Objective: The serum concentration of amino-terminal procollagen type III (PIIIP) is considered a useful marker of tissue fibrogenesis. The present study tested the hypothesis that: 1) serum PIIIP levels are elevated in patients with congenital heart disease (CHD) and abnormal hemodynamic loading and/or hypoxemia, 2) PIIIP levels are associated with severity of hemodynamic load or hypoxemia, both of which enhance myocardial fibrosis.
Methods and results: Serum PIIIP levels were measured in 5 groups of CHD patients [42 patients with ventricular septal defect (VSD), 26 with coarctation of the aorta (COA, n=19) or aortic stenosis (AS, n=7), 36 with atrial septal defect (ASD), 39 with pulmonary stenosis (PS) and 20 with tetralogy of Fallot (TOF)]. PIIIP levels of CHD patients were significantly higher than those of 42 control subjects (p<0.05, each). Serum PIIIP levels increased in parallel with increased ventricular volume load in VSD and ASD, and with severity of PS. In TOF patients, PIIIP levels correlated negatively with arterial oxygen saturation. Treatment with angiotensin converting enzyme inhibitor (ACEI) was associated with low levels of PIIIP in COA/AS patients despite existing hemodynamic load.
Conclusion: The increased serum PIIIP levels in proportion with the severity of ventricular load or cyanosis suggest enhanced myocardial synthesis of collagen type III in patients with CHD. Suppression of PIIIP level by ACEI suggests the involvement of the renin-angiotensin-aldosterone system in myocardial fibrosis. These data provide the basis for the development of new diagnostic and therapeutic strategies in patients with CHD.
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