Quantitative analysis of pulmonary vascular disease in total anomalous pulmonary venous connection in sixty infants.
Quantitative analysis of pulmonary vascular disease in total anomalous pulmonary venous connection in sixty infants.
复制标题
60例婴儿完全性肺静脉异位引流肺血管疾病的定量分析
DOI：
10.1016/s0022-5223(19)34743-9
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发表时间：
1992
影响因子：
6
通讯作者：
H. Mohri
中科院分区：
文献类型：
--
作者：
S. Yamaki;Minora Tsunemoto;M. Shimada;R. Ishizawa;M. Endo;Shingo Nakayama;Masaki Hata;H. Mohri
我们对肺静脉连接完全异常的尸检病例进行了小肺动脉、肺静脉和淋巴管的定量分析。资料来自60例肺静脉连接完全异常，无脾功能不全或肺狭窄，死亡时年龄从2天到19个月不等（平均年龄2.2个月）。其中32例患者在死亡前测量了肺动脉压。以室间隔缺损合并肺动脉高压患者20例，正常人15例为对照组。肺静脉连接全异常组肺动脉小动脉和小静脉中膜平均厚度分别为12.7 μm和7.6 μm，明显大于正常组和室间隔缺损组。随着老化，厚度没有变化。肺静脉阻塞患者的动脉和静脉内侧厚度大于无肺静脉阻塞的患者。肺静脉连接完全异常病例的小肺动脉内侧厚度与肺动脉压升高相关。当肺动脉压水平相同的患者进行比较时，肺静脉连接完全异常的患者的内侧厚度总是大于室间隔缺损的患者。肺静脉内侧厚度也与全异常肺静脉连接肺动脉压升高高度相关。与室间隔缺损患者相比，肺静脉连接完全异常的患者内膜病变的严重程度较轻，提示增厚的肺动脉中膜对内膜病变的发展具有保护作用。室间隔缺损病例中未见肺静脉内膜纤维增厚，但在全部肺静脉连接异常病例中有45%出现。62%的异常肺静脉连接病例以淋巴管扩张为特征性表现。间质性肺气肿通常是淋巴管扩张的并发症，并导致8例术后死亡。(J ThoracCardiovascSurg1992; 104:728-35)
A quantitative analysis of small pulmonary arteries, pulmonary veins, and lymphatic vessels was conducted in autopsy cases of total anomalous pulmonary venous connection. The materials were obtained from 60 cases of total anomalous pulmonary venous connection without asplenia or pulmonary stenosis, ages ranging from 2 days to 19 months at the time of death (mean age 2.2 months). Pulmonary arterial pressure had been measured in 32 of these patients before death. Twenty cases of ventricular septal defect with pulmonary hypertension and 15 normal individuals were used as the control group. The mean thickness of the media of small pulmonary arteries and veins was 12.7 and 7.6 μm, respectively, in the total anomalous pulmonary venous connection cases, both values being significantly larger than those for normal and ventricular septal defect cases. No changes in thickness with aging were found. Medial thickness in the arteries and veins was greater in the cases of pulmonary venous obstruction than in those without such obstruction. The medial thickness of small pulmonary arteries in total anomalous pulmonary venous connection cases correlated with increased pulmonary arterial pressure. When the patients with the same pulmonary arterial pressure levels were compared, the medial thickness was always greater in those who had total anomalous pulmonary venous connection than in those who had ventricular septal defect. The medial thickness of pulmonary veins was also highly correlated with increased pulmonary arterial pressure in total anomalous pulmonary venous connection. The severity of the intimal lesions was milder in those who had total anomalous pulmonary venous connection than in those who had ventricular septal defect, suggesting the protective role of the thickened pulmonary arterial media against development of intimal lesions. Intimal fibrous thickening of pulmonary veins was not seen in the cases of ventricular septal defect, but it was present in 45% of the total anomalous pulmonary venous connection cases. Lymphangiectasia was characteristically present in 62% of the total anomalous pulmonary venous connection cases. Interstitial emphysema was often a complication of lymphangiectasia, and it led to eight postoperative deaths. (J ThoracCardiovascSurg1992;104:728-35)