【正文】 al University Cardiac Amyloidosis ? 10 per million personyears in the . population ? No single noninvasive test or abnormality is pathognomonic of cardiac amyloid, casecontrol studies indicate that echocardiographic evidence of left ventricular wall thickening, biatrial enlargement, and increased echogenicity in conjunction with reduced electrocardiographic voltages is strongly suggestive of cardiac amyloidosis ? Newer echocardiographic techniques such as strain and strain rate imaging can demonstrate impairment in longitudinal function before ejection fraction bees abnormal J Am Coll Cardiol 2022 Beijing Anzhen Hospital Affiliated to Capital Medical University Cardiac Amyloidosis ? Recent observational studies also suggest that cardiovascular magic resonance imaging yields characteristic findings in amyloidosis, offering promise for the early detection of cardiac involvement ? Presence of detectable cardiac troponin and elevated Btype natriuretic peptide in serum of affected patients portends an adverse prognosis J Am Coll Cardiol 2022 Beijing Anzhen Hospital Affiliated to Capital Medical University Cardiac Amyloidosis ? Management strategies are largely based on nonrandomized singlecenter studies ? One of the few published randomized studies shows the superiority of oral prednisolone and melphalan pared with colchicine in systemic AL amyloidosis ? Intermediatedose infusional chemotherapy regimes (such as vincristine, adriamycin, and dexamethasone) and highdose chemotherapy with peripheral stem cell rescue have been used widely, but treatmentrelated mortality remains substantial with chemotherapy J Am Coll Cardiol 2022 Beijing Anzhen Hospital Affiliated to Capital Medical University Cardiac Amyloidosis ? Recent studies also indicate promising strategies to stabilize the native structures of amyloidogenic proteins。 inhibit fibril formation。 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。阻滯劑、硝酸酯 ？ 4. 利尿劑減量 ？ Beijing Anzhen Hospital Affiliated to Capital Medical University Key Point ? 心肌病： 1. 肥厚型 ？ 2. 限制型 ？ 3. 擴(kuò)張型 ？ 肥厚型心肌?。盒募》屎? ，繼而左房大，以雙房大為主要表現(xiàn)者少見； ， :1； 3. 右心功能受累相對(duì)晚，左室大 → 左房大 → 肺動(dòng)脈壓升高 → 右室、 右房大； ，心肌肥大非壞死，故不會(huì)出現(xiàn)“類心肌梗死” 樣表現(xiàn) 限制型心肌病：心肌侵潤或纖維化 ，肥厚不明顯，僵硬度升高，故雙房大為 主要表現(xiàn)； ； ，進(jìn)行性呼吸困難，舒張功能減退為主， 同時(shí)合并右心功能不全表現(xiàn)； 、壞死、纖維化，心電圖可出現(xiàn)“類心肌梗死”樣 表現(xiàn) 擴(kuò)張型心肌?。盒氖覕U(kuò)大，伴心肌肥厚 1. 以左室或雙室大為主，伴心肌肥厚，以雙房大為主要表現(xiàn)者少見； ，室壁厚度大致正常； ，少見舒張功能不全； 、免疫機(jī)制破壞，心肌細(xì)胞壞死、纖維化，心電圖也可出 現(xiàn)“類心肌梗死”樣表現(xiàn) Beijing Anzhen Hospital Affiliated to Capital Medical Universit