Correlation between hepatopulmonary syndrome and oxygen saturation pulse oximetry in cirrhotic patients

SUMMARY Hepatopulmonary Syndrome (HPS) is a complication of cirrhosis that worsens the disease's prognosis, pre and post liver transplant. The objective of this study is to analyze the prevalence of HPS in cirrhotic patients at our service and to correlate it with oxygen saturation (SatO2) using a pulse oximeter to evaluate if this is useful as a screening test for HPS. A prospective study was conducted in patients with hepatic cirrhosis conventionally selected from 2014 to 2016. All the patients underwent an echocardiogram with microbubbles and oxygen saturation measurement by pulse oximetry. Those with intrapulmonary shunt were submitted to arterial blood gas analysis. The relationship between oxygen saturation and HPS was assessed by the multivariate model of binary logistic regression. We analyzed 77 patients, and 23.3% (18 patients) had all criteria for HPS. The relationship between HPS and SatO2 did not show statistical significance, even after the variables were adjusted for sex, age, and smoking. Oxygen saturation alone was not able to detect HPS in the sample of cirrhotic patients. More accurate methods for screening and diagnosis of the syndrome should be used.

RESUMO A Síndrome Hepatopulmonar (SHP) é uma complicação da cirrose que piora o prognóstico da doença pré e pós-transplante hepático. O objetivo do trabalho é analisar a prevalência de SHP em pacientes cirróticos de nosso serviço e correlacioná-la com a saturação de oxigênio (SatO2) pelo oxímetro de pulso, e avaliar se este seria útil como um exame de triagem no diagnóstico de SHP. Foi realizado um estudo prospectivo em pacientes portadores de cirrose hepática no período de 2014 a 2016. Todos os pacientes foram submetidos a um ecocardiogama com microbolhas e a saturação de oxigênio pela oximetria de pulso. Aqueles com shunt intrapulmonar foram submetidos a gasometria arterial. A relação entre a saturação de oxigênio e SHP foi avaliada pelo modelo multivariado de regressão logística binário. Foram analisados 77 pacientes, destes 23,3% (18 pacientes) apresentaram todos os critérios para SHP. A relação entre a SHP com a SatO2 não obteve significância estatística, mesmo após as variáveis terem sido ajustadas pelo sexo, idade e tabagismo atual ou passado. A saturação de oxigênio, de forma isolada, não foi capaz de distinguir a SHP na amostra de pacientes cirróticos em nosso estudo. Deve-se utilizar métodos mais acurados para a triagem e diagnóstico dessa síndrome.

Value of Contrast Transesophageal Echocardiography in the Detection of Intrapulmonary Vascular Dilatations in Hepatosplenic Schistosomiasis / Valor do Ecocardiograma Transesofágico com Contraste no Diagnóstico da Dilatação Vascular Intrapulmonar na Esquistossomose Hepatoesplênica

Abstract Background: Hepatopulmonary syndrome (HPS), found in cirrhotic patients, has been little studied in hepatosplenic schistosomiasis (HSS) and includes the occurrence of intrapulmonary vascular dilatations (IPVD). Contrast transesophageal echocardiography (cTEE) with microbubbles is more sensitive than contrast transthoracic echocardiography (cTTE) with microbubbles in the detection of IPVD in cirrhosis. Objective: To assess the performance of the cTEE, compared with that of cTTE, in detecting IPVD for the diagnosis of HPS in patients with HSS. Methods: cTEE and cTTE for investigation of IPVD and laboratory tests were performed in 22 patients with HSS. Agitated saline solution was injected in peripheral vein during the cTEE and cTTE procedures. Late appearance of the microbubbles in the left chambers indicated the presence of IPVD. Results of the two methods were compared by the Student's t-test and the chi-square test (p < 0.05). Results: cTEE was performed in all patients without complications. Three patients were excluded due to the presence of patent foramen ovale (PFO). The presence of IPVD was confirmed in 13 (68%) of 19 patients according to the cTEE and in only six (32%, p < 0.01) according to the cTTE. No significant differences in clinical or laboratory data were found between the groups with and without IPVD, including the alveolar-arterial gradient. The diagnosis of HPS (presence of IPVD with changes in the arterial blood gas analysis) was made in five patients by the cTEE and in only one by the cTTE (p = 0.09). Conclusion: In HSS patients, cTEE was safe and superior to cTTE in detecting IPVD and allowed the exclusion of PFO.

