Institute for Cardiovascular Science, University College London, London, UK; Barts Heart Centre, St Bartholomew's Hospital, London, UK.
Institute for Cardiovascular Science, University College London, London, UK; Barts Heart Centre, St Bartholomew's Hospital, London, UK; John Radcliffe Hospital, Oxford University Hospitals, Oxford, UK.
Int J Cardiol. 2019 Jun 1;284:99-104. doi: 10.1016/j.ijcard.2018.10.083. Epub 2018 Oct 26.
Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a heritable heart muscle disease that causes sudden cardiac death in the young. Inflammatory myocardial infiltrates have been described at autopsy and on biopsy, but there are few data on the presence of myocarditis in living patients with ARVC using non-invasive imaging techniques. FDG-PET is a validated technique for detecting myocardial inflammation in clinically suspected myocarditis. We aimed to determine the prevalence of myocardial inflammation in patients with ARVC using F-fluorodeoxyglucose positron emission tomography (FDG-PET).
We performed a retrospective analysis of a single centre cohort of patients with ARVC referred for FDG-PET scans between 2012 and 2017 for investigation of symptoms or suspected device infection. Sixteen patients (12 male; age 42 ± 13 years) with a definite diagnosis of ARVC were identified. Seven had positive FDG-PET scans, two of whom had cardiac sarcoidosis on endomyocardial biopsy. Of the remaining five, two carried pathogenic desmoplakin mutations. FDG uptake was found in the left ventricular myocardium in all cases. One patient also had right ventricular uptake.
In this exploratory study, we show that some patients with ARVC have evidence for myocardial inflammation on FDG-PET, suggesting that myocarditis plays a role in disease pathogenesis.
致心律失常性右室心肌病(ARVC)是一种遗传性心肌疾病,可导致年轻人心脏性猝死。尸检和活检均描述了炎性心肌浸润,但使用非侵入性成像技术在活的 ARVC 患者中存在心肌炎的资料很少。FDG-PET 是一种用于检测临床疑似心肌炎的心肌炎症的验证技术。我们旨在使用 F-氟脱氧葡萄糖正电子发射断层扫描(FDG-PET)确定 ARVC 患者心肌炎症的患病率。
我们对 2012 年至 2017 年间因症状或疑似器械感染而接受 FDG-PET 扫描的 ARVC 患者的单中心队列进行了回顾性分析。确定了 16 名(12 名男性;年龄 42±13 岁)具有明确 ARVC 诊断的患者。7 名患者的 FDG-PET 扫描呈阳性,其中 2 名患者在心肌活检中发现心脏结节病。其余 5 名患者中,有 2 名携带致病性桥粒蛋白基因突变。所有病例均在左心室心肌中发现 FDG 摄取。1 名患者还存在右心室摄取。
在这项探索性研究中,我们表明一些 ARVC 患者的 FDG-PET 显示存在心肌炎症证据,这表明心肌炎在疾病发病机制中起作用。