2012-08-012024-05-14https://scholars.lib.ntu.edu.tw/handle/123456789/660399摘要：心房中膈缺損因早期症狀不明顯，其治療常被延誤，這些病人常併隨中重度至嚴重度肺動脈高壓，甚至已形成肺動脈阻塞(pulmonary vascular occlusive disease)之不可逆反應。部份肺動脈高壓病人，其Qp/Qs 介於1.5~1.3 之間，肺動脈阻力介於6UM2~12UM2 者，常讓臨床治療陷於進退維谷的窘境，關閉後其肺動脈高壓，仍可能持續上升且右心衰竭。截至目前，仍無一決定性指標可以預測其預後，乃導致許多大型回顧型研究認為大於40 歲肺動脈高壓中度以上的病人，關閉ASD 的治療並沒優於保守的藥物治療。本計劃假設右心室功能及其心肌之變化可能是預後的決定性因子，研究對象為35歲以上的成人，罹患第二型或靜脈竇型心房中膈缺損沒有合併其他先天性及後天性心臟病，其治療前檢查包括血液血球檢查(CBC)、心電圖、心臟超音波(包括TR、PR 之逆流速及右心室壁收縮狀況)，運動心肺功能檢查、心導管血行力學檢查(包括RVP、PAP、PAWP、PARI、Qp/Qs、Rp/Rs、RVEF、LVEF)及核磁共振檢查(CMRI with LGEimaging) (包括RV & LV 質量、大小、RV&LVEF、TR 及PR 之fraction 及心室壁纖維化之評估)，並測量血清中BNP 及Collagen type1 metabilites (PICP, CITP, TIMP-1)的量，以右心室衰竭及測量心肌結締組織纖維化變性之代謝產物。選擇RVSP≧40mmHg，Qp/Qs 介於1.5~1.3 之間，Rp/Rs≦0.75 或肺動脈阻力指數6UM2~12UM2 的病人為實驗評估組，其餘為控制組，約有20~25 位病人，其治療後(外科或閉鎖器關閉) 一個月、三個月、半年、一年、兩年，再以前述檢查追蹤，而導管檢查則限有持續惡化之肺動脈高壓的病人才再安排檢查，核磁共振則於術後半年、一年、兩年後再追蹤。<br> Abstract: Atrial Septal Defect (ASD) is one of the most common congenital defect but the delayedtreatments quiet often occured in this population, which lead the treatment become adilemma to the surgeons and intervention pediatric cardiologists, especially for the patientswith moderate to severe pulmonary hypertension with lower Qp/Qs only 1.5~1.3 and higherPARI 6UM2~12UM2. There is no determinative data to predict the mid-and long-termoutcome. In this the project, we choose RV function deterioration and myocardial change inpulmonary hypertension as the predisposing factors.The patients age ≧ 35 years old, with secumdum type or/and sinus venosus type ASDswithout associated congenital and acquired anomalies are enrolled : The pretreatmentexamination data collection including CBC, electrocardiography, echocardiography (routinedata and TR, PR velocities, RV&LVEF, RV wall motion ), Exercise cardiopulmonary functiontest (O2 consumption, O2 and CO2 slope, Heart rate and rhythm) Cardiac Catheterizationhemodynamics study(RVP, PAP, PAWP, PARI, RVEF, LVEF, Qp/Qs, Rp/Rs) and CMRI(Cardiovascular magnetic resonance imaging) with LGE imaging(Late GadoliniumEnhancement Imaging) (RV and LV mass, systolic and diastolic volumes, and ejectionfractions, TR and PR fractions , the RV&LV myocardial degenerated or fibrotic change). Wechoose two biomarkers, BNP and Collagen type1 metabilites (PICP, CITP, TIMP-1), forevaluation and prediction of RV myocardial function, the former one for RV failure and theother for myocardial degenerated fibrosis.The patients with hemodynamics study RVSP≧40mmHg Qp/Qs between 1.5 and 1.3,Rp/Rs less than 0.75, PARI between 6 UM2 and 12 UM2 wood units were enrolled to theexperimental group and others become the control group. Total patient numbers will be 20 to25.The Post-treatment (surgical closure or ASD occluder ) follow up repeats the sameexaminations as the schedule of 1 month, 3 months, 6 months and 12 months and 24 months.Except that the patients develop progressive pulmonary hypertension by echocardiographyand deteriorated clinical manifestations, then, cardiac Catheterization will be advised againand the CMRI with LGE Imaging will be followed in 6 months, 12 months and 24 months.