2012-08-012024-05-13https://scholars.lib.ntu.edu.tw/handle/123456789/655257摘要：左心室是心臟中最重要的一個結構，在正常情況下它可以有效並強健的維持全身體循環。在眾多先天性心臟病中，雙心室修復術乃是能將此一缺損心臟達到完全矯正的理想最後目標。然而，在有一群以右側心室為主的不平衡心室型先天性心臟中（例如：右心室雙出口，左心發育不全症候群，體位異常、、、、等）的病患中，以雙心室修復術矯治的預後仍然不明[1-7]。這最主要原因出自於，無法能在手術前以具體的標準預測怎樣情況的左心室，在雙心室修復術後真的能夠扮演一個稱職左心室的功能[8-11]。取而代之的，這樣的病人被迫去接受全體靜脈接到肺動脈的循環模式[12-13]。然而，這樣的Fontan 循環模式，在已有的長期追蹤文獻上，顯現出比那些能夠接受雙心室修復術的人，存活率較低，且生活品質也較差[14-18]。顯影增強並心電圖同步的心臟電腦斷層掃描已被證實，得以幫助先天性心臟病患者，獲得相當清晰的解剖構造以及功能性分析[19-25]。早在民國八十四年起，本院是國內第一家引進心臟電腦斷層掃描儀的醫院，因而也累積了眾多的個案，成為擁有全國最大的先天性心臟病相關的電腦斷層影像數位資料庫。迄今，本資料庫中有超過700 例個案是屬於上述三大疾病的右側為主的不平衡心室型先天性心臟病。本院自民國九十七年起，也是全國第一家設立整合型成人期先天性心臟門診的醫院。因而，我們更能夠追蹤到這些小時候開刀矯治修復過的長期病人，了解他們目前的情況。本團隊曾被天下雜誌評定是本國最佳的先天性心臟病醫療團隊。更由於本院是本國第三級的後送醫院，因而我們常常需要處理從他院轉來的複雜性先天性心臟病。在這些個案中，本團隊在未來三年期的計畫中，平均每年應可以再增加40 名左右的右側為主的不平衡心室型先天性心臟病，因而個案數相當充足。為了能達到如何能在雙心室修復術前，能有效的預測當下左心室的品質及潛力，本計畫嘗試分析在完全矯治前各個時期左心室的參數。我們希望能建立起一些基於心臟電腦斷層掃描影像上的準則，來協助臨床醫師，在決策這些以右側為主的不平衡心室型先天性心臟病中，能否接受雙心室修復術，以及其預後。本三年期的計畫中，第一年藉由回溯性的分析，是要先建立起在心臟電腦斷層掃描中，以年齡為相關的各種正常左心室特性的變數曲線。第二年則是用來建立能否接受雙心室修復術的左心室最低條件。第三年，則是將已成功完成雙心室修復術的以右側為主的不平衡心室型先天性心臟病個案找出來，分析連續幾次的心臟電腦斷層掃描影像，並由此影像所建立的左心室形狀模型（shape model），的重新塑造（remodeling）分析中，找出左心室型變的趨勢，用以探討其左心室能恢復功能的潛在機轉。藉由上述三年的計畫執行，最終，我們想藉由心臟電腦斷層掃描此一可靠工具，提供一個清楚的準則，讓在此類病人於左心室準備的不同時期中，能診斷當下左心室的品質及潛力是否能承擔起未來計畫中的雙心室修復術，以更進一步的提升長期的預後。<br> Abstract: Left ventricle (LV) is the most important chamber of heart; it effectively and strongly supportsthe systemic circulation when in normal function. Bi-ventricular repair is the final purpose fortotal correction of the congenital heart disease (CHD). However, the prognosis of thebiventricular repair for right-dominant unbalanced ventricles (such as double outlet of rightventricle; hypoplastic left heart syndrome; heterotaxy…etc.) remains uncertain[1-7]. Becausethere is no solid criteria before surgical correction to predict the quality of the LV in the futurefor playing a sustainable role after biventricular repair[8-11]. The trade-off strategy is goingfor total cava pulmonary circulation in such case[12-13]. However, the survival and lifequality of patient with Fontan circulation is not good after long term follow up[14-18].Contrast-enhanced ECG-gating cardiac computed tomography (CT) has demonstrated itspowerful ability on structural delineation and functional analysis on the CHD[19-25]. Ourinstitute is the first hospital in this country to have ability to get clear cardiac CT, so weaccumulate has the largest database of CHD CT images in Taiwan since 1995. Up to now, wehave more than 700 CHD cases with right-dominant unbalanced ventricles. We also have the1st integrated adult CHD clinic in Taiwan since 2008, and have collected a lot of adult caseswith long term clinical experience after surgical correction in their childhood. Our team hasbeen evaluated as the number one CHD team in this country. As a tertiary referring center, wereceived the most number of complex CHD patients from other hospital in this country.Among them, we will have another 40 cases with right-dominant unbalanced ventricles peryear in average.For providing an effective prediction on the LV quality for biventricular repair, we will like toanalyze variables on cardiac CT of the LV at different stages before total surgical correction.We hope we can set up a guideline to help clinician in predicting the outcome of thebiventricular repair on these CHD with the right-dominant unbalanced ventricles. In this3-year project, we will build age-related normal reference curves of LV variables on cardiacCT in the first year. In the second year, we will establish the lowest-tolerance limit ofreference curves of LV variables on cardiac CT as guideline of bi-ventricular repair. And, inthe third year, by analyze the characters of remodeling of the LV shape models after favorablebiventricular repair in such right-dominant unbalanced ventricles, we will like to disclose itsunderlying recovery mechanism. Finally, at the end of this project, we will provide clearguidelines by cardiac CT at different stages of the LV preparing about its adequacy to dointended biventricular repair in order to improve their long term outcome.右側為主的不平衡心室型先天性心臟病心臟電腦斷層掃描雙心室修復術指引right-dominant unbalanced ventriclescardiac computed tomographyguideline of biventricular repair