Isolated Intrapulmonary Vascular Dilatations and the Risk of Developing Hepatopulmonary Syndrome in Liver Transplant Candidates.

BACKGROUND: The natural history of intrapulmonary vascular dilations (IPVD) and their impact on patient outcomes in the setting of portal hypertension has only been described in small series. AIMS: To assess the development of hepatopulmonary síndrome (HPS) in patients with isolated IPVD and to evaluate outcomes of IPVD and HPS among patients evaluated for liver transplantation (LT). MATERIAL AND METHODS: Data from a prospective cohort of patients evaluated for LT with standardized screening for HPS were analyzed. IPVDs were defined as the presence of microbubbles in the left atrium > 3 cycles following right atrial opacification. HPS was defined as the presence of IPVD and hypoxemia (Alveolar-arterial gradient ≥ 15 mmHg) in the absence of concomitant cardiopulmonary disease. RESULTS: A total of 104 patients with negative contrast-enhanced echocardiogram (CE) were compared to 63 patients with IPVD and 63 patients with HPS. Only four patients were categorized as 'severe' HPS based on degree of hipoxemia (defined as PaO2 < 60 mmHg). Twenty IPVD patients were followed with ABG over a mean duration of 21 months (range 9-43), of whom 7 (35%) subsequently met HPS criteria. Overall unadjusted survival from the time of LT evaluation using multi-state survival models that accounted for pre- and post-LT time was not statistically different among the three groups (negative CE, IPVD, and HPS; p > 0.5). CONCLUSIONS: Patients with IPVD appear to have a substantial risk of developing oxygenation impairment over time and progress to HPS. In our cohort, survival in patients with HPS and isolated IPVD is not different when compared to those without IPVDs.

Biliary atresia: Indications and timing of liver transplantation and optimization of pretransplant care.

Biliary atresia (BA) is a progressive, fibro-obliterative disorder of the intrahepatic and extrahepatic bile ducts in infancy. The majority of affected children will eventually develop end-stage liver disease and require liver transplantation (LT). Indications for LT in BA include failed Kasai portoenterostomy, significant and recalcitrant malnutrition, recurrent cholangitis, and the progressive manifestations of portal hypertension. Extrahepatic complications of this disease, such as hepatopulmonary syndrome and portopulmonary hypertension, are also indications for LT. Optimal pretransplant management of these potentially life-threatening complications and maximizing nutrition and growth require the expertise of a multidisciplinary team with experience caring for BA. The timing of transplant for BA requires careful consideration of the potential risk of transplant versus the survival benefit at any given stage of disease. Children with BA often experience long wait times for transplant unless exception points are granted to reflect severity of disease. Family preparedness for this arduous process is therefore critical. Liver Transplantation 23:96-109 2017 AASLD.

Clinical characteristics, predictors, and survival among patients with hepatopulmonary syndrome.

BACKGROUND: Hepatopulmonary syndrome (HPS) is a complication of advanced liver disease. The impact of HPS on survival is not clearly understood. MATERIAL AND METHODS: A prospective study was carried out at Department of Medicine, King Edward Medical University Lahore from June 2011 to May 2012. Patients with cirrhosis of liver were evaluated for presence of HPS with arterial blood gas analysis and saline bubble echocardiography. All patients were followed for 6 months for complications and mortality. Cox regression analysis was done to evaluate role of HPS on patient survival. RESULTS: 110 patients were included in the study. Twenty-nine patients (26%) had HPS. MELD score was significantly higher (p < 0.01) in patients with HPS (18.93 ± 3.51) as compared to that in patients without HPS (13.52 ± 3.3). Twenty two (75.9%) patients of Child class C, 5 (17.2%) patients of Child class B and 2 (6.9%) patients of Child class A had HPS (P 0.03). The clinical variables associated with presence of HPS were spider nevi, digital clubbing, dyspnea, and platypnea. HPS significantly increased mortality during six month follow up period (HR: 2.47, 95% CI: 1.10- 5.55). Child-Pugh and MELD scores were also associated with increased mortality. HPS was no longer associated with mortality when adjustment was done for age, gender, Child-Pugh, and MELD scores (HR: 0.44, 95% CI: 0.14-1.41). Both the Child-Pugh and MELD scores remained significantly associated with mortality in the multivariate survival analysis. CONCLUSIONS: HPS indicates advanced liver disease. HPS does not affect mortality when adjusted for severity of cirrhosis.

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% to 32% 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%) 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% 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.

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.