The Use of Citrulline for Pediatric Pulmonary Hypertension

Abstract

Pulmonary hypertension can be diagnosed at all ages and characterized by increased vascular resistance in the lungs. Pulmonary hypertension in the pediatric population is less studied than in adults with much of the therapy extrapolated from adult data. Suboptimal survival rate of patients with pulmonary hypertension and the economic impact this disease has on the healthcare system warrant the need for new therapeutic options, such as citrulline. Citrulline is a non-essential amino acid involved in the urea cycle, which ultimately converts to nitric oxide to help dilate the pulmonary vasculature. Based on the available animal and few human studies, citrulline can potentially be a preventive remedy for pediatric patients at high risk of developing pulmonary hypertension after undergoing congenital cardiac surgery. However, new clinical trials are needed before using citrulline as a standard treatment option and incorporating it into practice guidelines.