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Objectives To evaluate the changes of serum intact terminal peptide of procollagen in patients with chronic Keshan disease (KD) and investigate their clinical significance. Methods The concentrations of serum intact N-terminal peptide of type Ⅰ procollagen (P Ⅰ NP) and intact N-terminal peptide of type Ⅲ procollagen (P Ⅲ NP) were measured by radioimmunoassay in 35 patients with chronic KD and 31 normal control. Doppler ultrasounds was used to determine several parameters of left ventricular systole and diastole functions. Results The concentration of serum P Ⅰ NP (74.07±16.74)μg/L and the ratio of P ⅠNP/ P Ⅲ NP (18.02±4.60) in chronic KD were significantly increased as compared to the control (39.63±12.07 μg/L, 12.12±4.24; P ＜ 0.001). Serum P Ⅲ NP (4.19±0.64)μg/L in chronic KD was higher than that in the control (3.36±0.65 μg/L,P ＜ 0.001)too. The higher of serum concentration of P Ⅰ NP and the ratio of P Ⅰ NP/ P Ⅲ NP, the worse of cardiac function in patients with chronic KD. A negative correlation was found between serum P Ⅰ NP/P Ⅲ NP,P Ⅰ NP and VE/VA, LVEF(γ=-0.4502, -0.4608, P＜0.01 and γ=-0.3936, -0.3904, respectively; P ＜ 0.05).Conclusions These findings suggested that tissue synthesis of collagen type Ⅲ and type I was abnormally increased in chronic KD. On the other hand, our results indicated that P Ⅰ NP and P Ⅲ NP were related to several functional alterations of the left ventricle. Serum procollagen peptide measurements might therefore provide indirectly diagnostic information on myocardial fibrosis associated with chronic KD.