respiratory function recovered well; 1 case received oxygen therapy, with no improvement in hypoxemia; 1 case was waiting for liver transplantation. Conclusions: The onset of Budd-Chiari syndrome with HPS is insidious. The most common clinical manifestations are dyspnea and cyanosis. It can reduce misdiagnosis to confirm intrapulmonary vascular dilatations with CE-TTE at an early stage. Liver transplantation is helpful in improving the prognosis.

The hepatopulmonary syndrome / Síndrome hepatopulmonar

INTRODUCTION: The hepatopulmonary syndrome has been acknowledged as an important vascular complication in lungs developing systemic hypoxemia in patients with cirrhosis and portal hypertension. Is formed by arterial oxygenation abnormalities induced from intrapulmonary vascular dilatations with liver disease. It is present in 4-32% of patients with cirrhosis. It increases mortality in the setting of cirrhosis and may influence the frequency and severity. Initially the hypoxemia responds to low-flow supplemental oxygen, but over time, the need for oxygen supplementation is necessary. The liver transplantation is the only effective therapeutic option for its resolution. AIM: To update clinical manifestation, diagnosis and treatment of this entity. METHOD: A literature review was performed on management of hepatopulmonary syndrome. The electronic search was held of the Medline-PubMed, in English crossing the headings "hepatopulmonary syndrome", "liver transplantation" and "surgery". The search was completed in September 2013. RESULTS: Hepatopulmonary syndrome is classically defined by a widened alveolar-arterial oxygen gradient (AaPO2) on room air (>15 mmHg, or >20 mmHg in patients >64 years of age) with or without hypoxemia resulting from intrapulmonary vasodilatation in the presence of hepatic dysfunction or portal hypertension. Clinical manifestation, diagnosis, classification, treatments and outcomes are varied. CONCLUSION: The severity of hepatopulmonary syndrome is an important survival predictor and determine the improvement, the time and risks for liver transplantation. The liver transplantation still remains the only effective therapeutic. .

INTRODUÇÃO: A síndrome hepatopulmonar (SHP) tem sido reconhecida como importante complicação vascular nos pulmões desenvolvendo hipoxemia sistêmica em pacientes com cirrose e hipertensão portal. Ela é formada pela presença de anormalidade na oxigenação arterial induzida por dilatações vasculares intrapulmonares com a doença hepática e está presente em 4-32% dos pacientes com cirrose. Aumenta a mortalidade no cenário de cirrose podendo influenciar na frequência e gravidade. Inicialmente, a hipoxemia nos pacientes com esta síndrome responde à suplementação com baixo fluxo de oxigênio; mas, ao longo do tempo, há necessidade de maior suplementação de oxigênio. O transplante de fígado é a única opção terapêutica eficaz para a resolução. OBJETIVO: Atualizar conhecimentos sobre a síndrome hepatopulmonar, suas manifestações clínicas, diagnóstico e tratamento. MÉTODO: Foi realizada revisão da literatura com busca eletrônica realizada no Medline-PubMed em inglês cruzando-se os descritores "síndrome hepatopulmonar", "transplante de fígado" e "cirurgia". A pesquisa foi concluída em setembro de 2013. RESULTADOS: Síndrome hepatopulmonar é classicamente definida por alteração no gradiente alvéolo-arterial (AaPO2) em ar ambiente (>15 mmHg, ou >20 mmHg em pacientes >64 anos de idade) com ou sem hipoxemia resultante da vasodilatação intrapulmonar na presença de disfunção hepática ou hipertensão portal. As manifestações clínicas, diagnóstico, classificação e tratamento são variados. CONCLUSÃO: A avaliação da gravidade da síndrome hepatopulmonar é importante meio de predição ...

Pulmonary vascular complications of chronic liver disease: pathophysiology, imaging, and treatment

To review the pathogenesis of pulmonary vascular complications of liver disease, we discuss their clinical implications, and therapeutic considerations, with emphasis on potential reversibility of the hepatopulmonary syndrome after liver transplantation. In this review, we also discuss the role of imaging in pulmonary vascular complications associated with liver disease

Hepatopulmonary syndrome: an update / Síndrome hepatopulmonar: atualização

Hepatopulmonary syndrome (HPS) is a clinical threesome composed of liver disease, intrapulmonary vascular dilatation (IPVD) and arterial gas abnormalities. Its occurrence has been described in up to 32 percent of cirrhotic candidates for liver transplantation. It also affects non-cirrhotic patients with portal hypertension. Its pathogenesis is not well defined, but an association of factors such as imbalance in the endothelin receptor response, pulmonary microvascular remodeling and genetic predisposition is thought to lead to IPVD. Diagnosis is based on imaging methods that identify these dilatations, such as contrast echocardiography or perfusion scintigraphy with 99mTc, as well as analysis of arterial gases to identify elevated alveolar-arterial differences in O2 or hypoxemia. There is no effective pharmacological treatment and complete resolution only occurs through liver transplantation. The importance of diagnosing HPS lies in prioritizing transplant candidates, since presence of HPS is associated with worse prognosis. The aim of this paper was to review the pathogenetic theories and current diagnostic criteria regarding HPS, and to critically analyze the prioritization of patients with HPS on the liver transplant waiting list. Searches were carried out in the Medline (Medical Literature Analysis and Retrieval System Online) via PubMed, Cochrane Library and Lilacs (Literatura Latino-Americana e do Caribe em Ciências da Saúde) databases for articles published between January 2002 and December 2007 involving adults and written either in English or in Portuguese, using the term hepatopulmonary syndrome. The studies of greatest relevance were included in the review, along with text books and articles cited in references that were obtained through the review.

