title: Oral l-citrulline malate in patients with idiopathic pulmonary arterial hypertension and Eisenmenger Syndrome: a clinical trial.
abstract: OBJECTIVE
  Citrulline is an amino acid which is produced by the urea cycle and also a precursor for NO, that is, a vasodilator for normal function of pulmonary vasculature. Thereby, enhancing l-citrulline malate in patients with idiopathic pulmonary arterial hypertension and those with congenital heart disease identified as Eisenmenger Syndrome results in reduction of pulmonary hypertension.
  
  
  METHODS
  In this clinical trial before and after study, we assigned 25 patients with arterial pulmonary hypertension (idiopathic or Eisenmenger Syndrome) to receive l-citrulline malate 1g three times daily for two weeks. The primary measurement was the change in exercise capacity, as considered as a result of the total distance walked in six minutes, from baseline to week 2. We also assessed mean pulmonary artery pressure, the change in the quality of life, and the change in pro-brain natriuretic peptide (BNP) level. The study was not powered to evaluate mortality.
  
  
  RESULTS
  The mean walking distance in six minutes was significantly increased by about 44m (p=0.005) after receiving l-citrulline malate. Mean pulmonary artery pressure significantly reduced from 83.34mmHg before receiving l-citrulline malate to 79.1mmHg after that (p=0.01). All dimensions of the quality of life had statistical differences after receiving l-citrulline malate except limit due to physical health, limit due to emotional health and social functioning (p>0.05). Finally, pro-BNP difference was not statistically significant (p=0.9).
  
  
  CONCLUSIONS
  l-Citrulline malate improves the distance walk in six minutes and also the quality of life of patients with idiopathic arterial pulmonary hypertension and Eisenmenger Syndrome and also reduced mean arterial pulmonary hypertension.
authors: Cardiology Department, Lung Transplantation Research Center, National Research Institute of Tuberculosis and Lung Diseases (NRITLD), Shahid Beheshti University of Medical Sciences, PO Box 19575-154, Tehran 19569-444413, Iran.
authors: Babak
authors: B
authors: Sharif Kashani
authors: Lung Transplantation Research Center, National Research Institute of Tuberculosis and Lung Diseases (NRITLD), Shahid Beheshti University of Medical Sciences, PO Box 19575-154, Tehran 19569-444413, Iran. Electronic address: paritash_t@yahoo.com.
authors: Paritash
authors: P
authors: Tahmaseb Pour
authors: Tracheal Diseases Research Center, National Research Institute of Tuberculosis and Lung Diseases (NRITLD), Shahid Beheshti University of Medical Sciences, PO Box 19575-154, Tehran 19569-444413, Iran.
authors: Majid
authors: M
authors: Malekmohammad
authors: Chronic Respiratory Diseases Research Center, National Research Institute of Tuberculosis and Lung Diseases (NRITLD), Shahid Beheshti University of Medical Sciences, PO Box 19575-154, Tehran 19569-444413, Iran.
authors: Neda
authors: N
authors: Behzadnia
authors: Tobacco Prevention and Control Research Center, National Research Institute of Tuberculosis and Lung Diseases (NRITLD), Shahid Beheshti University of Medical Sciences, PO Box 19575-154, Tehran 19569-444413, Iran.
authors: Faezeh
authors: F
authors: Sheybani-Afshar
authors: Tobacco Prevention and Control Research Center, National Research Institute of Tuberculosis and Lung Diseases (NRITLD), Shahid Beheshti University of Medical Sciences, PO Box 19575-154, Tehran 19569-444413, Iran.
authors: Mohammad
authors: M
authors: Fakhri
authors: Department of Biostatistics, Faculty of Paramedics, Shahid Beheshti University of Medical Sciences, PO Box 19575-154, Tehran 19569-444413, Iran.
authors: Samira
authors: S
authors: Chaibakhsh
authors: Lung Transplantation Research Center, National Research Institute of Tuberculosis and Lung Diseases (NRITLD), Shahid Beheshti University of Medical Sciences, PO Box 19575-154, Tehran 19569-444413, Iran.
authors: Farah
authors: F
authors: Naghashzadeh
authors: Tobacco Prevention and Control Research Center, National Research Institute of Tuberculosis and Lung Diseases (NRITLD), Shahid Beheshti University of Medical Sciences, PO Box 19575-154, Tehran 19569-444413, Iran.
authors: Salavan
authors: S
authors: Aidenlou
mesh_terms: D000284:Administration, Oral; D000328:Adult; D062186:Arterial Pressure; D002956:Citrulline / Q000008:administration & dosage / Q000031:analogs & derivatives*; D004541:Eisenmenger Complex / Q000188:drug therapy* / Q000503:physiopathology; D065627:Familial Primary Pulmonary Hypertension / Q000188:drug therapy* / Q000503:physiopathology; D005260:Female; D006801:Humans; D008293:Malates / Q000008:administration & dosage*; D008297:Male; D020097:Natriuretic Peptide, Brain / Q000097:blood; D011651:Pulmonary Artery / Q000503:physiopathology; D011788:Quality of Life; D016896:Treatment Outcome; D016138:Walking; D055815:Young Adult
chemical_list: D008293:Malates; D020097:Natriuretic Peptide, Brain; D002956:Citrulline; C071162:citrulline malate
keywords: Eisenmenger Syndrome; Exercise capacity; Idiopathic pulmonary arterial hypertension; Quality of life; l-Citrulline malate