左単冠状動脈，心室中隔欠損症を伴ったDiscrete型右心室漏斗部狭窄症の1例

A Case of an Isolated Discrete Infundibular Stenosis Accompanied with a Ventricular Septal Defect and Left Single Coronary Artery

　右心室漏斗部狭窄症は漏斗部中隔が逸脱し，輪状柱稜部分に輪状狭窄を来すことにより発生するといわれ，心室中隔欠損症を伴うものが大部分であり，心室中隔が正常である純型漏斗部狭窄症は稀である．形態的にはDiffuse型とDiscrete型に分類されるが，前者が大半を占め後者は稀である．われわれは，左単冠状動脈を伴った純型Discrete型漏斗部狭窄症の診断で経肺動脈的に狭窄を解除し，術後，初めて心室中隔欠損症の存在が明らかになった症例を経験した．臨床経過から心室中隔欠損の自然閉鎖に伴って二次的に生じたDiscrete型漏斗部狭窄症と思われたので，その発症機構について若干の文献的考察を加えて報告する．

An infundibular pulmonic stenosis with intact ventricular septum is an unusual cardiac abnormality which is considered to be a primary congenital defect.

In morphology, diffuse types are rather common but discrete types are rare. Initially, under the diagnosis of an isolated discrete infundibular stenosis with a left single coronary artery, stenotic tissues were able to be resected with ease through the trans - pulmonicapproach. Postoperatively, we noticed that a minor ventricular septal defect had been present. It would be thought more natural if the discrete infundibular pul-monic stenosis had developed gradually with a sponta-neous evolutional closure of the ventricular septal defect rather than that those two factors should havecoexisted since birth.