Gastl Mareike, Behm Patrick, Jacoby Christoph, Kelm Malte, Bönner Florian
Department of Cardiology, Pulmonology and Vascular Medicine, Heinrich-Heine University Düsseldorf, Düsseldorf, Germany.
BMC Cardiovasc Disord. 2017 Mar 11;17(1):74. doi: 10.1186/s12872-017-0492-7.
Endocarditis parietalis fibroplastica Löfflein (EPF) is a rare form of primary restrictive cardiomyopathy with poor prognosis. It is generally caused by hypereosinophilic syndrome with eosinophilic penetration of the heart. This leads to congestive heart failure in three different stages. As a frequent manifestation of neoplastic diseases, cardiac involvement means poor prognosis.
The present report deals with a case of EPF caused by non-specified T-cell lymphoma (T-NOS). Besides an elevated Troponin-T enzyme, the electrocardiogram and the transthoracic echocardiography did not show any characteristic results. Due to risk/benefit assessment and low thrombocyte amounts, endomyocardial biopsy and catheterization were discarded. Using cardiovascular magnetic resonance (CMR) with steady-state free precession sequences, T2-mappping, strain analysis and late gadolinium enhancement, we were able to clearly highlight cardiac involvement at different stages. These findings characterized T-NOS as a palliative situation.
Multiparametric CMR can not only identify EPF but also characterize the patchy disease state. This provides an individual prognosis assessment. Aside from prognosis estimation, it can also be used for therapy monitoring.
Löfflein 纤维增生性心内膜炎（EPF）是一种罕见的原发性限制性心肌病，预后较差。它通常由嗜酸性粒细胞增多综合征导致嗜酸性粒细胞浸润心脏引起。这会在三个不同阶段导致充血性心力衰竭。作为肿瘤性疾病的常见表现，心脏受累意味着预后不良。
本报告涉及一例由未特定的 T 细胞淋巴瘤（T-NOS）引起的 EPF 病例。除了肌钙蛋白-T 酶升高外，心电图和经胸超声心动图未显示任何特征性结果。由于风险/获益评估以及血小板数量低，心内膜活检和导管检查被放弃。使用具有稳态自由进动序列、T2 映射、应变分析和延迟钆增强的心血管磁共振（CMR），我们能够清楚地突出不同阶段的心脏受累情况。这些发现将 T-NOS 特征化为一种姑息性情况。
多参数 CMR 不仅可以识别 EPF，还可以表征斑片状疾病状态。这提供了个体预后评估。除了预后估计外，它还可用于治疗监测。
