2014-08-012024-05-14https://scholars.lib.ntu.edu.tw/handle/123456789/657444摘要：本研究的目的在於探討一個針對成年人先天性心臟病合併嚴重肺高血壓新的治療策略，先天性心臟病患者不及時診治，往往會造成肺高血壓，肺高血壓會產生肺動脈內壁增生、肥厚甚至阻塞，病理學判斷肺血管阻塞病變第四級以上即為開刀治療為禁忌症，過去是否可以開刀常以病理學為依據，肺部切片也存在風險，若以血行動力學測定的肺動脈阻抗來決定是否可以開刀，仍存有一些灰色地帶，心房中膈缺損皆可以心導管關閉，將以心導管技術來克服肺高血壓的問題，術後再以治療肺高血壓的藥物如長期投予病人，這就是修補後再積極治療的策略。本研究將納入20名18歲以上有嚴重肺高血壓的心房中膈缺損病患，進行心導管修補，修補時在關閉器上挖7-8mm的洞,再投予 viagra。並測量術前術後肺血壓的變化，以及心肺功能改善的程度，須排除超高的肺動脈阻抗嚴重心衰竭等,術後須在一、三月檢測肺動脈收縮壓，並在六個月後測量心肺運動功能，請測定血中proBNP的濃度,內皮細胞，比較術前術後的症狀改善情況。本研究預計可治療20位患有心房中隔缺損合併肺高血壓症的病人，以本方法可以治療肺動脈壓超過外科關閉手術所允許的上限的病人，配合降肺高壓藥物的使用,這些病人預期心臟功能及肺高壓均會在術後獲得改善。<br> Abstract: The study aims to evaluate the effectiveness of a strategy of “repair-and –treat” in the management of adult congenital heart disease patients (ACHD) with advanced pulmonary hypertension. Advanced pulmonary hypertension may occur if congenital heart disease with significant left-to right shunt is not repaired in time. Intimal hyperplasia and medial layer hypertrophy will occur in pulmonary arteries as a result of long term increased blood flow. Endothelial dysfunction and vascular remodeling occur in pulmonary vascular bed. With time, obliteration or thrombosis of distal pulmonary arteries occurs. Pulmonary vascular obstructive disease (PVOD) has been used to describe this condition. Heath and Edward has proposed a classification with 6 grades to classify the degree of PVOD. Pathology of the lung tissue was used to evaluate the “operability” in those patients. If the degree of PVOD is greater than grade IV, it is considered as irreversible changes where operation is contraindicated. Repair the defect is patients with grade II PVOD or below is considered as feasible. But the grade III PVOD is frequently considered as “gray zone”. Since the risk of lung biopsy in those patients is not low, operability is evaluated based on pulmonary vascular resistance index (PVI) measured during catheterization. There is no consensus on the upper limits of pulmonary vascular resistance for “operability”. In the past, a PVI greater than 8 wood unit is considered as “inoperable”. With the advance of transctheter techniques, 90% of atrial septal defect can be closed non-surgically. Many drugs have been successful used to treat pulmonary hypertension. We aim to expand the upper limit of “operability” by adopting a strategy by combining catheter technique and medical treatment.(repair and treat) We will modify the transcatheter technique, by creating a hole 7-8 mm in diameter on the disk of the device. Thus, the shunt can be gradually reduced. The fenestration will function as a check-valve. After the procedure, all patients will receive sildenafil. We will enroll 20 adults (age >18 years) with unrepaired secundum type atrial septal defect. All patients will receive catheter closure using a fenestrated device (7-8 mm fenestration). Then sildenafil 20mg tid will be given. We will evaluate the changes of the pulmonary artery pressure and cardiopulmonary function after catheter closure. We will exclude patients with1.extremely high pulmonary artery resistance 2.severe ventricular dysfunction or functional class IV. 3. O2 saturation <85% 4.Renal failure requiring analysis, or liver cirrhosis. 5 Malignant disease under chemotherapy. We will evaluate the pulmonary artery pressure and cardiac function on echocardiogram 1 month, 3 months & then every 3 months after the procedure cardiopulmonary function & symptoms will be evaluated at 6 months. We will also measure the changes of the biomarkers of pulmonary hypertension that are circulating endothelial cell (CEC) and endothelial progenitor cell. (EPC) The changes in CEC & EPC number will be used to correlate the changes inpulmonary artery pressure. Using this “repair and treat” strategy,. patients will survive with a better long-term survival and improved quality of life.