Faraj Raid, Bourouhou Zaineb, Oussama Sidaty, Bouamoud Asmaa, Rami Hasna, Samih Amina, Fellat Ibtissam, Zarzur Jamila, Cherti Mohamed
Mohammed V University, Rabat, Morocco.
Ann Med Surg (Lond). 2022 Mar 3;75:103410. doi: 10.1016/j.amsu.2022.103410. eCollection 2022 Mar.
Cardiac amyloidosis (CA) is a rare condition, characterized by fibrillary proteins infiltration in the extracellular space of the heart. Even though many types of cardiac amyloidosis exist, light chain amyloidosis (AL) and transthyretin amyloidosis (ATTR) remain the most described forms. The diagnosis of amyloidosis represents a real challenge for clinicians, requiring both invasive and non-invasive investigations. Conduction defects and atrial arrhythmias are well known complications of cardiac amyloidosis. However, only a few studies have reported junctional rhythm a primary presentation of light chain cardiac amyloidosis (AL). An early diagnosis and proper management are crucial to improve the prognosis of this disease.
Here, we report a rare case of a 48 year-old patient, in acutely decompensated heart failure, presenting an accelerated junctional rhythm (AJR) as initial presentation of light-chain cardiac amyloidosis. The diagnosis was made based on clinical, biological, radiological and histological findings. This case shows diagnostic difficulties and management of this rare disease.
心脏淀粉样变性（CA）是一种罕见疾病，其特征是纤维蛋白浸润于心脏的细胞外间隙。尽管存在多种类型的心脏淀粉样变性，但轻链淀粉样变性（AL）和转甲状腺素蛋白淀粉样变性（ATTR）仍是描述最多的类型。淀粉样变性的诊断对临床医生而言是一项真正的挑战，需要进行有创和无创检查。传导缺陷和房性心律失常是心脏淀粉样变性的常见并发症。然而，仅有少数研究报道交界性心律是轻链心脏淀粉样变性（AL）的主要表现形式。早期诊断和恰当治疗对于改善该疾病的预后至关重要。
在此，我们报告一例罕见病例，一名48岁急性失代偿性心力衰竭患者，以加速性交界性心律（AJR）作为轻链心脏淀粉样变性的首发表现。诊断基于临床、生物学、放射学和组织学检查结果。该病例展示了这种罕见疾病的诊断困难及治疗情况。