Resumo Fundamento: A síndrome hepatopulmonar (SHP), presente em pacientes cirróticos, é pouco estudada na esquistossomose hepatoesplênica (EHE) e inclui a ocorrência de dilatações vasculares intrapulmonares (DVP). O ecocardiograma transesofágico com contraste (ETEc) de microbolhas é mais sensível que o ecocardiograma transtorácico com contraste (ETTc) de microbolhas na identificação de DVP na cirrose. Objetivo: Avaliar o desempenho do ETEc comparado ao ETTc na identificação de DVP para diagnóstico de SHP em pacientes com EHE. Métodos: Incluímos 22 pacientes com EHE submetidos a ETEc e ETTc para pesquisa de DVP, além de exames laboratoriais. Os ETEc e ETTc foram realizados empregando-se solução salina agitada, injetada em veia periférica. A visualização tardia das microbolhas em câmaras esquerdas indicava presença de DVP. Os resultados foram comparados entre os dois métodos pelos testes t de Stu dent e qui-quadrado (significância p < 0,05). Resultados: Todos os 22 pacientes realizaram ETEc sem intercorrências. Foram excluídos três pela presença de forame oval patente (FOP), e a análise final foi realizada nos outros 19. A DVP esteve presente ao ETEc em 13 pacientes (68%) e em apenas seis ao ETTc (32%, p < 0,01). Não houve diferenças significativas nos dados clínicos e laboratoriais entre os grupos com e sem DVP, incluindo a diferença alveoloarterial de oxigênio. O diagnóstico de SHP (presença de DVP com alterações gasométricas) ocorreu em cinco pacientes pelo ETEc e em apenas um pelo ETTc (p = 0,09). Conclusão: Em pacientes com EHE, o ETEc foi seguro e superior ao ETTc na detecção de DVP não identificada ao ETTc, o que possibilitou adicionalmente excluir FOP.

Schistosomiasis and hepatopulmonary syndrome: the role of concomitant liver cirrhosis

BACKGROUND Hepatopulmonary syndrome (HPS) is defined as an oxygenation defect induced by intrapulmonary vasodilation in patients with liver disease or portal hypertension. It is investigated in patients with liver cirrhosis and less frequently in those with portal hypertension without liver cirrhosis, as may occur in hepatosplenic schistosomiasis (HSS). OBJECTIVES To investigate the prevalence of HPS in patients with HSS, and to determine whether the occurrence of HPS is influenced by concomitant cirrhosis. METHODS We evaluated patients with HSS with or without concomitant liver cirrhosis. All patients underwent laboratory testing, ultrasound, endoscopy, contrast echocardiography, and arterial blood gas analysis. FINDINGS Of the 121 patients with HSS, 64 were also diagnosed with liver cirrhosis. HPS was diagnosed in 42 patients (35%) and was more frequent among patients with concomitant liver cirrhosis than in those without cirrhosis (42% vs. 26%), but the difference was not significant (p = 0.069). HPS was more common in those with spider naevi, Child-Pugh classes B or C and high model for end stage liver disease (MELD) scores (p < 0.05 each). MAIN CONCLUSIONS The prevalence of HPS was 35% in this study. The occurrence of liver cirrhosis concomitantly with HSS may have influenced the frequency of patients presenting with HPS.

Características clínicas y laboratoriales de pacientes hospitalizados con hipertensión portal y complicaciones vasculares pulmonares en el Instituto Nacional de Salud del Niño / Clinical and laboratory characteristics of patients with pulmonary hypertension and pulmonary vascular complications hospitalized at the Instituto Nacional de Salud del Niño

