Sarquella-Brugada Georgia, Cesar Sergi, Zambrano Maria Dolores, Fernandez-Falgueras Anna, Fiol Victoria, Iglesias Anna, Torres Francesc, Garcia-Algar Oscar, Arbelo Elena, Brugada Josep, Brugada Ramon, Campuzano Oscar
Arrhythmias Unit, Hospital Sant Joan de Deu, University of Barcelona, Barcelona, Spain.
Medical Science Department, School of Medicine, University of Girona, Girona, Spain.
Curr Cardiol Rev. 2019;15(1):30-37. doi: 10.2174/1573403X14666180913114806.
Sudden death of a newborn is a rare entity, which may be caused by genetic cardiac arrhythmias. Among these diseases, Long QT syndrome is the most prevalent arrhythmia in neonates, but other diseases such as Brugada syndrome, Short QT syndrome and Catecholaminergic Polymorphic Ventricular Tachycardia also cause sudden death in infants. All these entities are characterized by well-known alterations in the electrocardiogram and the first symptom of the disease may be an unexpected death. Despite the low prevalence of these diseases, the performance of an electrocardiogram in the first hours or days after birth could help identify these electrical disruptions and adopt preventive measures. In recent years, there has been an important impulse by some experts in the scientific community towards the initiation of a newborn electrocardiogram-screening program, for the detection of these electrocardiographic abnormalities. In addition, the use of genetic analysis in neonates could identify the cause of these heart alterations. Identification of relatives carrying the genetic alteration associated with the disease allows adoption of measures to prevent lethal episodes.
Recent technological advances enable a comprehensive genetic screening of a large number of genes in a cost-effective way. However, the interpretation of genetic data and its translation into clinical practice are the main challenges for cardiologists and geneticists. However, there is important controversy as to the clinical value, and cost-effectiveness of the use of electrocardiogram as well as of genetic testing to detect these cases. Our review focuses on these current matters of argue.
新生儿猝死是一种罕见的情况,可能由遗传性心律失常引起。在这些疾病中,长QT综合征是新生儿中最常见的心律失常,但其他疾病如Brugada综合征、短QT综合征和儿茶酚胺能多形性室性心动过速也会导致婴儿猝死。所有这些疾病的特征都是心电图有众所周知的改变,而疾病的首发症状可能是意外死亡。尽管这些疾病的发病率较低,但在出生后的最初几个小时或几天内进行心电图检查有助于识别这些电紊乱并采取预防措施。近年来,科学界的一些专家大力推动启动新生儿心电图筛查项目,以检测这些心电图异常。此外,对新生儿进行基因分析可以确定这些心脏改变的原因。识别携带与疾病相关的基因改变的亲属,有助于采取措施预防致命发作。
最近的技术进步使人们能够以经济有效的方式对大量基因进行全面的基因筛查。然而,对基因数据的解读及其转化为临床实践是心脏病专家和遗传学家面临的主要挑战。然而,对于使用心电图以及基因检测来检测这些病例的临床价值和成本效益存在重要争议。我们的综述聚焦于这些当前有争议的问题。