Improved survival in children with HPS: Experience from two high volume liver transplant centers across continents

Abstract

Background: Severe HPS increases morbidity and mortality after LT in children. We reviewed the combined experience of LT for HPS in children from two LT centers in Europe and Asia. Methods: All children with "proven" HPS as per ERS Task Force criteria (detailed in manuscript) who underwent LT were categorized into M (PaO 2 ≥80 mmHg), Mo (PaO 2 = 60-79 mmHg), S (50-59 mmHg), and VS (PaO 2 <50 mmHg) HPS, based on room air PaO 2 .Results: Twenty-four children with HPS underwent 25 LT (one re-transplantation) at a median age of 8 years (IQR, 5-12), after a median duration of 8 (4-12) months following HPS diagnosis. Mechanical ventilation was required for a median of 3 (1.5-27) days after LT. Ten children had "S" post-operative hypoxemia, requiring iNO for a median of 5 (6-27) days. "VS" category patients had significantly prolonged invasive ventilation (median 35 vs. 3 and 1.5 days; p = .008), ICU stay (median 39 vs. 8 and 8 days; p = .007), and hospital stay (64 vs. 26.5 and 23 days; p < .001) when compared to "S" and "M/Mo" groups, respectively. The need for pre-transplant home oxygen therapy was the only factor predicting need for re-intubation. Patient and graft survival at 32 (17-98) months were 100% and 95.8%. All children ultimately had complete resolution of HPS.Conclusions: VS HPS is associated with longer duration of mechanical ventilation and hospital stay, which emphasizes the need for early LT in these children.

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.…”

Challenges faced in establishing a pediatric liver transplant program in a lower‐middle‐income country with free healthcare service

“…However, these programs are primarily based in the private sector. [5][6][7][8][9] Sri Lanka (SL) is an island nation located in South Asia, next to India geographically. It is categorized as a low-middle income country with a diverse population, comprising various ethnic groups.…”

“…India being one of the SA countries is successfully performing a high volume of pediatric liver transplants (PLT) each year, including auxiliary and domino LTs. However, these programs are primarily based in the private sector 5–9 …”