Muser Daniele, Nucifora Gaetano, Muser Daniele, Nucifora Gaetano, Pieroni Maurizio, Castro Simon A, Casado Arroyo Ruben, Maeda Shingo, Benhayon Daniel A, Liuba Ioan, Sadek Mouhannad, Magnani Silvia, Enriquez Andres, Liang Jackson J, Sassone Biagio, Desjardins Benoit, Dixit Sanjay, Deo Rajat, Garcia Fermin C, Callans David J, Frankel David S, Selvanayagam Joseph B, Marchlinski Francis E, Santangeli Pasquale
Cardiac Electrophysiology, Cardiovascular Division (D.M., S.A.C., I.L., A.E., J.J.L., S.D., R.D., F.C.G., D.J.C., D.S.F., F.E.M., P.S.), Hospital of the University of Pennsylvania, Philadelphia.
Cardiothoracic Department, Udine Civil Hospital, Italy (D.M.).
Circulation. 2021 Apr 6;143(14):1359-1373. doi: 10.1161/CIRCULATIONAHA.120.047640. Epub 2021 Jan 6.
Left ventricular (LV) scar on late gadolinium enhancement (LGE) cardiac magnetic resonance has been correlated with life-threatening arrhythmic events in patients with apparently idiopathic ventricular arrhythmias (VAs). We investigated the prognostic significance of a specific LV-LGE phenotype characterized by a ringlike pattern of fibrosis.
A total of 686 patients with apparently idiopathic nonsustained VA underwent contrast-enhanced cardiac magnetic resonance. A ringlike pattern of LV scar was defined as LV subepicardial/midmyocardial LGE involving at least 3 contiguous segments in the same short-axis slice. The end point of the study was time to the composite outcome of all-cause death, resuscitated cardiac arrest because of ventricular fibrillation or hemodynamically unstable ventricular tachycardia and appropriate implantable cardioverter defibrillator therapy.
A total of 28 patients (4%) had a ringlike pattern of scar (group A), 78 (11%) had a non-ringlike pattern (group B), and 580 (85%) had normal cardiac magnetic resonance with no LGE (group C). Group A patients were younger compared with groups B and C (median age, 40 vs 52 vs 45 years; <0.01), more frequently men (96% vs 82% vs 55%; <0.01), with a higher prevalence of family history of sudden cardiac death or cardiomyopathy (39% vs 14% vs 6%; <0.01) and more frequent history of unexplained syncope (18% vs 9% vs 3%; <0.01). All patients in group A showed VA with a right bundle-branch block morphology versus 69% in group B and 21% in group C (<0.01). Multifocal VAs were observed in 46% of group A patients compared with 26% of group B and 4% of group C (<0.01). After a median follow-up of 61 months (range, 34-84 months), the composite outcome occurred in 14 patients (50.0%) in group A versus 15 (19.0%) in group B and 2 (0.3%) in group C (<0.01). After multivariable adjustment, the presence of LGE with ringlike pattern remained independently associated with increased risk of the composite end point (hazard ratio, 68.98 [95% CI, 14.67-324.39], <0.01).
In patients with apparently idiopathic nonsustained VA, nonischemic LV scar with a ringlike pattern is associated with malignant arrhythmic events.
延迟钆增强（LGE）心脏磁共振成像显示的左心室（LV）瘢痕与明显特发性室性心律失常（VA）患者的危及生命的心律失常事件相关。我们研究了以环状纤维化模式为特征的特定左心室LGE表型的预后意义。
共有686例明显特发性非持续性VA患者接受了对比增强心脏磁共振成像检查。左心室瘢痕的环状模式定义为左心室心外膜下/心肌中层LGE累及同一短轴切片中至少3个连续节段。研究终点为全因死亡、因室颤或血流动力学不稳定的室性心动过速导致的心脏骤停复苏以及适当的植入式心律转复除颤器治疗的复合结局发生时间。
共有28例患者（4%）有瘢痕的环状模式（A组），78例（11%）有非环状模式（B组），580例（85%）心脏磁共振成像正常且无LGE（C组）。A组患者比B组和C组更年轻（中位年龄，40岁对52岁对45岁；<0.01），男性更常见（96%对82%对55%；<0.01），有心脏性猝死或心肌病家族史的患病率更高（39%对14%对6%；<0.01），不明原因晕厥史更常见（18%对9%对3%；<0.01）。A组所有患者的室性心律失常呈右束支传导阻滞形态，而B组为69%，C组为21%（<0.01）。A组46%的患者观察到多灶性室性心律失常，而B组为26%，C组为4%（<0.01）。中位随访61个月（范围34 - 84个月）后，A组14例患者（50.0%）出现复合结局，B组15例（19.0%），C组2例（0.3%）（<0.01）。多变量调整后，存在环状模式的LGE仍与复合终点风险增加独立相关（风险比，68.98[95%CI，14.67 - 324.39]，<0.01）。
在明显特发性非持续性VA患者中，具有环状模式的非缺血性左心室瘢痕与恶性心律失常事件相关。