El síndrome hepatopulmonar (SHP) y la hipertensión portopulmonar (HTPP) son distintas complicaciones vasculares pulmonares de la hipertensión portal (HTP) y se asocian con una mayor morbilidad y mortalidad. Objetivos: Describir las Características Clínicas y Laboratoriales de los pacientes con HTP y complicaciones vasculares pulmonares hospitalizados en el Instituto Nacional de Salud del Niño. Materiales y métodos: se incluyeron los pacientes con HTP hospitalizados desde enero del 2012 hasta junio del 2013 y que durante su evolución cursaron con SHP o HTPP. Para el análisis se les dividió en un primer grupo de pacientes con cirrosis hepática y un segundo grupo con obstrucción extra hepática de vena porta. Resultados: De 22 pacientes con HTP el 45,5% fueron varones y el rango de edad fue entre 1 mes y 17 años. La etiología en el grupo de cirrosis (n=14) fue: hepatitis autoinmune (35,7%), cirrosis criptogénica (35,7%), error innato del metabolismo (14,3%), hepatitis viral crónica por virus C (7,15%) y atresia de vías biliares extra hepática (7,15%). Las complicaciones vasculares pulmonares, se presentaron más frecuentemente en los pacientes con cirrosis hepática (1 caso de síndrome hepatopulmonar y un caso de hipertensión portopulmonar). En ellos se encontró más frecuentemente disnea, astenia, edema, desnutrición, ascitis, hiperesplenismo y hemorragia digestiva por várices esofágicas, además de valores elevados de ALT, fosfatasa alcalina y menores niveles de albúmina sérica. Conclusiones: En niños con HTP, las complicaciones vasculares pulmonares son muy infrecuentes. En la evaluación de estos pacientes debería incluirse la oximetría de pulso para detectar hipoxemia y posteriormente, de ser necesario una ecocardiografía Doppler y de contraste. Ante el hallazgo de hipertensión sistólica pulmonar es necesario realizar un cateterismo cardiaco derecho.

The hepatopulmonary syndrome (HPS) and portopulmonary hypertension (PPHN) are distinct pulmonary vascular complications of portal hypertension (PHT) and are associated with increased morbidity and mortality. Objectives: To describe the clinical and laboratory characteristics of patients with pulmonary hypertension and pulmonary vascular complications hospitalized at the Instituto Nacional de Salud del Niño. Materials and methods: We included patients with HTP hospitalized from January 2012 to June 2013 and that during its evolution progressed with SHP or HTPP. For analysis, they were divided into a first group of patients with liver cirrhosis and a second group with extrahepatic portal vein obstruction. Results: Of 22 patients with HPT 45.5% were male and the age range was between 1 month and 17 years. The etiology in the group of cirrhosis (n=14) was: autoimmune hepatitis (35.7%), cryptogenic cirrhosis (35.7%), inborn error of metabolism (14.3%), chronic viral hepatitis C (7.15%) virus and atresia extra-hepatic bile ducts (7.15%). Pulmonary vascular complications more frequently occurred in patients with liver cirrhosis (1 case of HPS and a case of PPHTN). They most often dyspnea, asthenia, edema, malnutrition, ascites, hypersplenism and gastrointestinal bleeding from esophageal varices was found. Also, they had elevated ALT values, alkaline phosphatase and serum albumin values decreased. Conclusions: In children with pulmonary hypertension, pulmonary vascular complications are rare. In the evaluation of these patients pulse oximetry should be included to detect hypoxemia and subsequently a Doppler echocardiography and contrast echocardiography necessary. Dueto the finding of systolic pulmonary hypertension it is necessary to perform right heart catheterization.

Síndrome hepatopulmonar: poco común, pero mortal / Hepatopulmonary syndrome: uncommon, but deadly

Patients with cirrhosis may present with portal hypertension (PHT), which can lead to various complications. The most common areas cites, variceal bleeding, and hepatic encephalopathy. However, there is another entity o flow prevalence but high morbidity and mortality: the hepato pulmonary syndrome (HPS). We report the case of a 25 year-old woman with cirrhosis secondary to autoimmunehepatitis. She was admitted with respiratory symptoms suggestive of viral etiology, but evolved unfavorably. Various diagnoses of cardiovascular and respiratory conditions were successively ruled out, persisting with severe hypoxemia. Concomitantly, she developed progressive pain in the left hypochondrium area, and imaging studies show edsplenic and perisplenic form of manifestation of PHT. In context fHTP and hypoxemia, the diagnosis of HPS vs portopulmonary syndrome was considered, with the bubble test echocardiogramas a key study for such dilemma.

