内皮素在左向右分流型先天性心脏病肺动脉高压中作用的研究

A study on the role of endothelin 1 in pulmonary hypertension secondary to congenital heart defects with left to right shunt

为探讨内皮素在左向右分流型先天性心脏病（简称先心病）肺动脉高压（简称肺高压）中的作用，采用放射免疫法测定５２例肺高压和１１例非肺高压先心病患儿腔静脉和肺动脉血浆内皮素（ｉｒＥＴ－１）水平，并对其中１０例肺高压患儿进行肺活检免疫细胞化学染色。结果，肺高压患儿肺动脉和腔静脉血浆ｉｒＥＴ－１水平均随肺动脉压力升高而增加，并高于非肺高压患儿（Ｆ＝２．９５；３．３９，Ｐ＜０．０５）。肺高压患儿血浆ｉｒＥＴ－１与肺动脉压力及阻力成正相关（ｒ＝０．６５；０．５８，Ｐ＜０．０１）。肺高压患儿肌性和弹性肺小动脉ｉｒＥＴ－１染色明显加深。研究提示，左向右分流型先心病患儿内皮素产生的增加可导致肺高压患儿肺血管的中膜肥厚和内膜增生，促进了肺高压的发展。

To investigate the role of endothelin 1 (ET 1) in congenital heart defects (CHD) assoicated with pulmonary hypertension (PH), the authors measured the plasma irET 1 level in superior vena cava (SVC) and pulmonary artery (PA) in 52 cases of PH secondary to CHD with left to right shunt and 11 non PH patients by radioimmunoassay. Also, the immunocytochemical staining of lung biopsy was analyzed in 10 cases of PH. The irET 1 level elevated along with an increase in pulmonary artery systolic pressure (PASP) both in SVC and in PA in PH patients, and was higher than that in non PH group. The concentration of irET 1 correlated positively with PASP and pulmonary vascular resistance (PVR) ( r =0.65, 0.58, P <0.01), respectively. The ET 1 like staining of muscular and elastic pulmonary artery was abundant in PH group compared with the controls. These results suggest that ET 1 play an important role in the medial hypertrophy and intimal hyperplasia of the pulmonary artery and promote the progression of PH in cases of CHD.