Itoi Toshiyuki, Satou Hisashi, Hamaoka Kenji
Department of Pediatric Cardiology and Nephrology, Graduate School of Medical Science, Kyoto Prefectural University of Medicine, Kyoto, Japan.
Circ J. 2007 Sep;71(9):1492-5. doi: 10.1253/circj.71.1492.
Coronary artery multiple fistulae of the left ventricular chamber are extremely rare congenital malformations. We report on 13-year-old monozygotic twin brothers who have identical abnormalities of coronary flow reserve as well as the same morphological findings of multiple coronary fistulae of the 3 major coronary arteries to the left ventricular chamber. The left circumflex coronary artery (LCX) had abundant fine communications with the left ventricular chamber and had a higher flow rate than the left anterior-descending coronary artery. The coronary flow reserve obtained from the LCX was lower than the normal value matched for the same age group. The increased shunt flow resulted in enlargement of the left ventricular chamber. This is the first report of coronary flow characteristics in children with multiple coronary fistulae.
冠状动脉左心室多发瘘是极其罕见的先天性畸形。我们报告了一对13岁的同卵双胞胎兄弟,他们具有相同的冠状动脉血流储备异常,以及三大冠状动脉至左心室的多发冠状动脉瘘相同的形态学表现。左旋冠状动脉(LCX)与左心室有丰富的细微交通,且血流速度高于左前降支冠状动脉。从LCX获得的冠状动脉血流储备低于同年龄组匹配的正常值。分流增加导致左心室扩大。这是关于多发冠状动脉瘘患儿冠状动脉血流特征的首次报告。