심근병증 개요

기본 개념

심근병증

  • 정의: 선천성, 판막 질환, 고혈압, 관동맥 질환, 심낭 질환 등 다른 심장 질환 없이 심장 근육에 이상이 발생하는 여러 질환군. 흔히 유전적 문제로 발생한다.

  • 분류

  • DCM, HCM, RCM 개요

    • 확장성 심근증(Dilated Cardiomyopathy, DCM): “심장이 커짐”

    • 비후성 심근증(Hypertrophic Cardiomyopathy, HCM): “심장이 두꺼워짐”

    • 제한성 심근증(Restrictive Cardiomyopathy, RCM): “심장이 잘 안늘어남”


** Typical Presentation with Symptomatic Cardiomyopathy (출처: Harrison)


DILATED

RESTRICTIVE

HYPERTROPHIC

Ejection fraction (normal >55%)

Usually <30% when symptoms severe

Usually >40-50%

>60%

Left ventricular diastolic dimension (normal <55 mm)

>60 mm if chronic

<60 mm (may be decreased)

Often decreased

Left ventricular wall thickness

Normal or decreased

Normal or increased

Markedly increased

Atrial size

Increased, left before right

Increased; may be massive and involve both atria equally

Increased; related to elevated filling pressures

Valvular regurgitation

Related to annular and ventricular dilation; mitral appears early during decompensation; tricuspid regurgitation with right ventricular dysfunction

Related to endocardial involvement; frequent mitral and tricuspid regurgitation, rarely severe

Related to valve-septum interaction; mitral regurgitation

Common first symptoms

Exertional intolerance

Exertional intolerance, fluid retention early, may have dominant right-sided symptoms

Exertional intolerance; may have chest pain

Congestive symptoms*

Left before right, except right prominent in young adults

Right often dominates

Left-sided congestion at rest may develop late

Arrhythmias

Ventricular tachyarrhythmia; conduction block in Chagas' disease, and some genetic etiologies. Atrial fibrillation

Conduction disease is common in amyloidosis, in which ventricular arrhythmias are uncommon. Atrial fibrillation is very common

Ventricular tachyarrhythmias; atrial fibrillation


[1] Harrison 21e, pg.1954-1955

[2] AHA - Contemporary Definitions and Classification of the Cardiomyopathies, pg.1809-1810, 1814

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