Maeda Katsuhide, Yamaki Shigeo, Nishiyama Mitsunori, Murakami Yasuo, Takahashi Yukihiro, Takamoto Shinichi
Department of Cardiothoracic Surgery, Faculty of Medicine, University of Tokyo, Tokyo, Japan.
Jpn J Thorac Cardiovasc Surg. 2003 Sep;51(9):430-3. doi: 10.1007/BF02719596.
A 15-year-old boy with a ventricular septal defect, pulmonary hypertension, Down's syndrome, and extremely thickened media (ETM) of the small pulmonary arteries died of heart failure and pulmonary hypertension 13 years after intracardiac repair. Microscopic examination of lung specimens collected prior to the intracardiac repair and at the time of autopsy revealed that the ETM had remained unchanged and that the arteries connected to the vessels with ETM had become severely thickened. The present case shows that even a small percentage of arteries with ETM can cause pulmonary hypertension, and illustrates one of the mechanisms of how pulmonary hypertension can fail to be resolved after intracardiac repair.
一名患有室间隔缺损、肺动脉高压、唐氏综合征且小肺动脉中层极度增厚（ETM）的15岁男孩，在心脏内修复13年后死于心力衰竭和肺动脉高压。对心脏内修复前及尸检时采集的肺标本进行显微镜检查发现，ETM没有变化，且与存在ETM的血管相连的动脉已严重增厚。本病例表明，即使只有一小部分动脉存在ETM也可导致肺动脉高压，并说明了心脏内修复后肺动脉高压无法缓解的一种机制。
