Fatal hyperammonemia after orthotopic lung transplantation

Ann Intern Med. 2000 Feb 15;132(4):283-7. doi: 10.7326/0003-4819-132-4-200002150-00006.


Background: A case of fatal hyperammonemia complicating orthotopic lung transplantation was previously reported.

Objective: To describe the incidence, clinical features, and treatment of hyperammonemia associated with orthotopic lung transplantation.

Design: Retrospective cohort analysis.

Setting: Academic medical center and lung transplantation center in Philadelphia, Pennsylvania.

Patients: 145 sequential adult patients who underwent orthotopic lung transplantation.

Measurements: Plasma ammonium levels.

Results: Six of the 145 patients who had had orthotopic lung transplantation developed hyperammonemia, all within the first 26 days after transplantation. The 30-day post-transplantation mortality rate was 67% for patients with hyperammonemia compared with 17% for those without hyperammonemia (P = 0.01). Development of major gastrointestinal complications (P = 0.03), use of total parenteral nutrition (P < 0.001), and lung transplantation for primary pulmonary hypertension (P = 0.045) were associated with hyperammonemia.

Conclusions: Hyperammonemia is a potentially fatal event occurring after orthotopic lung transplantation. It is associated with high nitrogen load, concurrent medical stressors, primary pulmonary hypertension, and hepatic glutamine synthetase deficiency.

MeSH terms

  • Adult
  • Ammonia / blood*
  • Cohort Studies
  • Gastrointestinal Diseases / etiology
  • Glutamate-Ammonia Ligase / deficiency
  • Humans
  • Hypertension, Pulmonary / surgery
  • Liver / enzymology
  • Lung Transplantation*
  • Middle Aged
  • Parenteral Nutrition, Total
  • Postoperative Complications / blood*
  • Retrospective Studies
  • Transplantation, Heterotopic
  • Treatment Outcome


  • Ammonia
  • Glutamate-Ammonia Ligase