Los pacientes con daño hepático crónico (DHC) pueden cursar con hipertensión portal (HTP), que puede generar diversas complicaciones. Las más frecuentes son la ascitis, hemorragia variceal y encefalopatía hepática. Sin embargo, existe otra entidad de baja prevalencia, pero elevada morbimortalidad: el síndrome hepatopulmonar (SHP). Reportamos el caso de una mujer de 25 años con DHC secundario a hepatitis autoinmune. Ella ingresó con un cuadro sugerente de infección respiratoria alta de etiología viral, pero evolucionó tórpidamente. Se descartaron sucesivamente diversos diagnósticos de la esfera cardiovascular y respiratoria, persistiendo con hipoxemia grave. En forma concomitante desarrolló dolor en hipocondrio izquierdo de carácter progresivo, y las imágenes evidenciaron manifestaciones de HTP de predominio esplénico y periesplénico. En contexto de HTP e hipoxemia se consideró el diagnóstico de síndrome hepatopulmonar vs síndrome porto pulmonar, siendo el ecocardiograma con test de burbujas un estudio clave para el diagnóstico definitivo.

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 ...

Improved severe hepatopulmonary syndrome after liver transplantation in an adolescent with end-stage liver disease secondary to biliary atresia

Hepatopulmonary syndrome (HPS) is a serious complication of end-stage liver disease, which is characterized by hypoxia, intrapulmonary vascular dilatation, and liver cirrhosis. Liver transplantation (LT) is the only curative treatment modality for patients with HPS. However, morbidity and mortality after LT, especially in cases of severe HPS, remain high. This case report describes a patient with typical findings of an extracardiac pulmonary arteriovenous shunt on contrast-enhanced transesophageal echocardiography (TEE), and clubbing fingers, who had complete correction of HPS by deceased donor LT. The patient was a 16-year-old female who was born with biliary atresia and underwent porto-enterostomy on the 55th day after birth. She had been suffered from progressive liver failure with dyspnea, clubbing fingers, and cyanosis. Preoperative arterial blood gas analysis revealed severe hypoxia (arterial O2 tension of 54.5 mmHg and O2 saturation of 84.2%). Contrast-enhanced TEE revealed an extracardiac right-to-left shunt, which suggested an intrapulmonary arteriovenous shunt. The patient recovered successfully after LT, not only with respect to physical parameters but also for pychosocial activity, including school performance, during the 30-month follow-up period.

Síndrome hepatopulmonar en pediatría: presentación de un caso y revisión de la literatura / Hepatopulmonary syndrome in children: a case report and literature review

Hepatopulmonary syndrome is a rare disease that affects patients of any age with acute or chronic liver disease. The prevalence and its outcome in children are unknown. Clinically are common shortness of breath, orthodeoxia and platypnoea, together with cyanosis, digital clubbing and spider naevi. Its diagnosis is based in a clinical triad characterized by liver disease with or whithout portal hypertension, hypoxemia and intrapulmonary vascular dilatation leading to right and left shunts, demonstrated by echocardiography with contrast, the perfusion lung scanning using technetium-labelled macro-aggregates albumin estimate the shunt fraction. Pulmonary angiography is useful to demonstrate macroscopic arteriovenous communications. The orthotopic liver transplantation is the only efficacy treatment in patients without several gas exchange abnormality.

El síndrome hepatopulmonar es una enfermedad poco frecuente que afecta a pacientes de cualquier edad con enfermedad hepática aguda o crónica. La prevalencia y la evolución en pediatría son desconocidas. Clínicamente es común encontrar disnea de esfuerzo, platipnea y ortodeoxia, además de cianosis, acropaquia y nevi aracniformes. El diagnóstico está basado en una tríada clínica caracterizada por una enfermedad hepática con o sin hipertensión portal, hipoxemia y vasodilatación intrapulmonar que conduce a un shunt de derecha a izquierda, demostrado mediante ecocardiografía con contraste y cintigrafía de perfusión pulmonar con albúmina macroagregada marcada contecnesio-99m que permite la estimación de la fracción del shunt. La angiografía pulmonar es útil para demostrar comunicaciones arteriovenosas macroscópicas. El trasplante hepático es el único tratamiento demostradamente eficaz, excepto en aquellos pacientes con un trastorno ventilatorio grave, por su mayor morbimortalidad.

