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[心脏性猝死年轻受害者存活家庭成员的心脏学评估:诊断结果]

[Cardiologic assessment of surviving family members of young victims of sudden cardiac death: diagnostic yield].

作者信息

Autore Camillo, Pagannone Erika, Re Federica, Romeo Daniela, Musumeci Beatrice, Baratta Pasquale, Marino Laura, Cauti Filippo Maria, Zachara Elisabetta

机构信息

Cattedra di Cardiologia, II Facoltà di Medicina e Chirurgia, Sapienza Università, Roma.

出版信息

G Ital Cardiol (Rome). 2010 Jul-Aug;11(7-8):557-65.

Abstract

Sudden cardiac death (SCD) is one of the most common causes of death in developed countries. In Italy, an annual incidence of 0.7 per 1000 inhabitants per year can be estimated. SCD represents the main cause of sudden death in children, adolescents and young adults and often occurs in young and previously asymptomatic patients. This issue has acquired even greater relevance since implantable cardioverter-defibrillators have proved to be highly effective in preventing sudden death in high-risk subjects. Autopsy findings of young SCD victims include inherited cardiac disorders with a defined morphologic substrate but also hearts without any identifiable structural abnormalities (sudden unexplained death). The potential heritability of the underlying disorder makes surviving relatives at risk of sudden death. A cardiological workup in these families may allow identification of cardiac disease and may unmask affected surviving relatives in whom the disease had remained unrecognized. Cardiological and genetic assessment of relatives of SCD victims based on current literature is reported in this review as well as our experience on SCD in young people in the Lazio Region (Italy) between 2001 and 2008.

摘要

心脏性猝死(SCD)是发达国家最常见的死亡原因之一。在意大利,据估计每年每1000名居民中有0.7例的发病率。SCD是儿童、青少年和年轻人猝死的主要原因,且常发生于年轻且此前无症状的患者。自从植入式心脏复律除颤器已被证明在预防高危人群的猝死方面非常有效以来,这个问题变得更加重要。年轻SCD受害者的尸检结果包括具有特定形态学基础的遗传性心脏疾病,但也有心脏没有任何可识别结构异常的情况(不明原因猝死)。潜在疾病的遗传可能性使存活的亲属有猝死风险。对这些家庭进行心脏病学检查可能有助于识别心脏疾病,并可能发现那些疾病未被识别的受影响存活亲属。本综述根据当前文献报道了SCD受害者亲属的心脏病学和遗传学评估,以及我们在2001年至2008年期间对意大利拉齐奥地区年轻人SCD的经验。

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