title: Effect of a single IV administration of L-propionylcarnitine on myocardial microcirculation assessed by coronary flow velocity reserve measurement in patients with systemic sclerosis: a pilot study.
abstract: BACKGROUND
  Scleroderma-related cardiac involvement primarily affects coronary microvascular structures and function. The microvasculature disorder is responsible for impairment of coronary flow velocity reserve (CFVR), which has been reported in studies of patients with systemic sclerosis (SSc). L-propionylcarnitine (L-PC) is a metabolic substance that is associated with a beneficial effect on both microcirculation and myocyte function.
  
  
  OBJECTIVE
  The objective of this study was to determine whether or not CFVR was acutely improved or restored in patients with SSc after a single administration of IV L-PC.
  
  
  METHODS
  In this pilot study, we screened volunteers with SSc who had no clinical evidence of ischemic heart disease. CFVR was determined by a blinded investigator by evaluating the left anterior descending coronary artery (LADCA) by transthoracic echocardiography during adenosine infusion (140 microg/kg x min(-1) for 5 minutes), 30 minutes before and 15 minutes after administration of L-PC (300 mg IV in 5-minute bolus).
  
  
  RESULTS
  Thirty-three patients were screened for this study. Fourteen patients (mean [SD] age, 54.3 [11.2] years; mean [SD] weight, 63.8 [14.5] kg; mean [SD] height, 156.3 [8.7] cm) with SSc and no evidence of coronary heart disease were included in the study; 13 women and 1 man (4 with the diffuse cutaneous form of SSc and 10 with the limited cutaneous form). After administration of L-PC to patients with SSc, median CFVR was significantly increased from 2.60 to 3.23 (P < 0.001), whereas peak diastolic velocity in the LADCA decreased significantly at the basal evaluation (30.0 vs 26.0, P = 0.009) and significantly increased (80.0 vs 87.5, P = 0.005) during adenosine infusion. No adverse events occurred before, during, or after L-PC infusion.
  
  
  CONCLUSIONS
  Acute administration of L-PC was associated with a short-term beneficial effect on CFVR in this pilot study of patients with SSc. These results suggest that further, randomized, controlled, double-blind evaluation of longer-term administration to patients with SSc should be considered.
authors: Department of Cardiovascular and Neurological Sciences, University of Cagliari, Cagliari, Italy. rmontis@tin.it
authors: Roberta
authors: R
authors: Montisci
authors: Massimo
authors: M
authors: Ruscazio
authors: Stefania
authors: S
authors: Lai
authors: Alessandra
authors: A
authors: Vacca
authors: Alberto
authors: A
authors: Cauli
authors: Giuseppe
authors: G
authors: Passiu
authors: Massimo
authors: M
authors: Montisci
authors: Luigi
authors: L
authors: Meloni
authors: Alessandro
authors: A
authors: Mathieu
authors: Sabino
authors: S
authors: Iliceto
mesh_terms: D000241:Adenosine; D000328:Adult; D000368:Aged; D001783:Blood Flow Velocity; D002316:Cardiotonic Agents / Q000009:adverse effects / Q000494:pharmacology*; D002318:Cardiovascular Diseases / Q000188:drug therapy* / Q000209:etiology; D002331:Carnitine / Q000009:adverse effects / Q000031:analogs & derivatives* / Q000494:pharmacology; D003326:Coronary Circulation / Q000187:drug effects*; D003331:Coronary Vessels / Q000187:drug effects / Q000503:physiopathology; D015150:Echocardiography, Doppler; D005260:Female; D006801:Humans; D007275:Injections, Intravenous; D008297:Male; D008833:Microcirculation / Q000187:drug effects / Q000503:physiopathology; D008875:Middle Aged; D009206:Myocardium / Q000378:metabolism; D010865:Pilot Projects; D012595:Scleroderma, Systemic / Q000150:complications* / Q000503:physiopathology
chemical_list: D002316:Cardiotonic Agents; C003223:propionylcarnitine; D000241:Adenosine; D002331:Carnitine