【正文】 and disrupt established deposits using antibodies, synthetic peptides, and smallmolecule drugs J Am Coll Cardiol 2022 Beijing Anzhen Hospital Affiliated to Capital Medical University Endomyocardial Biopsy Technique Beijing Anzhen Hospital Affiliated to Capital Medical University Bioptome Beijing Anzhen Hospital Affiliated to Capital Medical University When do we biopsy? Beijing Anzhen Hospital Affiliated to Capital Medical University Does Biospy Help? Beijing Anzhen Hospital Affiliated to Capital Medical University 小結(jié) ? 男性， 49歲 ? 1年前開始間斷胸悶、氣短，活動(dòng)耐量進(jìn)行性下降，伴雙下肢浮腫和尿量減少，近 2月加重，伴腹脹 ? 無高血壓、糖尿病史；吸煙 30年 ? 外院心電圖示 II、 III、 aVF和 V1V3導(dǎo)聯(lián)出現(xiàn)病理性 Q波伴 ST段抬高；超聲心動(dòng)圖示雙房擴(kuò)大，左室輕大，室間隔（ 14mm）及左室游離壁（ 13mm）增厚，LVEF 45%；核素掃描示左室游離壁節(jié)段性灌注減低 ? 體格檢查：頸靜脈充盈，雙肺無羅音，心率 62次 /分，律齊， P2亢進(jìn)，有 S3，雙下肢無浮腫 Beijing Anzhen Hospital Affiliated to Capital Medical University 心電圖 Beijing Anzhen Hospital Affiliated to Capital Medical University 24小時(shí)血壓監(jiān)測 ? 24小時(shí)血壓波動(dòng)于 82128/4068mmHg ? 偶有體位性低血壓 Beijing Anzhen Hospital Affiliated to Capital Medical University 能否排除淀粉樣變？ If 皮膚、齒齦活檢陰性 Beijing Anzhen Hospital Affiliated to Capital Medical University 治療調(diào)整 ? 修正診斷后，我們調(diào)整了治療方案： 1. 停用地高辛 ？ 2. 停用 ACEI ？ 3. 停用 223。336:267276 The Differential Diagnosis of Restrictive Cardiomyopathy and Constrictive Pericarditis Beijing Anzhen Hospital Affiliated to Capital Medical University Cardiac Amyloidosis ? Causes Restrictive Cardiomyopathy ? Myocyte Destruction and Replacement with Amyloid Fibrils Leading to Progressive Thickening of the Ventricular Walls. ? More Common in Primary (AL) Amyloidosis, a Plasma Cell Dyscrasia Where Immunoglobulins Form Amyloid Protein Beijing Anzhen Hospital Affiliated to Capital Medical University 定義： 細(xì)胞間質(zhì)，特別是小血管基底膜出現(xiàn)淀粉樣蛋白質(zhì)－粘多糖復(fù)合物蓄積，稱為淀粉樣變 （ Amyloidosis） 鏡下： 淡紅色均質(zhì)狀物 （ HE染色 ） 顯示淀粉樣呈色反應(yīng)： 剛果紅染色為橘紅色 遇碘為棕色，再加稀硫酸呈藍(lán)色 Cardiac Amyloidosis Beijing Anzhen Hospital Affiliated to Capital Medical University Beijing Anzhen Hospital Affiliated to Capital Medical University Beijing Anzhen Hospital Affiliated to Capital Medical University Cardiac Amyloidosis ? EKG: Often Have Bundle Branch Block, Low Voltage Despite Thick Ventricles, or AFib ? Most Patients with Amyloidosis, Even Those with no Cardiac Symptoms, Have Abnormal Echocardiograms with: Ventricular Wall Thickening (70%), Isolated Septal Wall Thickening (30%), Diastolic Dysfunction (57%), Systolic Dysfunction (27%), Pericardial Effusion (40%), Myocardial Sparkling Pattern in 2D (27%) Beijing Anzhen Hospital Affiliated to Capital Medical University 圖A為體表ECHO，可見三尖瓣（TV）增厚， 右心房心內(nèi)膜可見圓形增厚的區(qū)域（箭頭）， 圖B為經(jīng)食道ECHO，可見房間隔 （IAS）明顯增厚 Beijing Anzhen Hospital Affiliated to Capital Medical University 左室肥厚和斑點(diǎn)樣改變 雙側(cè)心室彌漫性釓攝取延遲 Beijing Anzhen Hospital Affiliated to Capital Medical University Cardiac Amyloidosis ? Diagnosis with Tissue Biopsy Revealing Amyloidosis, Often Abdominal Fat Pad ? Prognosis Less Than 1 Year, Survival at 5 Years 5% ? Treatment: Primary Amyliodosis Limited Benefit of Alkylating Agents ? Secondary Amyloidosis: Treat Underlying Disease Beijing Anzhen Hospital Affiliated to Capital Medical University 低倍，心內(nèi)膜結(jié)節(jié)樣沉積的淀粉樣物質(zhì)（ HE染色） 低倍，剛果紅染色證實(shí)心內(nèi)膜沉積物為淀粉樣物質(zhì) 中倍， γ免疫球蛋白抗體染色呈強(qiáng)陽性反應(yīng) 中部，前白蛋白（運(yùn)甲狀腺素蛋白）免疫組化染色陰性 Beijing Anzhen Hospital Affiliated to Capital Medical University 剛果紅染色示蘋果綠折射（陽性） J Am Coll Cardiol 2022 Beijing Anzhen Hospital Affiliated to Capital Medical University Summary of clinical investigations leading to a diagnosis of cardiac amyloidosis Nat Clin