Comparison of Outcomes in Patients With Nonobstructive, Labile-Obstructive, and Chronically Obstructive Hypertrophic Cardiomyopathy.
Comparison of Outcomes in Patients With Nonobstructive, Labile-Obstructive, and Chronically Obstructive Hypertrophic Cardiomyopathy.
复制标题
DOI：
10.1016/j.amjcard.2015.06.018
复制
发表时间：
2015-09-15
期刊：
影响因子：
--
通讯作者：
Abraham TP
中科院分区：
文献类型：
--
作者：
Pozios I;Corona-Villalobos C;Sorensen LL;Bravo PE;Canepa M;Pisanello C;Pinheiro A;Dimaano VL;Luo H;Dardari Z;Zhou X;Kamel I;Zimmerman SL;Bluemke DA;Abraham MR;Abraham TP
非梗阻性肥厚型心肌病(HC)患者被认为是低风险的，通常不需要积极的干预。然而，非梗阻型和不稳定梗阻型HC传统上被归类在一起，目前尚不清楚这两个亚组是否具有不同的风险特征。我们比较了293例HC患者(96例非梗阻，114例不稳定梗阻，83例梗阻)的心血管结果，这些患者接受了运动超声心动图和磁共振成像检查，并随访了3.3±3.6年。亚组(34例非梗阻，28例不稳定梗阻，21例梗阻)行正电子发射断层扫描(PET)。不同组间心脏性猝死危险因素的平均数相似(非梗阻性：1.4对不稳定梗阻性：1.2：1.4危险因素，p=0.2)。各组间晚期Gd增强(LGE)的发生率相似，但更多的非梗阻患者的LGE≥为心肌质量的20%[23(30%)对19(18%)不稳定梗阻和8(11%)梗阻，P=0.01]。不稳定梗阻型患者出现局部PET血流灌注异常的患者较少[12例(46%)，非梗阻型30例(81%)，梗阻型17例(85%)，P=0.003]。在随访期间，记录了60个事件(36个VT/VF，包括30个除颤器放电，12个心力衰竭恶化和2个死亡)。与不稳定梗阻患者相比，非梗阻患者在随访时发生VT/VF的风险更高(HR 0.18，95%CI 0.04~0.84，p=0.03)，在调整了年龄、性别、晕厥、心脏性猝死家族史、异常血压反应和间隔≥3 cm后，这种风险仍然存在(p=0.04)。与不稳定梗阻患者[0(0%)，p=0.02]相比，非梗阻患者[8(18%)]出现适当的除颤器放电的频率更高。总之，与不稳定梗阻相比，非梗阻性血流动力学与更明显的纤维化和缺血有关，并且是HC中室性心动过速/室颤的独立预测因子。
Non-obstructive hypertrophic cardiomyopathy (HC) patients are considered low-risk, generally not requiring aggressive intervention. However, non- and labile-obstructive HC have been traditionally classified together and it is unknown if these 2 sub-groups have distinct risk profiles. We compared cardiovascular outcomes in 293 HC patients (96 non-obstructive, 114 labile-obstructive and 83 obstructive) referred for exercise echocardiography and magnetic resonance imaging and followed for 3.3±3.6 years. A sub-group (34 non-obstructive, 28 labile-obstructive, 21 obstructive) underwent positron emission tomography (PET). The mean number of sudden cardiac death risk factors was similar among groups (non-obstructive: 1.4 vs. labile-obstructive: 1.2 vs. obstructive: 1.4 risk factors, p=0.2). Prevalence of late gadolinium enhancement (LGE) was similar across groups but more non-obstructive patients had LGE≥20% of myocardial mass [23(30%) vs. 19(18%) labile-obstructive and 8(11%) obstructive, p=0.01]. Fewer labile-obstructive patients had regional PET perfusion abnormalities [12(46%) vs. non-obstructive 30(81%) and obstructive 17(85%), p=0.003]. During follow-up, 60 events were recorded (36 VT/VF, including 30 defibrillator discharges, 12 heart failure worsening and 2 deaths). Non-obstructive patients were at higher risk of VT/VF at follow-up, when compared to labile-obstructive (HR 0.18, 95%CI 0.04–0.84, p=0.03) and the risk persisted after adjusting for age, gender, syncope, family history of sudden cardiac death, abnormal blood pressure response and septum≥3cm (p=0.04). Appropriate defibrillator discharges were more frequent in non-obstructive [8(18%)] compared to labile-obstructive [0(0%), p=0.02] patients. In conclusion, non-obstructive hemodynamics is associated with more pronounced fibrosis and ischemia than labile-obstructive and is an independent predictor of VT/VF in HC.