Uchita Shunji, Harada Yorikazu, Honda Kentaro, Toguchi Koji, Nishimura Yoshiharu, Suenaga Tomohiro, Takeuchi Takashi, Suzuki Hiroyuki, Okamura Yoshitaka
Department of Thoracic and Cardiovascular Surgery, Wakayama Medical University, 811-1 Kimiidera, Wakayama city, Wakayama, 641-8509, Japan.
J Cardiothorac Surg. 2013 May 28;8:136. doi: 10.1186/1749-8090-8-136.
We report a successful staged repair for a quite rare combination of truncus arteriosus (TA), Van Praagh type A4, and abnormal origin of the left coronary artery (CA). Furthermore, the case was complicated by a variant of the chromosomal anomaly in cat-cry syndrome. The presence of interruption of the aortic arch (IAA) and abnormal CA origin has been previously reported to increase mortality. To decrease the risk of bronchomalacia in infants, bilateral pulmonary artery banding (PAB) was performed as the first stage procedure for adjusting the pulmonary flow. Staged repair is a useful strategy for infants with complex TA.
我们报告了一例成功的分期修复手术，该病例为罕见的动脉干（TA）Van Praagh A4型合并左冠状动脉（CA）异常起源。此外，该病例还合并了猫叫综合征染色体异常的一种变异型。此前有报道称，主动脉弓中断（IAA）和CA异常起源会增加死亡率。为降低婴儿发生支气管软化的风险，作为第一阶段手术进行了双侧肺动脉环扎术（PAB）以调节肺血流量。分期修复是治疗复杂TA婴儿的一种有效策略。
