Cardiomyopathies: HOCM, Dilated, Arrythmogenic RV

Question 1

Types of cardiomyopathy and their possible presentations

Answer 1

Acquired
Inherited

Can present in a variety of ways

• dilated
• hypertrophic
• restrictive (stiff walls)
• with arrythmias

Question 2

Inherited cardiomyopathies
Main issues
-dilated cardiomyopathy
-hypertrophic cardiomyopathies
-arrythmogenic cardiomyopathies

Answer 2

Dilated cardiomyopathy - cell structure => stiff tissues, difficulty contracting

Hypertrophic cardiomyopathy - muscle contraction issues => difficulty relaxing

Arrythmogenic RV cardiomyopathy - conduction and electrical issues => arrythmias

Question 3

HOCM

-pathophysiology

Answer 3

AD genetic disorder - most common cause of SCD in young

Diastolic dysfunction
-LVH => stiff => low CO

Question 4

HOCM

• presentation
• signs

Answer 4

Asymptomatic
SOBOE
Angina
Syncope post exercise
SCD from ventricular arrythmia, HF

Ejection systolic murmur

• increases with Valsalva
• decreases with squats

MR
SAM of ant mitral valve leaflet (VSH makes cords less taut)
Asymmetric hypertrophy

Question 5

HOCM

-Investigations and findings

Answer 5

ECG

• LVH
• ST, T abnormalities
• Deep Q waves

Echo

Question 6

HOCM

-Associated conditions

Answer 6

Friedreich’s ataxia

Wolff-Parkinson white

Question 7

HOCM

-Management

Answer 7

High risk of SCD - VT caused by ischemia on exertion
-ICD

Low risk of SCD, asymptomatic - watch and wait

Question 8

Dilated cardiomyopathy

• epidemiology
• causes

Answer 8

Most common cardiomyopathy

• most are idiopathic
• myocarditis, CHD, HTN
• peripartum
• alcohol, cocaine
• FHx, DMD

Question 9

Dilated cardiomyopathy

• pathophysiology
• presentation

Answer 9

Dilated heart => weak muscle => systolic dysfunction
Eccentric hypertrophy

Heart failure findings
Systolic murmur - stretching of valves => regurgitation
Balloon appearance on CXR

Question 10

Arrythmogenic right ventricular cardiomyopathy

• pathophysiology
• presentation
• management

Answer 10

AD
2nd most common form of SCD

Palpitations
Syncope
SCD

Soltalol
Catheter ablation => prevent VT
ICD