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心室中隔正常之肺動脈瓣閉鎖症－病例報告
Pulmonary Atresia with Intact Ventricular Septum: A Case Report
摘要
雖然心室中隔正常之肺動脈瓣閉鎖症（pulmonary atresia with intact ventricular septum）是一相當少見的先天性心臟病，但是在患有發紺性心臟病之新生兒中，却有30%單獨或合併有此症之存在。此病症經常合併其他解剖上的異常，包括三尖瓣、右心室出口，以及開放性動脈導管。最近本院發現一典型病例，由於相當罕見，特別提出報告，並回顧文獻探討解剖異常的多變性及治療的方針。
關鍵字
心室中隔正常之肺動脈瓣閉鎖症並列摘要
Although pulmonary atresia with intact ventricular septum is not a common lesion, among all neonates with cyanotic congenital diseases, those with pulmonary atresia with intact ventricular septum account for nearly 30 percent. This rare defect may be associated with other anatomic anomalies in various locations including tricuspid valve, right ventricular outflow tract, ductus arteriosus, etc. This report presents a new-born baby who suffered from cyanosis after delivery. Cardiac catheterization and cineangiography revealed pulmonary atresia with intact ventricular septum and fistulous communications between right ventricular myocardial sinusoids and the coronary circulation. Blalock-Taussig shunt was performed at his of 20 days, but the patient died of persistent hypoxia eventually. Autopsy confirmed the diagnosis and also disclosed right ventricular hypertrophy. This case was reported because it is rare. Literatures were reviewed, and, anatomical variations guidelines of treatment were also discussed.
延伸閱讀
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