收缩期功能障碍对小儿孤立性左心室非压实性临床及诊断参数的影响。

IF 0.9 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS
Fatma Sevinc Şengul, Pelin Ayyıldız, Sezen Ugan Atik, Sinem Aydin, Alper Güzeltaş, Yakup Ergül
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引用次数: 0

摘要

目的:左心室不压实是一种罕见的心内膜形态发生早期停止的心肌病。本研究旨在介绍小儿左室非压实的临床和心电图特点、诊断特点、治疗策略、收缩功能障碍对临床和诊断参数的影响以及随访情况。方法:回顾性分析2010年1月至2020年6月在Mehmet Akif Ersoy胸心血管外科培训与研究医院就诊的孤立性左心室不压实患儿。结果:55例患儿被诊断为左心室不压实。32例(58.2%)为男性,中位发病年龄为8.5岁(1个月,17.9岁)。中位随访时间为19个月(1-121个月)。14例(25.5%)出现收缩功能障碍(射血分数< 45%),2例出现复苏/流产的心脏骤停。78.2%的患者出现心电图异常。6例患者QRS片段化,17例(30.9%)QTc持续时间在450毫秒及以上。射血分数< 45%组多见心电图异常、QRS电压低、QRS碎片化、血栓形成。在射血分数< 45%和≥45%组中,房性和室性心律失常(包括室性颤动- vf)的发生率相似。1例完全性房室传导阻滞患者和1例长QT综合征和严重心动过缓患者接受了永久性起搏器植入。5例(9.1%)患者死亡。结论:儿童期左心室不致密性具有不同的临床表现。随着病程的发展,有必要密切随访患者是否出现室性功能障碍或心律失常。需要进一步的研究,因为即使在保留射血分数的患者中也可以看到危及生命的室性心律失常。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Effects of Systolic Dysfunction on Clinical and Diagnostic Parameters in Pediatric Patients with Isolated Left Ventricular Non-compaction.

Objective: Left ventricular non-compaction is a rare cardiomyopathy following an early arrest in endomyocardial morphogenesis. This study aimed to present the clinical and electrocardiographic characteristics, diagnostic features, treatment strategies, effects of systolic dysfunction on clinical and diagnostic parameters, and follow-up of pediatric patients diagnosed with left ventricular non-compaction.

Methods: We retrospectively reviewed children with isolated left ventricular non-compaction at Mehmet Akif Ersoy Thoracic and Cardiovascular Surgery Training and Research Hospital from January 2010 to June 2020.

Results: Fifty-five children were diagnosed with left ventricular non-compaction. Thirty-two patients (58.2%) were male, and the median age of presentation was 8.5 years (1 month-17.9 years). The median follow-up of the study was 19 months (1-121 months). Fourteen (25.5%) presented with systolic dysfunction (ejection fraction < 45%), and 2 presented with resuscitated/aborted cardiac arrest. Electrocardiographic abnormalities were present in 78.2%. Fragmented QRS was observed in 6 patients, and QTc duration was 450 milliseconds and above in 17 patients (30.9%). Electrocardiographic abnormalities, low QRS voltage, fragmented QRS, and thrombus were common in patients with ejection fraction < 45% group. Atrial and ventricular arrhythmias (including ventricular fibrillation-VF) were found with similar frequency in both ejection fraction < 45% and ≥45% groups. One patient with a complete atrioventricular block and 1 with long QT syndrome and severe bradycardia underwent permanent pacemaker implantation. Five (9.1%) patients died.

Conclusions: Left ventricular non-compaction has heterogeneous clinical findings in childhood. It is essential to follow-up with the patients closely for the development of ventricular dysfunction or arrhythmias due to the progressive course of the disease. Further studies are needed since life-threatening ventricular arrhythmias can be seen, even in patients with preserved ejection fraction.

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来源期刊
CiteScore
1.30
自引率
12.50%
发文量
124
审稿时长
32 weeks
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