Exercise Capacity and Quality of Life in Patients With PPH Receiving Short Term Oral L-Citrulline Malate
Due to vasodilatory properties of the NO, one of the therapeutic approaches for IPAH is oral use of nitric oxide precursors (10). Efficacy of L-arginine is well-documented in the current literature but there is paucity of data with regard to L-citrulline- malate. Hence, this study will evaluate therapeutic efficacy of L-citrulline- malate in two categories of patients with pulmonary hypertension (IPAH, and Eisenmeger syndrome). This randomized clinical trial utilizes 6-minute walk, pro BNP levels and the echocardiographic indexes an indicator of functional improvement of the patients.
Idiopathic Pulmonary Arterial Hypertension
Drug: L-Citrulline Malate
|Study Design:||Endpoint Classification: Efficacy Study
Intervention Model: Single Group Assignment
Masking: Open Label
|Official Title:||Exercise Capacity and Quality of Life in Patients With Idiopathic Pulmonary Hypertension and Eisenmenger Syndrome Receiving Short Term Oral L-Citrulline Malate|
- the change in exercise capacity [ Time Frame: 2 weeks ] [ Designated as safety issue: No ]The primary measure of efficacy was the change in exercise capacity, as measured by the total distance walked in six minutes, from baseline to week 2. (15)
- changes in mean pulmonary-artery pressure [ Time Frame: 2 weeks ] [ Designated as safety issue: No ]changes in mean pulmonary-artery pressure from baseline to week 2.
- change in the quality of life [ Time Frame: 2 weeks ] [ Designated as safety issue: No ]change in the quality of life from baseline to week 2
|Study Start Date:||August 2012|
|Estimated Study Completion Date:||December 2012|
|Estimated Primary Completion Date:||December 2012 (Final data collection date for primary outcome measure)|
Experimental: L-Citrulline, Exercise Capacity
L-Citrulline malate, 1gr, oral, divided 3 times a day,for 2 weeks
Drug: L-Citrulline Malate
1 gr per day, oral, for 2 weeks
Other Name: Stimol
Pulmonary vascular tone is maintained by the action of vasoprotective compounds including nitric oxide (NO)(1).NO can be synthesized endogenously in the body via L-arginine and NOS-independent mechanism from the anion nitrite (NO2-)(2,3).Nitric oxide (NO) causes cyclic guanosine monophosphate-mediated vasodilatation of the pulmonary vasculature. Endogenous NO is also produced from the metabolism of citrulline; an amino acid generated by the urea cycle (4). NO is critical for normal development of the pulmonary vasculature and loss of this vasodilator factor and subsequent endothelial dysfunction is proposed as one of the possible explanations for development of pulmonary hypertension (1).
From a clinical standpoint, pulmonary hypertension is a common complication of chronic obstructive pulmonary disease (COPD).Its presence is associated with shorter survival and worse clinical outcome. In a setting of COPD, pulmonary hypertension tends to be of moderate severity and progresses slowly. Recent investigations have demonstrated endothelial dysfunction and changes in the expression of endothelial-derived mediators that regulate vascular tone and cell growth in the pulmonary arteries of patients with mild disease(5). Pulmonary vascular involvement from congenital heart disease like Eisenmeger syndrome is another important category of patients with PAH. In this congenital disease pulmonary vascular involvement follows a period in which pulmonary resistance is low and pulmonary blood flow is high (6, 7, 8). Finally, Idiopathic pulmonary hypertension (IPAH) is the third category of these patients. IPAH has unknown etiology and is characterized by progressive obliteration of small and medium size pulmonary arteries; elevation in pulmonary arterial pressure, and an increase in pulmonary vascular resistance. Presence of these pathologies eventually leads to right heart failure and death (9).
|Contact: babak sharif kashani, Cardiologistemail@example.com|
|Contact: paritash tahmasebpour, MDfirstname.lastname@example.org|
|Iran, Islamic Republic of|
|Tehran, Iran, Islamic Republic of, 021|
|Contact: babak sharif kashani, cardiologist 0098-02188883114 email@example.com|
|Contact: paritash tahmasebpour, MD 0098-09125037861 firstname.lastname@example.org|
|Principal Investigator: babak sharif kashani, cardiologist|
|Principal Investigator:||babak sharif kashani, cardiologist||Lung Transplantation Research Center, National Research Institute of Tuberculosis and Lung Disease (NRITLD), Shahid Beheshti University of Medical Science, Tehran, Iran.|
|Principal Investigator:||Paritash Tahmaseb pour, MD||MD|