Sykora Daniel, Bratcher Melanie, Churchill Robert, Kim B Michelle, Elwazir Mohamed, Young Kathleen, Ryan Sami, Kolluri Nikhil, Abou Ezzeddine Omar, Bois John, Giudicessi John, Cooper Leslie, Rosenbaum Andrew
Mayo Clinic School of Graduate Medical Education.
Department of Cardiovascular Diseases, Mayo Clinic Rochester.
Circ J. 2024 Dec 25;89(1):41-52. doi: 10.1253/circj.CJ-24-0205. Epub 2024 Sep 3.
Cardiac sarcoidosis (CS) may result in systolic heart failure (heart failure with reduced ejection fraction [HFrEF]), but its response to guideline-directed medical therapy (GDMT) remains uncertain.
We investigated 881 patients evaluated for CS to identify those with diagnosed CS, left ventricular ejection fraction (LVEF) ≤40% at diagnosis, and follow-up echocardiogram within 11-24 months. Demographics, LVEF, GDMT as quantified by Kansas City Medical Optimization (KCMO) score, and immunosuppressive treatment were recorded. The primary outcome was a composite of event-free survival (unplanned heart failure hospitalization, left ventricular assist device [LVAD]/heart transplant, or death). Seventy-nine (9%) CS patients met the inclusion criteria (35% female, median age 57 years, mean LVEF 30.9%, median New York Heart Association class II [46%], mean number of GDMT agents 1.7, and mean KCMO score 31.8). Most (87%) were treated with immunosuppressive treatment. At follow-up (median 16 months), the mean number of GDMT agents increased to 2.2 (P=0.02), and the mean KCMO score to 70.1 (P<0.001). Mean LVEF improved to 39.9% (excluding LVAD/transplant; P<0.001) and the change in LVEF was correlated with follow-up KCMO score (P<0.001). The primary outcome occurred in 13 (16%) patients and differed by KCMO score (log-rank P<0.001), but not by immunosuppressive treatment (log-rank P=0.36).
GDMT optimization is associated with better cardiac remodeling and clinical outcomes in CS patients with HFrEF.
心脏结节病（CS）可能导致收缩性心力衰竭（射血分数降低的心力衰竭[HFrEF]），但其对指南指导的药物治疗（GDMT）的反应仍不确定。
我们调查了881例接受CS评估的患者，以确定那些确诊为CS、诊断时左心室射血分数（LVEF）≤40%且在11 - 24个月内进行了随访超声心动图检查的患者。记录人口统计学资料、LVEF、通过堪萨斯城医学优化（KCMO）评分量化的GDMT以及免疫抑制治疗情况。主要结局是无事件生存期（计划外心力衰竭住院、左心室辅助装置[LVAD]/心脏移植或死亡）的复合指标。79例（9%）CS患者符合纳入标准（女性占35%，中位年龄57岁，平均LVEF 30.9%，纽约心脏协会中位分级为II级[46%]，平均GDMT药物数量为1.7，平均KCMO评分为31.8）。大多数（87%）接受了免疫抑制治疗。在随访时（中位时间16个月），平均GDMT药物数量增加至2.2（P = 0.02），平均KCMO评分增加至70.1（P < 0.001）。平均LVEF改善至39.9%（不包括LVAD/移植；P < 0.001），且LVEF的变化与随访KCMO评分相关（P < 0.001）。13例（16%）患者出现了主要结局，且主要结局因KCMO评分而异（对数秩检验P < 0.001），但不因免疫抑制治疗而异（对数秩检验P = 0.36）。
在HFrEF的CS患者中，GDMT优化与更好的心脏重塑和临床结局相关。
