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首页> 外文期刊>The American Journal of Cardiology >Surgical Ventricular Septal Myectomy for Patients With Noonan Syndrome and Symptomatic Left Ventricular Outflow Tract Obstruction
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Surgical Ventricular Septal Myectomy for Patients With Noonan Syndrome and Symptomatic Left Ventricular Outflow Tract Obstruction

机译：伴有症状性左心室流出道梗阻的Noanan综合征患者的外科室间隔肌切开术

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摘要

Apprmdmately 20% to 30% of patients with Noonan syndrome (NS) have asymmetric left ventricular hypertrophy (LVH) and LV outflow tract obstruction (LVOTO). The role of surgical myectomy in such patients is unknown. We sought to compare clinical features and outcomes of patients with NS and LVOTO with age- and gender-matched patients vvith nonsyndromic, obstructive hypertrophic cardiomyopathy (HC) after myectomy. Two cohorts were selected and retrospectively analyzed using Mayo Clinic databases from 1996 to 2014. Subjects included patients with NS with LVH and LVOTO and nonsyndromic controls with obstructive HC. Twenty-three patients with NS and LVH were identified, of whom 12 (8 males) underwent myectomy (10 septal and 2 combined septal/apical) for severe LVOTO (10 pediatric and 2 adults; 13 10 year old [range 1 to 39]). Similar echocardiographic improvements were noted in both groups. There were no perioperative deaths. Residual gradients were slightly higher in patients with NS. No improvement was noted in left atrial volume after myectomy in patients with NS. At early follow-up, the majority showed improvement in the New York Heart Association class (88% in NS vs 82% in HC, median of 6 and 2 months, respectively). At late follow-up (median of 7 years), the survival rate was 92% in NS and 100% in HC. In patients with NS with LVH and symptomatic LVOTO, myectomy reduces both gradient and the New York Heart Association class, similar to patients with nonsyndromic obstructive HC. Residual gradients were slightly higher, and left atrial dilation persisted in patients with NS. In conclusion, myectomy should be considered in patients older than 1 year with NS and symptomatic LVOTO. (C) 2015 Elsevier Inc. All rights reserved.
机译：约有20％至30％的Noonan综合征（NS）患者患有不对称的左心室肥大（LVH）和左室流出道梗阻（LVOTO）。在这类患者中，手术切除术的作用尚不清楚。我们试图比较非综合征，梗阻性肥厚型心肌病（HC）的NS患者和LVOTO患者的临床特征和结局，以及年龄和性别相匹配的患者。从1996年至2014年，选择了两个队列并使用Mayo Clinic数据库进行了回顾性分析。对象包括患有LVH和LVOTO的NS患者以及阻塞性HC的非综合征患者。鉴定出23例NS和LVH患者，其中12例（8例男性）接受了严重LVOTO的子宫切除术（10例中隔和2例中隔/根尖联合）（10例儿科和2例成人; 13例10岁[范围1至39]） ）。两组均发现了类似的超声心动图改善。没有围手术期死亡。 NS患者的残留梯度略高。 NS患者进行肌瘤切除术后左心房容积无改善。在早期随访中，大多数患者表现出纽约心脏协会等级的改善（NS组为88％，HC组为82％，中位数分别为6个月和2个月）。晚期随访（中位数为7年），NS的生存率为92％，HC的生存率为100％。在患有LVH和症状性LVOTO的NS患者中，与非综合征性阻塞性HC患者相似，肌切除术可同时降低梯度和纽约心脏协会等级。 NS患者的残留梯度略高，并且左房扩张持续。总之，患有NS和有症状LVOTO的1岁以上患者应考虑行肌瘤切除术。 （C）2015 Elsevier Inc.保留所有权利。

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