Are the spider nngiomas skin markers of hepatopulmonary syndrome? / Sao as aranhas vasculares marcadores cutaneos da sindrome hepatopulmonar?

Context Hepatopathies can significantly influence both veins and arteries, these changes may cause some cutaneous stigmas, such as spider angioma (SA) and some systemic vascular changes, such as those observed in hepatopulmonary syndrome (HPS). Based on this common pathophysiological root we can assume that the SA can be skin markers of HPS. Objective The objective of this study is to assess whether there is a relationship between the presence of SA and HPS. Methods Records of 40 patients with liver cirrhosis who underwent contrast echocardiography were evaluated, in which we researched the description of SA, physical examination, and other clinical and laboratory data. For diagnosis of HPS we use these signs of the disease: presence of liver disease (cirrhosis in the case), abnormalities in gas exchange by arterial blood gases, and evidence of pulmonary vasodilations by the contrast echocardiography. Results The SA were found in 21/40 (52.5%) patients and hepatopulmonary syndrome in 9/40 (22.5%). The HPS was observed in 8/21 (38.1%) of patients with SA and 1/19 (5.3%) patients were without this sign (P<0.01). We found no statistically significant difference between the SA and the presence of HPS with sex or age. Patients with SA had a higher hypoxemia [PaO2 84.8 ± 11.5 mmHg and 19.8 ± 14.7 mmHg alveolar-arterial gradient of oxygen (AAG)] than those without SA (PaO2 90.8 ± 10.7 mmHg and 10.9 ± 11.7 AAG mmHg) (P<0.05). Conclusion Our findings show a correlation between the presence of SA and HPS, suggesting that the SA may be cutaneous markers of HPS. .

Contexto As hepatopatias podem influenciar de forma considerável tanto as veias quanto as artérias, dessas alterações podem surgir alguns estigmas cutâneos, como as aranhas vasculares (AV) e algumas alterações vasculares sistêmicas, como as observadas na Síndrome Hepatopulmonar (SHP). Baseados nessa possível raiz fisiopatogênica comum, podemos supor que as AV sejam marcadores cutâneos da SHP. Objetivo Avaliar se há relação entre a presença das AV e a SHP. Métodos Foram avaliados os prontuários de 40 pacientes com cirrose hepática submetidos a ecocardiografia com contraste (ECC), nos quais pesquisamos a descrição de AV, no exame físico, e outros dados clínicos e laboratoriais. Para diagnóstico da SHP utilizamos os seguintes critério: presença de hepatopatia (no caso cirrose), de anormalidades nas trocas gasosas pela gasometria arterial, e evidências de vasodilatações pulmonares pela ecocardiografia com contraste. Resultados As AV foram encontradas em 21/40 (52,5%) pacientes e a síndrome hepatopulmonar em 9/40 (22,5%). A SHP foi observada em 8/21 (38,1%) dos pacientes com AV e em 1/19 (5,3%) dos pacientes sem esse sinal (P<0,01). Não encontramos diferença estatisticamente significativa entre a presença das AV e da SHP com sexo ou faixa etária. Pacientes com AV apresentaram maior hipoxemia (PaO2 84,8 ± 11,5 mmHg e GAA 19,8 ± 14,7 mmHg) que os sem AV (PaO2 90,8 ± 10,7 mmHg e GAA 10,9 ± 11,7 mmHg) (P<0,05). Conclusão Nossos achados mostram correlação entre a presença das AV e a SHP, sugerindo que as AV possam ser marcadores cutâneos da SHP. .

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

Ocorrência de síndrome hepatopulmonar em pacientes cirróticos candidatos a transplante de fígado / Occurrence of hepatopulmonary syndrome in patients with cirrhosis who are candidates for liver transplantation

