Tan Joo Hor, Yew Min Sen, Huang Wenjie, Tan Kenny
Department of Cardiology, Tan Tock Seng Hospital, 11 Jalan Tan Tock Seng Hospital, Singapore 308433, Singapore.
Eur Heart J Case Rep. 2021 Jan 4;5(2):ytaa510. doi: 10.1093/ehjcr/ytaa510. eCollection 2021 Feb.
POEMS syndrome (PS) is a paraneoplastic disorder from plasma cell dyscrasia, characterized by polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, and skin changes. Vascular endothelial growth factors (VEGFs)-driven fluid extracellular matrix expansion plays a key role in this condition. Associated cardiac involvement has been sparsely reported thus far.
A 55-year-old woman with PS presented with a pleural effusion and respiratory failure requiring mechanical ventilation. Transthoracic echocardiogram revealed left ventricular (LV) systolic dysfunction with a moderate pericardial effusion. She developed intermittent complete heart block and ventricular standstill, requiring temporary transcutaneous pacing. Further evaluation revealed no significant coronary stenosis on coronary angiogram and cardiac magnetic resonance (CMR) showed elevated T1 and extracellular volume suggestive of myocardial oedema with possible early cardiac infiltration. She had a dual-chamber permanent pacemaker implanted in view of recurrent high-grade heart block. She was initiated on a daratumumab-based chemotherapy regimen prior to discharge. She recovered well subsequently with a promising clinical response to chemotherapy.
We describe the first case of LV systolic dysfunction with concomitant significant bradyarrhythmia in a patient with PS. CMR revealed evidence suggestive of LV myocardial oedema and/or possible early infiltration. VEGF overexpression could explain oedema-related LV dysfunction which reversed with adequate diuresis, as well as damage to the conduction system. Early cardiac amyloidosis, which can be associated with PS, is an important differential diagnosis. Pacemaker implantation, adequate diuresis, and definitive chemotherapy are key to the management of concomitant ventricular myocardial and electrical dysfunction in such rare case.
POEMS综合征（PS）是一种由浆细胞异常增生引起的副肿瘤性疾病，其特征为多发性神经病、脏器肿大、内分泌病、单克隆蛋白及皮肤改变。血管内皮生长因子（VEGF）驱动的细胞外液基质扩张在该病症中起关键作用。迄今为止，相关的心脏受累情况报道较少。
一名55岁的PS女性患者出现胸腔积液及呼吸衰竭，需要机械通气。经胸超声心动图显示左心室（LV）收缩功能障碍并伴有中度心包积液。她出现间歇性完全性心脏传导阻滞及心室停搏，需要临时经皮起搏。进一步评估显示冠状动脉造影未见明显冠状动脉狭窄，心脏磁共振成像（CMR）显示T1及细胞外容积升高，提示心肌水肿且可能存在早期心脏浸润。鉴于反复出现高度心脏传导阻滞，她植入了双腔永久性起搏器。出院前开始使用基于达雷妥尤单抗的化疗方案。随后她恢复良好，对化疗有良好的临床反应。
我们描述了首例PS患者出现左心室收缩功能障碍并伴有显著缓慢性心律失常的病例。CMR显示有证据提示左心室心肌水肿和/或可能的早期浸润。VEGF过表达可解释与水肿相关的左心室功能障碍，这种障碍可通过充分利尿得到逆转，同时也可解释传导系统受损的情况。早期心脏淀粉样变性可与PS相关，是一个重要的鉴别诊断。起搏器植入、充分利尿及确定性化疗是处理此类罕见病例中并发的心室心肌和电功能障碍的关键。
