Terashima Rika, Nakagawa Takashi, Hara Hisao, Hiroi Yukio
Department of Cardiology, National Center for Global Health and Medicine, 1-21-1 Toyama, Shinjuku-ku, Tokyo 162-8655, Japan.
Eur Heart J Case Rep. 2023 Mar 2;7(3):ytad116. doi: 10.1093/ehjcr/ytad116. eCollection 2023 Mar.
Spontaneous left atrial intramural hematoma (LAIH) is extremely rare and there are only two cases of spontaneous LAIH involving cardiac amyloidosis (CA) reported in literature. In both cases, LAIH rapidly compromised hemodynamic stability proving to be a rare yet fatal complication.
An 83-year-old man presented with cardiogenic shock. Electrocardiogram showed complete atrioventricular block, and echocardiogram revealed severe hypokinesis and left ventricular hypertrophy. Coronary angiography revealed no significant coronary stenosis and tissue biopsy was taken from the left ventricle. The patient was intubated, placed on extracorporeal membrane oxygenation with intra-aortic balloon pump and temporary pacemaker, and admitted to ICU. Day 6 of admission, he became hemodynamically unstable, and presented with atrial fibrillation. Transesophageal echocardiography showed a newly formed large mass in the left atrium. Day 11 of admission, the patient passed away. Autopsy revealed cardiac amyloidosis and showed the mass to be a left atrial intramural hematoma. Diffuse amyloid deposits were found in the myocardium as well as the blood vessel walls of the region surrounding the LAIH.
LAIH is a rare yet fatal complication of CA. Autopsy revealed diffuse amyloid deposits within the left atrium may lead to left atrial fragility and contribute to development of LAIH. LAIH should be considered as an important differential diagnosis in the setting of a rapidly growing left atrial mass, and in hemodynamic instability in patients with CA.
自发性左房壁内血肿(LAIH)极为罕见,文献中仅报道了两例涉及心脏淀粉样变性(CA)的自发性LAIH病例。在这两例病例中,LAIH迅速损害血流动力学稳定性,证明是一种罕见但致命的并发症。
一名83岁男性出现心源性休克。心电图显示完全性房室传导阻滞,超声心动图显示严重运动减弱和左心室肥厚。冠状动脉造影显示无明显冠状动脉狭窄,并从左心室进行了组织活检。患者插管,使用主动脉内球囊泵和临时起搏器进行体外膜肺氧合,并入住重症监护病房。入院第6天,他的血流动力学变得不稳定,并出现房颤。经食管超声心动图显示左心房有一个新形成的大肿块。入院第11天,患者去世。尸检发现心脏淀粉样变性,肿块为左房壁内血肿。在心肌以及LAIH周围区域的血管壁中发现了弥漫性淀粉样沉积物。
LAIH是CA的一种罕见但致命的并发症。尸检发现左心房内弥漫性淀粉样沉积物可能导致左心房脆弱,并促成LAIH的发生。在左心房肿块迅速增大以及CA患者出现血流动力学不稳定的情况下,应将LAIH视为重要的鉴别诊断。