A síndrome hepatopulmonar (SHP) é considerada uma tríade clínica composta de doença hepática, dilatações vasculares intrapulmonares (IPVD) e alterações de gases arteriais. Há descrição de sua ocorrência em até 32 por cento dos cirróticos candidatos ao transplante de fígado, acometendo também não cirróticos com hipertensão portal. Sua etiopatogenia não está bem definida, mas se especula que a associação de fatores como o desequilíbrio na resposta dos receptores vasculares de endotelina, o remodelamento microvascular pulmonar e a predisposição genética propiciem as IPVD. O diagnóstico baseia-se em métodos de imagem que identifiquem essas dilatações, como a ecocardiografia com contraste ou a cintilografia de perfusão com 99mTc, além da análise de gases arteriais, para identificar elevação da diferença alvéolo-arterial de O2 ou hipoxemia. Não existe tratamento medicamentoso eficaz e sua resolução completa ocorre apenas com o transplante de fígado. A importância do diagnóstico da SHP está em priorizar os candidatos ao transplante, uma vez que sua presença está associada ao pior prognóstico. O objetivo deste artigo é fazer revisão das teorias de etiopatogenia da SHP, dos seus critérios diagnósticos atuais, além de realizar análise crítica sobre a prioridade dos pacientes com a síndrome na lista de espera pelo transplante de fígado. Buscaram-se na base de dados Medline (Medical Literature Analysis and Retrieval System Online) via PubMed, Cochrane Library e Lilacs (Literatura Latino-Americana e do Caribe em Ciências da Saúde), os artigos publicados no período de janeiro de 2002 a dezembro de 2007, envolvendo adultos, escritos nos idiomas inglês ou português, que apresentassem o termo síndrome hepatopulmonar. Os estudos de maior relevância foram incluídos, além de livros-texto e artigos citados nas referências obtidas na revisão.

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.

Sindrome hepatopulmonar / Hepatopulmonary syndrome

El síndrome hepatopulmonar constituye una causa conocida de insuficiencia respiratoria en cirrosis hepática. Se define en base a la tríada de enfermedad hepática generalmente con hipertensión portal, hipoxemia arterial y vasodilatación pulmonar capilar y precapilar, que condicionan shunts de derecha a izquierda, alteraciones en la ventilación-perfusión y en la difusión. Su incidencia oscila entre el 15 y 47 por ciento, y aunque se puede presentar en pacientes con hepatopatía aguda, es una complicación característica de pacientes con cirrosis. Clinicamente existe disnea de esfuerzo, platipnea y ortodeoxia, además de cianosis, acropaquia y nevi aracniformes. Su diagnóstico se basa en el estudio de la función pulmonar y el ecocardiograma con contraste de burbujas. La gammagrafía pulmonar de perfusión con albúmina macroagregada marcada con tecnesio-99m permite la estimación de la fracción del shunt. El trasplante hepático es el único tratamiento demostradamente eficaz, excepto en aquellos pacientes con un trastorno ventilatorio más grave, por su mayor morbi-mortalidad.

The Hepatopulmonary syndrome is a know cause of respiratory failure in cirrhosis. It is a clinical triad characterized by liver disease generally with portal hypertension, arterial hypoxaemia and precapillary-capillary intrapulmonary vascular dilatation leading to right and left shunts, ventilation/perfusion defects and diffusion impairment. Its incidence is about 15 to 47 percent in patients with acute liver disease but characteristly in chronic liver disease. Shortness of breath, orthodeoxia and platypnoea, togheter with cyanosis, digital clubbing and spider naevi are common. Its diagnosis on the basis of the pulmonary gas exchange abnormality and contrast-enhanced echocardiography. The perfusion lung scanning using technetium-labelled macro-aggregatesalbumin estimate the shunt fraction. The orthotopic liver transplantation is the only efficacy treatment in patients without several gas exchange abnormality.

Síndrome hepatopulmonar: patogenia, diagnóstico e tratamento / Hepatopulmonary syndrome: pathogenesis, diagnosis and treatment

RACIONAL: A síndrome hepatopulmonar é caracterizada por tríade clínica que consiste em: 1) doença hepática e/ou hipertensão portal, 2) dilatações vasculares intra-pulmonares, e 3) anormalidades da oxigenação arterial (pressão parcial de oxigênio <70 mm Hg ou gradiente alvéolo-arterial de oxigênio >20 mm Hg). Sua freqüência varia de acordo com os métodos diagnósticos utilizados. OBJETIVOS: Fazer revisão da literatura sobre patogenia, diagnóstico e tratamento da síndrome hepatopulmonar. MÉTODOS: Levantamento no MEDLINE das publicações nacionais e internacionais mais relevantes sobre a síndrome hepatopulmonar. RESULTADOS: A prevalência da síndrome hepatopulmonar varia de 4 por cento a 17,5 por cento, a depender dos critérios diagnósticos utilizados. A doença hepática associada mais comum é a cirrose. O gradiente alvéolo-arterial de oxigênio parece ser o melhor parâmetro para avaliação das anormalidades da oxigenação arterial. Em relação à detecção das dilatações vasculares intra-pulmonares, a ecocardiografia com contraste é o método de escolha, pois é de fácil realização e pode diferenciar as comunicações intra-pulmonares das intra-cardíacas. No tratamento da síndrome hepatopulmonar, poucos relatos bem sucedidos puderam ser reproduzidos e confirmados utilizando terapia farmacológica e/ou radiologia intervencionista. Atualmente, o transplante de fígado é considerado a principal opção terapêutica desses pacientes, com resultados animadores. CONCLUSÕES: A síndrome hepatopulmonar é uma enfermidade freqüente. Exames gasométricos são necessários para o seu diagnóstico. O transplante de fígado é o tratamento de escolha para os portadores da síndrome.