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

Naresh Shanmugam1, Abdul Rahman Hakeem1, Joseph J Valamparampil1

  • 1Dr. Rela Institute & Medical Centre, Bharath Institute of Higher Education and Research, Chennai, India.

Insights

Pediatric liver transplantation (LT) for hepatopulmonary syndrome (HPS) shows excellent survival. Very severe (VS) HPS requires longer ventilation and hospital stays, indicating a need for early LT.

Area of Science:

  • Pediatric Hepatology
  • Pulmonology
  • Transplantation Medicine

Background:

  • Severe hepatopulmonary syndrome (HPS) significantly increases morbidity and mortality in children undergoing liver transplantation (LT).
  • This study reviews the combined experience of LT for HPS in pediatric patients from two international centers.

Purpose of the Study:

  • To evaluate the outcomes of pediatric liver transplantation (LT) for hepatopulmonary syndrome (HPS).
  • To assess the impact of HPS severity, categorized by pre-transplant oxygenation levels, on post-transplant outcomes.

Main Methods:

  • Children with proven HPS underwent LT and were classified into four groups (M, Mo, S, VS) based on room air partial pressure of oxygen (PaO2).
  • Outcomes including mechanical ventilation duration, intensive care unit (ICU) stay, hospital stay, and survival rates were analyzed.

Main Results:

  • Twenty-four children underwent 25 LT procedures; 10 children experienced post-operative hypoxemia (S category) requiring inhaled nitric oxide (iNO).
  • Patients with very severe (VS) HPS had significantly longer invasive ventilation, ICU, and hospital stays compared to S and M/Mo groups.
  • Pre-transplant home oxygen therapy was the sole predictor for re-intubation; patient and graft survival rates were high (100% and 95.8%, respectively).

Conclusions:

  • Very severe (VS) HPS is linked to prolonged mechanical ventilation and hospitalization after LT in children.
  • These findings underscore the importance of considering early LT for children with VS HPS to improve outcomes.
  • All children achieved complete resolution of HPS post-transplantation.
Abstract