2010-08-012024-05-13https://scholars.lib.ntu.edu.tw/handle/123456789/648767摘要：Fontan 手術適用於單一心室的病人之部分矯正，病人在術後大部分可存活，不過心臟功能還是受到限制，而且併發症比率仍高。本研究的目的如下:1.研究Fontan 病人術後長期追蹤的結果存活率、併發症的種類以及發生率2.Fontan 手術後病人的心臟功能評估包括心室射出率，心室的容舒張及收縮期的容積，瓣膜逆流程度，血中B 型利鈉胜肽濃度與併發症發生的關係3.心律不整發生率，那一種心律不整 4.心肺及運動功能5.肝、腎功能發生異常的比率6.手術的類別以及心室的型態McGoon ratio與心臟功能及併發症的關係7.關閉殘留缺口(fenestration)及側支循環(systemic-to-pulmonary venous collaterals)是否改善心肺功能8.心導管治療肺動脈狹窄或Fontan 通道狹窄是否可改善蛋白流失腸病變或心衰竭。本研究將納入自 1990 年起至2007 年止，在本院執行Fontan 術式的病人，約150 名左右，以三年時間完成。第一年完成基本資料登錄，第一年年齡、性別以單心室的型態分組(左心室右心室及其他共三組)，並登錄開刀方式a.傳統Fontan b.納入右心室(right ventricle inclusion 或稱Bjork modification)的循環c.全靜脈與肺動脈吻合術totalcavopulmonary connections d.lateral tunnel 或extracardiac tunnel，並調閱病歷，病人是否存活以及是否有重大併發症，若病人失聯，可用電話或問卷查詢回門診追蹤，評估併發症有無及類別。Fontan 病人並分為兩組，第一組為沒有心臟併發症的病人，第二組為有心臟相關併發症。每位病人接受心臟超音波檢查，Holter 心電圖，抽血測定B 型利鈉胜肽、completeblood count、肝腎功能及protein C、protein S。並用核磁共振顯像(MRI)或電腦斷層(CT)測量病人的舒張及收縮容積及射出率，以及病人是否有Fontan 通道或肺動脈狹窄，並評估病人的心肺功能，並分析兩組間各種不同parameter 的差異。並探討發生併發症之因素，第二、三年則針對有殘留缺口(fenestration)，側支靜脈(venous collaterals)或Fontan 通路、肺動脈狹窄是否可以增加心肺功能，以及改善症狀，若病人有殘留缺口及側支靜脈，則評估是否需要關閉，若血氧濃度在90%以下，Hct > 50 或有潛在不良影響，則建議以心導管方式關閉，fenestration 以Amplatzer septaloccluder，側支靜脈以coil 或vascular plug 關閉。在關閉術後六個月再評估心肺功能，並找出蛋白流失腸病變的病人，查看其心臟功能的狀況以及其Fontan 通道是否阻塞，若有阻塞則進行心導管擴張或併用血管支架治療，並評估治療前後之生化學檢查之變化、心臟功能及心肺功能的變化。經由本研究可以得到Fontan 病人長期追蹤的資料。包括存活率併發症比率、種類以及心臟功能的狀況以及不同開刀方式病人各項parameter 的差異。並經由心導管治療方式治療Fontan 殘留的缺口及側支靜脈或肺動脈狹窄，是否可以增加心肺功能以及改善症狀。<br> Abstract: After development of Fontan-type operation,long term survivalbecomes possible in patients with single ventricle. However, therewere high incidence of complications during follow-up andcardiovascular function was usually impaired in those patients withcomplications. The purpose of this study is to investigate1. long-term survival and complicationｓ rate of Fontan-type surgery,and the types & incidenceof complications2. To study the cardiovascular function of Fontan patients includingventricular ejection fraction and atrioventricular valveregurgitation.3. incidence of severe arrhythmia4. Cardiopulmoanry function in patients after Fontan surgery &compare the cardiopulmonary function between patients with andwithout cardiovascular complications.5. liver and renal function after Fontan surgery6. To evaluate the relationship of morphology of dominant ventricleand types of operation to the development of above mentionedcardiovascular complications and cardiovascular function.7. To evaluate the clinical outcomes and cardiopulmonary exercisefunction changesfollowing catheter closure of systemic-to-pulmonary venouscollaterals or fenestration after Fontan surgery.8. To evaluate the clinical outcomes of transcatheter treatment forpulmonary artery stenosis or Fontan pathway obstructionThis study will enroll around 150 patietns who survivedFontan-type operation between 1990 and 2007 in this institution.In the first year, demographic data will be obtained in eachpatient. By chart review or searching from operation records,patients hemodynamic data before surgery will be obtained. Wewill record the complications encountered in each patient duringfollow-up. Patients will be devided into 2 groups. Group I willconsist of paitents without cardiac complications and group IIwill be comprised of patients with complications. If the patientlost to follow-up, we will contact the patient by phone orquestionares. The relevance of complications to age of operation,gender, period of follow-up, morphology of dominant ventricleand type of Fontan operation will be analyzed. There were 4 typesof Fontan type operations.a.atriopulmonary connections b.rightventricle inclusion operation (Bjork modification) c.totalcavopulmonary connections (TCPC) d.laterl tunnel orextracardiac tunnel. A complete echocardiography will beperformed in each patient to evaluate cardiac function andpresence of atrioventricular regurgitation. All patients willundergo computerized tomography or magnetic resonance imagingto evaluate pulmonary artery size and McGoon ratio. Ventricularend diastolic and end systolic volume will be measured. Presenceof pulmonary artery stenosis or systemic venous collaterals willbe recorded. Liver function as well as brain natriuretic peptide(BNP) level will be determined in each patient. We will comparethe above parameters between group I patients (withoutcomplications) and group II (with complications) patients. Themorphology of dominant ventricle & McGoon ratio will be comparedbetween the 2 groups to elucidate the determinant factors leadingto development of complications. The mean BNP value will becompared between patients receiving different types of surgery.In the second and third year study, we will evaluate theeffectiveness of transcatheter treatment in closingfenestration and venous collaterals and dilation of pulmonaryartery or Fontan pathway obstruction.The results in closure ofFontan fenestration and venous collaterals will be evaluated.If the O2 saturation ‹90% or HCT > 50%,the fenestration will beclosed with an Amplatzer device. The venous collaterals will beclosed using either Gianturco coils or Amplatzer vascular plugs. Thehemodynamic and O2 saturation changes will be compared before andimmediately after closure. Cardiopulmonary exercise function willbe compared before and 6-month after closure. Patients with proteinlosing enteropathy or cardiovascular complications will be evaluatedfor presence of obstruction in Fontan pathway or pulmonary artery.If there is obstruction in Fontan pathway or pulmonary artery,balloon dilation or stent implantation will be performed. Thebiochemistry data and hemodynamics will be compared between thosebefore and 6 months following the procedure.Through these study, we will have the long-term follow-up data ofFontan operation including mortality rate and complication rate. Therisk factors for developing complications can be determined. Theclinical outcomes of transcatheter treatment in the closure offenestration and venous collaterals and dilation of Fontan pathwayobstruction can be evaluated.