Right Ventricle-Dependent Coronary Circulation in Pulmonary Atresia with Intact Ventricular Septum: A Case Report

存在於肺動脈瓣閉鎖合併完整心室中隔的依賴右心室的冠狀動脈循環－病例報告

李孟勳(Meng-Hsun Lee) ； 吳俊仁(Jiunn-Ren Wu) ； 戴任恭(Zeng-Kong Dai) ； 徐仲豪(Jong-Hau Hsu) ； 邱肇基(Chau-Chi Chou) ； 陳懷民(Huai-Min Chen)

《The Kaohsiung Journal of Medical Sciences》 21卷5期 (2005/05) Pp. 236-240

摘要

肺動脈瓣閉鎖合併完整心室中隔(pulmonary atresia with intact ventricular septum)具有多樣性的形態表現，此種異常佔了嚴重先天性心臟病的l-3%。僅管外科手術方法日新月異，不過其死亡率仍然偏高。最適當的手術方法取決於右心室及三尖瓣的形態與大小，也要考量是否有依賴右心室的冠狀動脈循環(right ventricle-dependent coronary circulation)存在。所以，正確的了解病人形態學及血液動力學上的特徵，尤其是冠狀動脈的異常，對於治療方式的選擇是非常重要的。我們報告一位出生後不久即出現發紺的足月產女嬰。心臟超音波檢查顯示肺動脈瓣閉鎖合併完整心室中隔及右心室發育不全。心導管檢查證實心臟含超音波的診斷且更進一步發現依賴右心室的冠狀動脈循環。在詳盡了解病人形態學及血液動力學上的特徵之後，我們選擇為病人進行血管分流術(modified Blalock-Taussig shunt)，初步的治療效果是很令人滿意的。

關鍵字

肺動脈瓣閉鎖合併完整心室中隔 ； 依賴右心室的冠狀動脈循環

並列摘要

Pulmonary atresia with intact ventricular septum (PAIVS) is a morphologically heterogeneous lesion and accounts for 1-3% of critically ill infants with congenital heart disease. Numerous surgical approaches have been attempted with varying degrees of success, but the mortality rate is still high in most series. The optimal surgical procedure depends on the size and morphology of the tricuspid valve and right ventricle and the presence or absence of right ventricle-dependent coronary circulation. Therefore, it is pivotal to define the precise morphologic and hemodynamic characteristics, especially coronary artery anatomy. In this report, we describe a full-term female neonate with cyanosis soon after birth. Two-dimensional and color Doppler echocardiography corroborated the diagnosis of PAIVS and showed a small right ventricle. Cardiac catheterization indicated PAIVS and further revealed right ventricle-dependent coronary circulation. A systemic-to-pulmonary artery shunt was constructed with a positive immediate result.

並列關鍵字

pulmonary atresia with intact ventricular septum ； right ventricle-dependent coronary circulation