分離型肺動脈瓣缺失症候群：病例報告及文獻回顧

肺動脈瓣缺失症候群是一相當少見，但對生命有極大威脅的心臟畸型，它包括肺動脈瓣發育不全，肺動脈瓣環狹窄及極度擴大的肺動脈幹。這類畸型多半與心室中隔缺損或法洛氏四合群症合併存在，少見單獨存在型。臨床上這類病嬰早期多以呼吸窘迫，發紺來表現。文獻報告，無論以內科或外科方法治療，死亡率都相當高。本院最近發現一位嬰幼兒，出生不久即發生呼吸窘迫，發紺的徵象，物理檢查可於左側胸骨緣聽到第四度連續性雜音；心電圖檢查顯示心軸偏右，右心室擴大；胸部X光顯示心臟擴大，經超音波及心導管心臟血管攝影檢查，證實為單純型肺動脈瓣缺失症候群。由於此類病例相當罕見，特回顧文獻提出報告。

關鍵字

肺動脈瓣缺失症候群；分離型

並列摘要

The absent pulmonary valve syndrome is a rare, life-threating cardiac malformation which includes rudimentary pulmonary valvular leaflets, narrow pulmonary annular ring and marked dilation of pulmonary trunk. The syndrome is rarely an isolated lesion, but more commonly associates with ventricular septal defect or tetralogy of Fallot. The symptomatic infants may exhibit respiratory distress, cyanosis and congestive heart failure. Decision-making for proper therapy is still a problem. The prognosis is poor.Recently, we found a case with isolated form of absent pulmonary valve syndrome proved by echocardiography and angiocardiography. We reported and discussed this case with reference review.

並列關鍵字

Absent pulmonary valve syndrome ； Isolated form

國際替代計量

分離型肺動脈瓣缺失症候群：病例報告及文獻回顧

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