Arrhythmias and sudden death in neuromuscular diseases

被引：1

作者：

Zecchin, Massimo ^{[1
]}

Calvanese, Raimondo ^{[2
]}

Pignalberi, Carlo ^{[3
]}

Mascioli, Giosue ^{[4
]}

Bardari, Stefano ^{[1
]}

China, Paolo ^{[5
]}

Sgarrito, Giuseppe ^{[6
]}

Nesti, Martina ^{[7
]}

机构：

[1] Azienda Sanit Univ Giuliano Isontina, Trieste, Italy

[2] Osped Mare, Naples, Italy

[3] PO San Filippo Neri ASL Roma 1, Rome, Italy

[4] Humanitas Gavazzeni, Bergamo, Italy

[5] Osped Angelo, Mestre Venezia, Italy

[6] ARNAS PO Civ & Benfratelli, Palermo, Italy

[7] Osped San Donato, Arezzo, Italy

来源：

GIORNALE ITALIANO DI CARDIOLOGIA | 2021年 / 22卷 / 06期

关键词：

Cardiomyopathy; Implantable cardioverter-defibrillator; Neuromuscular diseases; Pacemaker; Sudden death; DREIFUSS-MUSCULAR-DYSTROPHY; LAMIN A/C MUTATION; MYOTONIC-DYSTROPHY; CARDIAC INVOLVEMENT; DILATED CARDIOMYOPATHY; VENTRICULAR-ARRHYTHMIAS; CONDUCTION DISEASE; EUROPEAN-SOCIETY; HEART; RISK;

D O I：

10.1714/3612.35931

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

Neuromuscular diseases (NMD) are a broadly defined group of disorders that all involve injury or dysfunction of peripheral nerves, neuromuscular junction or muscle, often with dominant or recessive pattern of inheritance. Cardiac involvement is uncommon, in particular with cardiomyopathies and brady/tachyarrhythmias. The causes of cardiac involvement are unclear: replacement fibrosis, alteration of membrane permeability, sympathetic hyperactivity, or accumulation of toxic metabolites can play a role in the pathogenesis of cardiac disorders. The early diagnosis is of pivotal importance to prevent evolution of the disease: electrocardiographic alterations and arrhythmias, particularly if associated with family history for cardiomyopathy or sudden death, can be an early signal of cardiomyopathy associated with NMD. Genetic analysis can improve prognostic stratification, particularly related to arrhythmic risk, and guide to a tailored therapy.

引用

页码：466 / 479

页数：14

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