OBJETIVO: Verificar a ocorrência da síndrome hepatopulmonar (SHP) em pacientes cirróticos candidatos a transplante de fígado; comparar as características demográficas, clínicas, laboratoriais e espirométricas, resultados de ecocardiografia, análise de gases arteriais e da gravidade da doença hepática nos pacientes com e sem SHP; e descrever a ocorrência de SHP no subgrupo de pacientes com cirrose associada à esquistossomose mansônica (doença hepática mista). MÉTODOS: Entre janeiro e novembro de 2007, foram avaliados 44 pacientes inscritos no Ambulatório de Transplante Hepático do Hospital das Clínicas da Universidade Federal de Pernambuco, em Recife (PE). Os critérios diagnósticos para SHP foram a presença de dilatações vasculares intrapulmonares, identificadas por ecocardiografia transtorácica, assim como diferença alveoloarterial de oxigênio > 15 mmHg ou PaO2 < 80 mmHg. RESULTADOS: A idade média foi 52 anos, e 31 pacientes (70 por cento) eram do sexo masculino. A causa mais frequente de cirrose foi uso de etanol. A esquistossomose esteve presente em 28 pacientes (64 por cento). Dos 44 pacientes, 20 (45,5 por cento) foram diagnosticados com SHP. Não foram observadas diferenças significativas em relação às características estudadas. No subgrupo de pacientes com cirrose associada à esquistossomose, 10/28 (35,7 por cento) receberam o diagnóstico de SHP. CONCLUSÕES: A SHP apresentou elevada prevalência nesta população estudada, não sendo observadas associações entre a sua ocorrência e as variáveis analisadas.

OBJECTIVE: To determine the occurrence of hepatopulmonary syndrome (HPS) in patients with cirrhosis who are candidates for liver transplantation; to compare demographic, clinical, laboratory, and spirometric characteristics, as well as echocardiography results, arterial blood gas analysis, and severity of liver disease between the groups of patients with and without HPS; and to describe the occurrence of HPS in the subgroup of patients with cirrhosis and schistosomiasis mansoni (mixed liver disease). METHODS: Between January and November of 2007, we evaluated 44 patients under treatment at the Liver Transplant Outpatient Clinic of the Federal University of Pernambuco Hospital das Clínicas, in the city of Recife, Brazil. The diagnostic criteria for HPS were intrapulmonary vascular dilatation, identified by transthoracic echocardiography, and an alveolar-arterial oxygen tension difference > 15 mmHg or a PaO2 < 80 mmHg. RESULTS: The mean age of the patients was 52 years, and 31 patients (70 percent) were male. The most common cause of cirrhosis was alcohol use. Schistosomiasis was present in 28 patients (64 percent). Of the 44 patients, 20 (45.5 percent) were diagnosed with HPS. No significant differences were found between those patients and the patients without HPS in terms of any of the characteristics studied. Of the 28 patients with cirrhosis and schistosomiasis, 10 (35.7 percent) were diagnosed with HPS. CONCLUSIONS: In the population studied, HPS was highly prevalent and did not correlate with any of the variables analyzed.

Pulmonary Artery Embolotherapy in a Patient with Type I Hepatopulmonary Syndrome after Liver Transplantation

Although liver transplantation (LT) is the only effective treatment option for hepatopulmonary syndrome (HPS), the post-LT morbidity and mortality have been high for patients with severe HPS. We performed post-LT embolotherapy in a 10-year-old boy who had severe type I HPS preoperatively, but he failed to recover early from his hypoxemic symptoms after an LT. Multiple embolizations were then successfully performed on the major branches that formed the abnormal vascular structures. After the embolotherapy, the patient had symptomatic improvement and he was discharged without complications.

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.

Avaliação semiquantitativa ecocardiográfica de dilatações vasculares intrapulmonares em candidatos a transplante hepático: correlação com avaliação de shunt e parâmetros funcionais pulmonares / Semiquantitative echocardiographic evaluation of intrapulmonary vascular dilatations: correlation with evaluation of shunt levels and pulmonary function parameters

