Impact of hypoxemia on pediatric liver transplantation for hepatopulmonary syndrome

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 Hg, 11 patients, and G2-PaO 2 < 50 mm Hg, 10 patients. Demographic, clinical, laboratory, and perioperative data; outcome variables; and post-transplant survival were compared between the groups.Results: In total, 2/11 (18.2%) patients in G1 and 8/10 (80%) patients in G2 required supplemental oxygen therapy at home (P = .005). Patients in G2 required prolonged MV (median 8.5 days in G2 vs 1 day in G1, P = .015) and prolonged ICU and hospital stays (P = .002 and P = .001, respectively). Oxygen weaning time was longer in G2 (median 127.5 days) than in G1 (median 3 days; P = .004). One (9.1%) patient in G1 and three (30%) patients in G2 died (P = .22). The survival at 90 months was 90.9% in G1 and 70% in G2 (P = .22). Conclusion:The survival between groups was similar. Patients with very severe HPS required a longer MV time, longer ICU and hospital stays, and a longer O 2 weaning time than those with mild, moderate, or severe HPS.

Model for End‐Stage Liver Disease/Pediatric End‐Stage Liver Disease exception policy and outcomes in pediatric patients with hepatopulmonary syndrome requiring liver transplantation

“…[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.…”

Beyond Varices: Complications of Cirrhotic Portal Hypertension in Pediatrics

“…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…”