Background

Although isolated cardiac sarcoidosis (CS) is not uncommon, little is known about the prognosis. We aimed to clarify clinical features and clinical outcomes in patients with isolated CS.

Methods

Two-hundred eighty-six patients with suspected CS were enrolled. Systemic CS (SCS) was diagnosed by histological or clinical confirmation of sarcoidosis according to the guidelines. Isolated CS was diagnosed by histological or clinical confirmation in the heart alone. The endpoint was cardiac death, hospitalization for heart failure, or fatal ventricular arrhythmia.

Results

Twenty-one patients were diagnosed with isolated CS, and 63 were diagnosed with SCS. The frequencies of diagnostic criteria, such as high-grade atrioventricular block or fatal ventricular arrhythmia, basal thinning of the ventricular septum, left ventricular contractile dysfunction, positive myocardial uptake of gallium-67 citrate scintigraphy or fluorine-18 fluorodeoxyglucose positron emission tomography, and delayed contrast enhancement of cardiac magnetic resonance, were higher or equivalent in patients with isolated CS, compared to those with SCS. Over a median follow-up of 31 months (range: 1–175 months), cardiac death, hospitalization for heart failure, or fatal ventricular arrhythmia occurred in 14 (67%) patients with isolated CS, 24 (38%) patients with SCS, and 63 (31%) patients without CS. The rate of cardiac events was higher in patients with isolated CS (log-rank test, p = 0.01). Cox proportional hazard analysis showed that isolated CS, age, and New York Heart Association functional class were independently associated with cardiac events.

Conclusions

Patients with isolated CS have clinical features compatible with SCS, and have cardiac events at a higher rate.

中文翻译：

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临床诊断标准证实的孤立性心脏结节病患者的临床结局

背景

尽管孤立的心脏结节病 (CS) 并不少见，但对其预后知之甚少。我们旨在阐明孤立性 CS 患者的临床特征和临床结果。

方法

招募了 286 名疑似 CS 的患者。根据指南，系统性 CS (SCS) 是通过结节病的组织学或临床确认来诊断的。孤立的 CS 仅通过心脏的组织学或临床确认来诊断。终点是心源性死亡、因心力衰竭住院或致命的室性心律失常。

结果

21名患者被诊断为孤立性CS，63名患者被诊断为SCS。诊断标准的频率，如高度房室传导阻滞或致死性室性心律失常、室间隔基底层变薄、左心室收缩功能障碍、67 柠檬酸镓闪烁显像或氟 18 氟脱氧葡萄糖正电子发射断层扫描阳性心肌摄取、延迟与 SCS 患者相比，单纯 CS 患者的心脏磁共振对比增强效果更高或相当。在 31 个月（范围：1-175 个月）的中位随访期间，14 名（67%）孤立性 CS 患者和 24 名（38%）名 SCS 患者发生心源性死亡、因心力衰竭住院或致死性室性心律失常，以及 63 名 (31%) 没有 CS 的患者。p  = 0.01)。Cox 比例风险分析表明，孤立的 CS、年龄和纽约心脏协会功能分级与心脏事件独立相关。

结论

孤立性 CS 患者具有与 SCS 相符的临床特征，并且发生心脏事件的发生率更高。