Pöyhönen Pauli, Lehtonen Jukka, Syväranta Suvi, Velikanova Diana, Mälkönen Henriikka, Simonen Piia, Nordenswan Hanna-Kaisa, Uusitalo Valtteri, Vihinen Tapani, Kaikkonen Kari, Haataja Petri, Kerola Tuomas, Rissanen Tuomas T, Vepsäläinen Ville, Alatalo Aleksi, Pietilä-Effati Päivi, Kupari Markku
Heart and Lung Center, Helsinki University Hospital and University of Helsinki, Finland (P.P., J.L., D.V., H.M., P.S., H.-K.N., M.K.).
Radiology, Helsinki University Hospital and University of Helsinki, Finland (P.P., S.S., V.U.).
Circ Arrhythm Electrophysiol. 2025 Jan;18(1):e013239. doi: 10.1161/CIRCEP.124.013239. Epub 2024 Dec 20.
Cardiac sarcoidosis involves a significant but difficult-to-define risk of sudden cardiac death (SCD). Current guidelines recommend consideration of an implantable cardioverter defibrillator for patients with extensive or significant myocardial late gadolinium enhancement (LGE) on cardiac magnetic resonance imaging. However, extensive/significant LGE is not defined.
A nationwide cardiac sarcoidosis registry was screened for patients entered before 2020 with cardiac magnetic resonance imaging done before or <3 months after diagnosis. Available studies were re-analyzed for LGE mass as a percentage of left ventricular (LV) mass and the number of LGE-positive LV segments in a 17-segment model. The occurrence of fatal or aborted SCD and ventricular tachycardia (VT) prompting therapy was recorded until the end of 2020 and subjected to cumulative incidence analyses, including competing events (LV assist device implantations, heart transplantations, and fatalities other than SCD). The predictors of SCD/VT were assessed using Fine and Gray modeling and time-dependent receiver operating characteristic analysis.
Altogether, 305 patients (66% women, median age 51) with clinically manifest, definite (45%) or probable cardiac sarcoidosis (55%) were analyzed. On follow-up (median, 4.0 years), 21 SCDs, 60 VTs, and 14 competing events were noted. Both LGE mass and the number of LGE segments predicted the composite of SCD/VT (<0.001), with receiver operating characteristic analyses identifying LGE mass ≥9.9% and ≥6 LGE segments as discriminative thresholds. At presentation, 70 patients were free of class I and class IIa implantable cardioverter defibrillator indications unrelated to LGE. Their 5-year rate of SCD/VT was 6.3% (0.0-14.8%) with LGE mass <9.9% versus 21.5% (6.5-36.6%) with higher LGE mass, and 6.9% (0.0-16.3%) with <6 LGE segments versus 20.5% (5.9-35.2%) with ≥6 segments.
In cardiac sarcoidosis, myocardial LGE making up ≥9.9% of LV mass or affecting ≥6 LV segments may suggest prognostically significant LV involvement and a high risk of SCD. However, prospective validation of the thresholds is needed.
心脏结节病存在显著但难以界定的心脏性猝死（SCD）风险。当前指南建议，对于心脏磁共振成像显示有广泛或显著心肌晚期钆增强（LGE）的患者，考虑植入植入式心律转复除颤器。然而，广泛/显著LGE尚未有明确界定。
在全国性心脏结节病登记处筛选出2020年前入组且在诊断前或诊断后<3个月进行过心脏磁共振成像的患者。对现有研究重新分析LGE质量占左心室（LV）质量的百分比以及17节段模型中LGE阳性LV节段的数量。记录直至2020年底发生的致命或非致命性SCD以及促使进行治疗的室性心动过速（VT）情况，并进行累积发病率分析，包括竞争事件（LV辅助装置植入、心脏移植以及非SCD导致的死亡）。使用Fine和Gray模型以及时间依赖性受试者工作特征分析评估SCD/VT的预测因素。
共分析了305例有临床表现、确诊（45%）或可能（55%）心脏结节病的患者（66%为女性，中位年龄51岁）。随访（中位时间4.0年）期间，记录到21例SCD、60例VT以及14例竞争事件。LGE质量和LGE节段数量均能预测SCD/VT的复合终点（<0.001），受试者工作特征分析确定LGE质量≥9.9%和≥6个LGE节段为判别阈值。就诊时，70例患者无与LGE无关的I类和IIa类植入式心律转复除颤器适应证。LGE质量<9.9%的患者5年SCD/VT发生率为6.3%（0.0 - 14.8%），而LGE质量较高者为21.5%（6.5 - 36.6%）；LGE节段<6个的患者5年SCD/VT发生率为6.9%（0.0 - 16.3%），而≥6个节段者为20.5%（5.9 - 35.2%）。
在心脏结节病中，心肌LGE占LV质量≥9.9%或累及≥6个LV节段可能提示LV受累具有预后意义且SCD风险高。然而，这些阈值需要前瞻性验证。
