[ANMCO立场文件:肥厚性心肌病:从诊断到治疗]。

IF 0.7 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS
Cristina Chimenti, Attilio Iacovoni, Andrea Montalto, Michele Emdin, Iacopo Olivotto, Cristina Basso, Benedetta Carla De Chiara, Irene Bottillo, Claudio Mario Ciampi, Santo Dellegrottaglie, Massimo Di Marco, Piero Gentile, Francesca Girolami, Paola Grammatico, Maria Iascone, Eluisa La Franca, Carla Lofiego, Andrea Matteucci, Daniele Pasqualucci, Samuele Pentiricci, Enrica Perugini, Maurizio Pieroni, Giovanni Quarta, Federica Re, Laura Scelsi, Giancarlo Todiere, Maria Alfarano, Monica De Gaspari, Claudio Bilato, Marco Corda, Leonardo De Luca, Giovanna Geraci, Massimo Milli, Alessandro Navazio, Vittorio Pascale, Carmine Riccio, Pietro Scicchitano, Emanuele Tizzani, Michele Massimo Gulizia, Federico Nardi, Domenico Gabrielli, Furio Colivicchi, Massimo Grimaldi, Fabrizio Oliva
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引用次数: 0

摘要

肥厚性心肌病(HCM)是一种非罕见的遗传性心肌病,估计患病率为1:500,其特征是在没有增加负荷条件下左心室壁厚度增加。肥厚大多不对称,主要影响基底隔和前壁。左心室流出道梗阻,在静息或刺激试验后,在许多患者中检测到,是功能能力下降的主要原因,也是心源性猝死和晚期心力衰竭的独立预测因素。直到大约1年前,有症状的患者,尽管使用-受体阻滞剂或钙通道阻滞剂进行了最大限度的治疗,有或没有使用双双酰胺,只有基底间隔缩小治疗,通过肌切除术或酒精性间隔消融作为额外的治疗选择。今天,一类抑制心肌肌球蛋白活性的新药可用于阻塞性HCM患者。鉴于新的治疗观点,对患病患者进行正确的临床治疗分类对心脏病专家来说至关重要。本立场文件的目的是增加心脏病专家在HCM领域的知识,定义其流行病学,遗传和病理特征,确定诊断标准和能够分层风险概况的工具方法,目的是为单个患者量身定制最佳治疗。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
[ANMCO Position paper: Hypertrophic cardiomyopathy: from diagnosis to treatment].

Hypertrophic cardiomyopathy (HCM) is a non-rare genetic cardiomyopathy, with an estimated prevalence of 1:500, characterized by an increase in the left ventricular wall thickness in absence of increased loading conditions. The hypertrophy is mostly asymmetric and predominantly affects the basal septum and anterior wall. Left ventricular outflow tract obstruction, at rest or after provocative tests, is detected in many patients and represents the primary cause of reduced functional capacity, as well as an independent predictor of sudden cardiac death and advanced heart failure. Until about 1 year ago, symptomatic patients despite maximal therapy with beta-blockers or calcium channel blockers, with or without disopyramide, had only basal septal reduction therapy via myectomy or alcohol septal ablation as additional therapeutic options. Today, a new class of drugs that inhibit cardiac myosin activity is available for patients with obstructive HCM.In light of the new treatment perspectives, the correct clinical-therapeutic classification of affected patients becomes of fundamental importance for the cardiologist. The aim of this position paper is to increase the knowledge of cardiologists in the field of HCM, defining its epidemiological, genetic and pathological characteristics, identifying the diagnostic criteria and instrumental methods capable of stratifying the risk profile, with the aim of an optimal therapy tailored on the single patient.

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Giornale italiano di cardiologia
Giornale italiano di cardiologia CARDIAC & CARDIOVASCULAR SYSTEMS-
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