Gupta Vrinda, Rahim Muhammed A, Brookmeyer Claire, Carrick Richard T, Kassamali Ali Asghar, Ranek Mark, Madrazo Jose, Vaishnav Joban
Department of Internal Medicine, The Johns Hopkins Hospital, Baltimore, Maryland, USA.
Department of Diagnostic Radiology, The Johns Hopkins Hospital, Baltimore, Maryland, USA.
JACC Case Rep. 2025 Jul 9;30(18):103946. doi: 10.1016/j.jaccas.2025.103946.
Cardiac amyloidosis (CA) is a highly morbid cause of heart failure with a poor untreated prognosis. The presence of left ventricular outflow tract (LVOT) obstruction may lead to misclassification of CA as hypertrophic obstructive cardiomyopathy (HOCM). Between 2022 and 2024, 7 patients-4 with light- chain cardiac amyloidosis (AL-CM) and 3 with transthyretin cardiac amyloidosis-initially presented with HOCM at our center (The Johns Hopkins Hospital, Baltimore, Maryland, USA). Diagnostic delay from HOCM to CA diagnosis ranged from 0 to 34 months. Patients with AL-CM had advanced disease (revised Mayo stage III) at the time of CA diagnosis. Six patients required reduction or withdrawal of therapy used for symptomatic LVOT obstruction, and 3 patients were referred for septal reduction therapy. Screening for CA should be considered in older adult patients presenting with HOCM. Symptomatic management of CA with LVOT obstruction is complex given patients' poor tolerance of negatively inotropic medications and the high burden of orthostatic hypotension in patients with CA.
心脏淀粉样变性（CA）是导致心力衰竭的高发病因，未经治疗时预后较差。左心室流出道（LVOT）梗阻的存在可能导致将CA误诊为肥厚性梗阻性心肌病（HOCM）。在2022年至2024年期间，我们中心（美国马里兰州巴尔的摩市约翰·霍普金斯医院）有7例患者——4例为轻链型心脏淀粉样变性（AL-CM），3例为转甲状腺素蛋白型心脏淀粉样变性——最初表现为HOCM。从HOCM诊断到CA诊断的延迟时间为0至34个月。AL-CM患者在CA诊断时已处于疾病晚期（修订的梅奥III期）。6例患者需要减少或停用用于治疗有症状LVOT梗阻的疗法，3例患者被转诊接受间隔减容治疗。对于表现为HOCM的老年患者，应考虑进行CA筛查。鉴于CA患者对负性肌力药物耐受性差以及体位性低血压负担重，对伴有LVOT梗阻的CA进行症状管理较为复杂。
