2015-08-012024-05-14https://scholars.lib.ntu.edu.tw/handle/123456789/657724摘要：肺動脈瓣閉鎖合併心室中膈無缺損(pulmonary atresia with intact ventricular septum PAIVS)因病人右心室發育狀況不同及合併結構上不同的問題而有不同的治療方式，目前的治療方式有三種，1.開刀 2.心導管治療 3.內外科協同治療(Hybrid)，每種治療有其優缺點，開刀治療的缺點是死亡率及發生併發症比率較高，住院日長，而且外科手術治療常常不是一次就好，有的還要多次開刀，心導管則是併發症少但失敗率稍高，心導管治療失敗率約5-10%，但是還可以轉為開刀治療，hybrid則介於兩種方式之間，但是目前大部分僅為個案報告，而且只能用於右心室發育良好的病人。本研究將訂定一個經由心導管治療的策略並評估短中期之效益，以及用歷史性的資料比較開刀與心導管治療之效果，包括成功率、死亡率、右心室發育、右心室功能，殘留壓力差以及是否還需要另外一次開刀來評估心導管的治療策略是否優於傳統的開心手術。依照病人出生後的右心室發育狀況來決定治療方式，分為發育尚可(mild hypoplasia)，稍不良(moderate hypoplasia)及發育很不好(severe hypoplasia)三組，右心室發育的狀況以胸前超音波來評估，包括三尖瓣的直徑，用apical 4 chamber view以及三尖瓣至心尖部的長度，以及右心室出口漏斗部的最大寬度肺動脈瓣的直徑，並以心導管右心室的攝影測量值當佐證，以三尖瓣直徑Tricuspidvalve (TV) annulus來代表右心室的發育程度以對照表量Z值，我們定義TV Z值 ≥ -1.5為mild hypoplasia(group I)，TV Z值在-1.5及-3之間為moderate hypoplasia(group Ⅱ)，TV Z值 < -3則為severe hypoplasia (group Ⅲ)。對於mild hypoplasia (group I)病人使用高週波導線radio frequency或高硬度尖銳導線穿破閉鎖的肺動脈瓣膜，再做氣球擴張，group Ⅱ moderate hypoplasia那組除了心導管擴張外加上PDA stenting，保持PDA之暢通，group Ⅲ只做PDA stenting，術後一天、七天以及一個月，三個月之後每三個月評估右心室的TV Z值，統計心導管這三組的併發症發生率死亡率，以及是否需要另外一次開刀，並以回溯方式統計過去十年間PA-IVS的病人開心手術之併發症比率。我們將探討這種策略在三組之間成功率、失敗率、併發症以及住院日數之比較，以及多少病人需要再度開刀，更重要的是右心室是否可以隨年齡而成長。<br> Abstract: Management of pulmonary atresia with intact ventricular septum (PA-IVS)is determinated basing on morphology & size of right ventricle and associated anomalies. Traditional therapy of PA-IVS is surgery. Recently, transcatheter treatment is applied to treat PA-IVS in selected patients. Hybrid therapy is also advocated in several case reports. There are advantages and disadvantages in each modality of treatment. Traditional operation carries a higher complication rate and mortality rate.In this study, we will evaluate the safety and efficacy of using a non-surgical strategy for treatment of PA-IVS with various RV size & morphology. In the past 10 years, patients who were managed with surgical technique is this institution will be used as historical controls. Transcatheter treatment strategy varies according to morphology and size of right ventricle. PA-IVS patients will be divided into 3 groups. Group I consists of newborns with mild hypoplasia of right ventricle. Group Ⅱ& Ⅲ consists of newborns with moderate hypoplasia and severe hypoplasia of right ventricle, respectively.The right ventricle size was measured on echocardiography. Tricuspid valve annulus is obtained from apical 4 chamber view, RV inlet & outflow tract dimension will be measured. Pulmonary valve annulus will be obtained from parasternal short axis view. Measurement from right ventricular angiography will be used as complementary information. Those with TV Z score > -1.5 is defined as mild hypoplasia RV (group I), TV Z score < 1.5, but > -3 as moderate hypoplasia (group Ⅱ) & TV Z score > -3 as severe hypoplasia (group Ⅲ).In group I patients, transcatheter perforation of pulmonary valve will be performed using radiofrequency guide wire or a stiff end coronary guide wire. Balloon dilation will be subsequently performed. In group Ⅱ, PDA stenting will be performed in addition to balloon dilation of pulmonary valve. In group Ⅲ, only PDA stenting will be performed in those patients assigned to single ventricle repair.We will compare the results of this strategic approach to those who underwent surgical repair in the past 10 years requiring complication rate, mortality rate & reintervention rate. Right ventricular development will also be compared between those pre-intervention and final visit.