OBJETIVO: Verificar a relação entre a avaliação semiquantitativa de dilatações vasculares intrapulmonares (DVIP) e a avaliação quantitativa de shunt, e descrever achados clínicos e funcionais pulmonares em uma amostra de hepatopatas com DVIP. MÉTODOS: Pacientes com ecocardiografia transtorácica (ETT) positiva para DVIP foram submetidos à avaliação clínica e de função pulmonar assim como à quantificação de shunt intrapulmonar (cintilografia com macroagregados de albumina marcados com tecnécio-99m e por gasometria com oxigênio a 100 por cento). RESULTADOS: Foram estudados 28 pacientes cirróticos (média de idade, 47,5 anos; 60,7 por cento dos casos classificados como Child-Pugh B). Uma escala de 4 pontos, em ordem ascendente, foi utilizada para medir a intensidade das DVIP, classificada de 1 a 4, respectivamente, em 13 (46,4 por cento), 9 (32,1 por cento), 2 (7,1 por cento) e 4 (14,3 por cento) dos pacientes. A amostra foi dividida em grupo baixa intensidade (escores 1 e 2) e grupo alta intensidade (escores 3 e 4). A média de shunt por cintilografia foi 14,9 por cento na amostra total, sendo menor no grupo baixa intensidade (11,7 por cento vs. 26,3 por cento; p = 0,01). O grupo alta intensidade teve maiores valores de shunt através de gasometria (8,3 por cento vs. 16,3 por cento; p < 0.001). A PaO2 média foi inferior no grupo alta intensidade. A intensidade de DVIP e a DLCO correlacionaram-se de forma inversa (r = -0,406, p = 0,01). CONCLUSÕES: A ETT é um método útil e seguro para avaliação da gravidade das DVIP em pacientes com hepatopatia. A classificação ecocardiográfica da intensidade das DVIP se correlacionou com valores de shunt intrapulmonar obtidos pelos métodos quantitativos avaliados, bem como com anormalidades nas trocas gasosas pulmonares.

OBJECTIVE: To correlate semiquantitative evaluation of intrapulmonary vascular dilatations (IPVD) with quantitative evaluation of shunt levels, as well as to describe clinical and pulmonary function findings in a sample of liver disease patients with IPVD. METHODS: Patients presenting transthoracic echocardiography (TTE) positivity for IPVD underwent clinical evaluation, pulmonary function tests and pulmonary shunt quantification (scintigraphy with technetium-99m-labeled albumin macroaggregates and blood gas analysis after pure oxygen breathing). RESULTS: A total of 28 liver cirrhosis patients were studied (mean age, 47.5 years; 60.7 percent were Child-Pugh class B). A 4-point, ascending scale was used as a measure of IPVD intensity, which was scored as 1, 2, 3 and 4, respectively, in 13 (46.4 percent), 9 (32.1 percent), 2 (7.1 percent) and 4 (14.3 percent) of the patients. Patients were divided into a low-intensity group (scores 1 and 2) and a high-intensity group (scores 3 and 4). The mean shunt assessed using scintigraphy was 14.9 percent in the sample as a whole and was lower in the low-intensity group (11.7 percent vs. 26.3 percent; p = 0.01). The mean shunt by blood gas analysis was higher in the high-intensity group (8.3 percent vs. 16.3 percent; p < 0.001). Mean PaO2 was lower in the high-intensity group. There was a negative correlation between DLCO and IPVD severity (r = -0.406, p = 0.01). CONCLUSIONS: TTE is a safe, useful tool for assessing IPVD severity in liver disease patients. The IPVD intensity assessed using TTE correlated with the intrapulmonary shunt values obtained through the quantitative methods evaluated, as well as with pulmonary gas exchange abnormalities.

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.

Hepatopulmonary syndrome.

Hepatopulmonary syndrome (HPS) is defined as clinical triad of advanced liver disease, arterial deoxygenation and intra pulmonary vascular dilatation. It is a rare complication of liver disease of varied etiology and indicates a poor prognosis. Many theories have been put forward to throw light over its pathogenesis. The major clinical manifestations are arterial hypoxemia, clubbed fingers and spider navei. Orthodeoxia and platypnea are usual clinical features. A simple non invasive method to screen HPS is desirable. Contrast enhanced 2D ECHO cardiography is the preferred screening test. No effective medical treatment has been found. Although liver transplant seems feasible to reverse (at least partially) this situation, however it is associated with increased post operative morbidity and mortality.

