臨床的にKartagener症候群が疑われた完全内臓逆位，円錐中隔全欠損，単冠状動脈

A New-born Case of Total Conus Defect and Single Coronary Artery with Situs Inversus Totalis Suspected Kartagener Syndrome

Kartagener症候群は内臓逆位，気管支拡張症，慢性副鼻腔炎を三徴とし，常染色体劣性遺伝と考えられる原発性線毛機能不全症候群の範疇に入るとされており，発生頻度は16,000人に1人1）といわれている．先天性の線毛器官構造異常による慢性上下気道感染や不妊症，角膜異常，網膜色素変性症の発症が多い反面，心内病変合併例の報告はまれである．確定診断は染色体検査により行われるのが通常であるが，われわれは臨床的に本症候群が疑われ，完全内臓逆位と円錐中隔全欠損を合併した1例を経験したので，文献的考察を含め報告する．

We report a new-born case of total conus defect type ventricular septal defect （VSD） and single coronary artery with situs inversus totalis, suspected Kartagener syndrome clinically. After the birth, as the patient had suffered from respiratory distress due to high pulmonary blood flow through the large defect, surgery was planned at age of 14-days after birth. Under median sternotomy and cardiac arrest, patch closure of VSD was performed as ordinary fashion. In spite of the situs inversus totalis and single coronary artery arose from right coronary sinus, operator could have completed all of surgical procedure at the right side of patient as usual. No remarkable respiratory complication was seen postoperatively and she was discharged from hospital 18th day in a good condition.