Ventricular fibrogenesis activity assessed by serum levels of procollagen type III N-terminal amino peptide during the staged Fontan procedure

The authors measured serum amino-terminal procollagen III (PIII) as a marker of myocardial fibrogenesis in patients undergoing the Fontan procedure for single ventricle circulation and in controls.  Patients with single venticle circulation had elevated PIII levels compared to controls.  Staged Fontan elements successively reduced PIII levels.   The levels of volume load and cyanosis were related to PIII levels.  Persisently elevated PIII levels were related to venticular stiffening.  PIII may be a useful marker of disease state and could indicate a therpeutic target.