Siebermair Johannes, Lehner Sebastian, Sattler Stefan M, Rizas Konstantinos D, Beckmann Britt-Maria, Becker Alexander, Schiller Julia, Metz Corona, Zacherl Mathias, Vonderlin Nadine, Rassaf Tienush, Dobrev Dobromir, Rischpler Christoph, Kääb Stefan, Hacker Marcus, Todica Andrei, Wakili Reza
Department of Cardiology and Vascular Medicine, West-German Heart and Vascular Center, University Duisburg-Essen, Essen, Germany; Department of Medicine I, University Hospital Munich, Ludwig-Maximilians University, Munich, Germany; German Cardiovascular Research Center (DZHK), Munich Heart Alliance, Munich, Germany.
Department of Nuclear Medicine, University Hospital, LMU Munich, Munich, Germany; Ambulatory Healthcare Center Dr. Neumaier & Colleagues, Radiology, Nuclear Medicine, Radiation Therapy, Regensburg, Germany.
Int J Cardiol. 2020 Aug 1;312:129-135. doi: 10.1016/j.ijcard.2020.03.013. Epub 2020 Mar 6.
Impaired myocardial sympathetic innervation assessed by Iodine-Metaiodobenzylguanidine (I-MIBG) scintigraphy is associated with cardiac events. Since regional disparities of structural abnormalities are common in inherited arrhythmia syndromes (iAS), a chamber-specific innervation assessment of the right (RV) and left ventricle (LV) could provide important insights for a patient-individual therapy. Aim of this study was to evaluate chamber-specific patterns of autonomic innervation by Single-photon emission computed tomography/computed tomography (SPECT/CT) in patients with iAS with respect to clinical outcome regarding cardiac events.
We assessed ventricular sympathetic innervation (LV, RV and planar heart/mediastinum-ratios, and washout-rates) by I-MIBG-SPECT/CT in 48 patients (arrhythmogenic right ventricular cardiomyopathy [ARVC], n = 26; laminopathy, n = 8; idiopathic ventricular fibrillation [iVF], n = 14) in relation to a composite clinical endpoint (ventricular arrhythmia; cardiac death; cardiac hospitalization). RV tracer uptake was lower in patients with ARVC than in laminopathy and iVF patients (1.7 ± 0.4 vs. 2.1 ± 0.7 and 2.1 ± 0.5, respectively). Over a median follow-up of 2.2 years, the combined endpoint was met in 18 patients (n = 12 ventricular tachyarrhythmias, n = 5 hospitalizations, n = 1 death). LV, but not RV H/M ratio was associated with the combined endpoint (hazard-ratio 2.82 [1.30-6.10], p < 0.01). After adjustment for LV and RV function, LV H/M-ratio still remained a significant predictor for cardiac events (hazard-ratio 2.79 [1.06-7.35], p = 0.04).
We demonstrated that chamber-specific MIBG-SPECT/CT imaging is feasible and that reduced LV sympathetic innervation was associated with worse outcome in iAS. These findings provide novel insights into the potential role of regional autonomic nervous system heterogeneity for the evolution of life-threatening cardiac events in iAS.
通过碘-间碘苄胍（I-MIBG）闪烁扫描评估的心肌交感神经支配受损与心脏事件相关。由于遗传性心律失常综合征（iAS）中结构异常的区域差异很常见，对右心室（RV）和左心室（LV）进行特定腔室的神经支配评估可为个体化治疗提供重要见解。本研究的目的是通过单光子发射计算机断层扫描/计算机断层扫描（SPECT/CT）评估iAS患者自主神经支配的特定腔室模式，并观察其与心脏事件临床结局的关系。
我们通过I-MIBG-SPECT/CT评估了48例患者（致心律失常性右心室心肌病[ARVC]，n = 26；层黏连蛋白病，n = 8；特发性室颤[iVF]，n = 14）的心室交感神经支配情况（左心室、右心室以及平面心脏/纵隔比值和清除率），并将其与复合临床终点（室性心律失常；心源性死亡；心脏住院）相关联。ARVC患者的右心室示踪剂摄取低于层黏连蛋白病和iVF患者（分别为1.7±0.4 vs. 2.1±0.7和2.1±0.5）。在中位随访2.2年期间，18例患者达到了联合终点（n = 1十二室性快速心律失常，n = 5次住院，n = 1例死亡）。左心室而非右心室的H/M比值与联合终点相关（风险比2.82 [1.30 - 6.10]，p < 0.01）。在调整左心室和右心室功能后，左心室H/M比值仍然是心脏事件的显著预测指标（风险比2.79 [1.06 - 7.35]，p = 0.04）。
我们证明了特定腔室的MIBG-SPECT/CT成像可行，且左心室交感神经支配减少与iAS患者的不良结局相关。这些发现为区域自主神经系统异质性在iAS中危及生命的心脏事件演变中的潜在作用提供了新的见解。
