Abstract:Background: The treatment of choice for patients with cirrhosis and HPS is LT. The clinical manifestations associated with hypoxemia result in limitations and a poor health-related quality of life of affected patients. The present report aims to study the differences in outcomes between patients with PaO 2 < 50 mm Hg and those with PaO 2 ≥ 50 mm Hg. Methods: This was a retrospective study of 21 patients under 18 years of age conducted from 2001 to 2018; the patients were divided into 2 groups: G1-PaO 2 ≥ 50 mm… Show more
“…[37] Although there are robust data to support an association between room air oxygenation and posttransplantation survival in adult HPS patients, the largest studies on this topic in children were just recently published and derived from retrospective cohorts of less than 25 patients each. [38,39] The authors observed that although children with very severe HPS (PaO 2 < 50 mm Hg) required longer durations of mechanical ventilation, longer intensive care unit (ICU) and hospital stays, and longer O 2 weaning time than those with mild, moderate, or severe HPS (PaO 2 > 50 mm Hg), there was no difference in mortality across subgroups. Although our findings also indicate that severity of HPS does not impact post-LT survival in children, our analysis expands on the size and depth of this work.…”
Hepatopulmonary syndrome (HPS) is associated with increased waitlist mortality in liver transplantation (LT) candidates. Children with HPS are granted Model for End‐Stage Liver Disease (MELD)/Pediatric End‐Stage Liver Disease (PELD) exception points for waitlist prioritization in the United States based on criterion developed for adults. In this study, the impact of this MELD/PELD exception policy on post‐LT survival in children was examined. A retrospective cohort of patients aged younger than 18 years with a MELD/PELD exception request who underwent LT between 2007 and 2018 were identified in the Scientific Registry of Transplant Recipients. Patients were stratified by waitlist partial pressure of arterial oxygen (PaO2) to assess risk factors for waitlist mortality and post‐LT survival. Among 3082 pediatric LT recipients included in the study, 124 patients (4%) received MELD/PELD exception points for HPS. Patients with HPS were a median age of 9 years (interquartile range: 6, 12 years), 54.8% were girls, and 54% were White. Most patients (87.9%) were listed with laboratory MELD/PELD scores <15. Waitlist mortality for patients with HPS exception points was rare and not different from patients without HPS. When stratified by pre‐LT PaO2, hypoxemia severity was not associated with differences in 1‐, 3‐, or 5‐year survival rates after LT (p = 0.13). However, patients with HPS showed a slightly lower survival rate at 5 years compared with patients without HPS (88.7% vs. 93.4%; p = 0.04). MELD/PELD exceptions for children with HPS mitigated waitlist mortality, and recipients with HPS experienced excellent 5‐year survival after LT, although slightly lower than in patients without HPS. Unlike adults with HPS, the severity of pre‐LT hypoxemia in children does not impact post‐LT survival. These data suggest that adult criteria for granting MELD/PELD exception points may not appropriately capture HPS severity in pediatric patients. Further prospective multicenter studies to examine the risk factors predicting negative survival outcomes in children with HPS are warranted.
“…[37] Although there are robust data to support an association between room air oxygenation and posttransplantation survival in adult HPS patients, the largest studies on this topic in children were just recently published and derived from retrospective cohorts of less than 25 patients each. [38,39] The authors observed that although children with very severe HPS (PaO 2 < 50 mm Hg) required longer durations of mechanical ventilation, longer intensive care unit (ICU) and hospital stays, and longer O 2 weaning time than those with mild, moderate, or severe HPS (PaO 2 > 50 mm Hg), there was no difference in mortality across subgroups. Although our findings also indicate that severity of HPS does not impact post-LT survival in children, our analysis expands on the size and depth of this work.…”
Hepatopulmonary syndrome (HPS) is associated with increased waitlist mortality in liver transplantation (LT) candidates. Children with HPS are granted Model for End‐Stage Liver Disease (MELD)/Pediatric End‐Stage Liver Disease (PELD) exception points for waitlist prioritization in the United States based on criterion developed for adults. In this study, the impact of this MELD/PELD exception policy on post‐LT survival in children was examined. A retrospective cohort of patients aged younger than 18 years with a MELD/PELD exception request who underwent LT between 2007 and 2018 were identified in the Scientific Registry of Transplant Recipients. Patients were stratified by waitlist partial pressure of arterial oxygen (PaO2) to assess risk factors for waitlist mortality and post‐LT survival. Among 3082 pediatric LT recipients included in the study, 124 patients (4%) received MELD/PELD exception points for HPS. Patients with HPS were a median age of 9 years (interquartile range: 6, 12 years), 54.8% were girls, and 54% were White. Most patients (87.9%) were listed with laboratory MELD/PELD scores <15. Waitlist mortality for patients with HPS exception points was rare and not different from patients without HPS. When stratified by pre‐LT PaO2, hypoxemia severity was not associated with differences in 1‐, 3‐, or 5‐year survival rates after LT (p = 0.13). However, patients with HPS showed a slightly lower survival rate at 5 years compared with patients without HPS (88.7% vs. 93.4%; p = 0.04). MELD/PELD exceptions for children with HPS mitigated waitlist mortality, and recipients with HPS experienced excellent 5‐year survival after LT, although slightly lower than in patients without HPS. Unlike adults with HPS, the severity of pre‐LT hypoxemia in children does not impact post‐LT survival. These data suggest that adult criteria for granting MELD/PELD exception points may not appropriately capture HPS severity in pediatric patients. Further prospective multicenter studies to examine the risk factors predicting negative survival outcomes in children with HPS are warranted.
“…94,[105][106][107][108] In single-center studies, children with very severe HPS (PaO 2 < 50 mm Hg) had longer need for supplementation oxygen, mechanical ventilator, and ICU stay as compared with children with less severe degrees of hypoxemia. 93,109 Additional management strategies in children with HPS and persistent hypoxemia have been reported including the use of inhaled NO and high-flow nasal cannula and even in these children, eventual resolution of HPS is achieved. 110,111…”
Complications of cirrhotic portal hypertension (PHTN) in children are broad and include clinical manifestations ranging from variceal hemorrhage, hepatic encephalopathy (HE), ascites, spontaneous bacterial peritonitis (SBP), and hepatorenal syndrome (HRS) to less common conditions such as hepatopulmonary syndrome, portopulmonary hypertension, and cirrhotic cardiomyopathy. The approaches to the diagnosis and management of these complications have become standard of practice in adults with cirrhosis with many guidance statements available. However, there is limited literature on the diagnosis and management of these complications of PHTN in children with much of the current guidance available focused on variceal hemorrhage. The aim of this review is to summarize the current literature in adults who experience these complications of cirrhotic PHTN beyond variceal hemorrhage and present the available literature in children, with a focus on diagnosis, management, and liver transplant decision making in children with cirrhosis who develop ascites, SBP, HRS, HE, and cardiopulmonary complications.
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