Treatment of Hyperammonemia Syndrome in Lung Transplant Recipients

Hyperammonemia syndrome is a complication that has been reported to occur in 1-4% of lung transplant patients with mortality rates as high as 60-80%, making detection and management crucial components of post-transplant care. Patients are treated with a multimodal strategy that may include renal replacement therapy, bowel decontamination, supplementation of urea cycle intermediates, nitrogen scavengers, antibiotics against Mollicutes, protein restriction, and restriction of parenteral nutrition. In this review we provide a framework of pharmacologic mechanisms, medication doses, adverse effects, and available evidence for commonly used treatments to consider when initiating therapy. In the absence of evidence for individual strategies and conclusive knowledge of the causes of hyperammonemia syndrome, clinicians should continue to design multimodal regimens based on suspected etiologies, institutional drug availability, patient ability to tolerate enteral medications and nutrition, and availability of intravenous access.