Prevalence of hepatopulmonary syndrome in patients with decompensated chronic liver disease and its impact on short-term survival

BACKGROUND: Hepatopulmonary syndrome is reported to occur in 4 percent to 32 percent of the patients with chronic liver disease and is associated with poor liver function and shortened patient survival before and after liver transplantation. AIMS: To assess the frequency of hepatopulmonary syndrome in Brazilian patients with decompensated chronic liver disease and to investigate its impact on patient survival. METHODS: One hundred and thirty patients (101 males, mean age 61 ± 12 years) with decompensated chronic liver disease were evaluated for the presence of hepatopulmonary syndrome. The diagnosis of hepatopulmonary syndrome was considered in the presence of alveolar arterial oxygen gradient of more than 15 mm Hg and of pulmonary vascular dilatation assessed by contrast enhanced echocardiography. RESULTS: Hepatopulmonary syndrome was observed in 21 (16 percent) patients. The presence of hepatopulmonary syndrome was significantly associated with severity of liver disease assessed by the MELD (Model for End-Stage Liver Disease) score, but not with in hospital mortality after admission due to decompensated chronic liver disease. CONCLUSIONS: Hepatopulmonary syndrome occurs in 16 percent of patients with chronic liver disease and is associated with disease severity according to the MELD score. Short term mortality following decompensation of chronic liver disease was not associated with hepatopulmonary syndrome.

RACIONAL: A síndrome hepatopulmonar ocorre em 4 por cento a 32 por cento dos pacientes com doença crônica parenquimatosa do fígado e está associada à gravidade da doença hepática e menor sobrevida antes e após o transplante hepático. OBJETIVOS: Avaliar a freqüência da síndrome hepatopulmonar em pacientes brasileiros hospitalizados com doença crônica parenquimatosa do fígado descompensada e investigar o seu impacto na sobrevida intra-hospitalar. MÉTODOS: Cento e trinta doentes (101 homens, idade média 61 ± 12 anos) com doença crônica parenquimatosa do fígado descompensada foram avaliados quanto à presença de síndrome hepatopulmonar. O diagnóstico de síndrome hepatopulmonar foi considerado na presença de aumento do gradiente alvéolo arterial de oxigênio maior que 15 mm Hg e de dilatação vascular pulmonar avaliada pelo ecocardiografia com microbolhas. RESULTADOS: Síndrome hepatopulmonar foi observada em 21 (16 por cento) dos doentes. Sua presença foi associada à gravidade da doença hepática avaliada pela pontuação MELD, mas não com mortalidade intra-hospitalar. CONCLUSÕES: A síndrome hepatopulmonar ocorre em 16 por cento dos pacientes hospitalizados com doença crônica parenquimatosa do fígado, estando associada à gravidade da doença hepática. Mortalidade a curto prazo por descompensação da doença crônica parenquimatosa do fígado não foi associada à presença de síndrome hepatopulmonar.

Complicaciones pulmonares de la cirrosis hepática: hipertensión portopulmonar y síndrome hepatopulmonar: la paradoja de la vasoconstricción y vasodilatación pulmonar / Pulmonary complications of hepatic cirrhosis: portopulmonary hypertension and hepatopulmonary syndrome: the paradox of pulmonary vasoconstriction and vasodilation

Las complicaciones pulmonares de las enfermedades crónicas del hígado han sido descritas desde hace más de un siglo. Aunque originadas por una lesión común y vías fisiopatológicas similares, sus manifestaciones clínicas y el diagnóstico son divergentes. Su impacto clínico es importante ya que afectan de forma deletérea el pronóstico de los pacientes. Hasta el momento no existe un tratamiento efectivo para el manejo de estas enfermedades y el trasplante hepático debe ser evaluado de forma muy cuidadosa. En esta revisión se analizan los aspectos más importantes de la hipertensión portopulmonar y del síndrome hepatopulmonar, entidades que bajo el entorno de la hipertensión portal se caracterizan respectivamente por procesos de vasoconstricción y vasodilatación pulmonar.

Cardiopulmonar complications in chronic liver diseases were described 100 years ago. Altough both hepatopulmonary sindrome and portopulmonary hypertension originates from liver damage, clinical findings and diagnosis are very different. These complications are important due to the highly deleterous impact on disease evolution and prognosis. Currently, there is not an ideal treatment for these diseases and liver transplantation should be adequately evaluated. In this review we analyze the most important issues on hepatopulmonary sindrome and portopulmonary hypertension. These complications,under the cornerstone of portal hypertension are characterized by pulmonary constriction and dilatation, respectively.

Hepatopulmonary syndrome

This is a report of a 56-year-old male who was admitted to the Intensive Care Unit of the San Juan V.A. Medical Center with altered mental status and severe hypoxemia. He was diagnosed with severe hyponatremia and hepatopulmonary syndrome.