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Cardiomyopathy Flashcards

1
Q

classification of cardiomyopathy

A

Dilated cardiomyopathy
Hypertrophic cardiomyopathy

Restrictive cardiomyopathy

Arrhythmogenic RV cardiomyopathy

(Unclassified)

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2
Q

cardiomyopathies

A
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3
Q

hypertrophic cardiomyopathy

A

Genetic cardiac disease [AD]
Incidence 1:500
Variable genetic, structural, clinical expression
Asymmetric LVH
Variable site
Variable severity
Differential diagnosis: AS, sub-AS, LVH …, athletes heart

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4
Q

sarconmere mutations in HCM

A

Beta myosin heavy chain (35%)

Myosin-binding protein C (15%)

Troponin T (15%)

alpha-Tropomyosin (5%)

Myosin light chain (1%)

Cardiac contraction occurs when calcium binds the troponin complex (subunits C, I, and T) and alpha-tropomyosin. Actin stimulates ATPase activity in the globular myosin head and results in the production of force along actin filaments. Cardiac myosin-binding protein C binds myosin and modulates contraction. In hypertrophic cardiomyopthy, mutations may impair these and other protein interactions, result in ineffectual contraction, and produce hypertrophy. Percentages represent the estimated frequency with which a mutation causes hypertrophic cardiomyopathy

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5
Q

hcm history

A

Dyspnoea/fatigue
i.e. heart failure symptoms

Angina
normal coronary arteries

Palpitations/collapse

Family history

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6
Q

hcm examination

A

Cardiomegaly
Apical systolic thrill/heave
Brisk/jerky carotid pulse
S4
Ejection systolic murmur
(increase with Valsalva, GTN, exercise)

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7
Q

hcm ecg

A

Q waves
LVH
Abnormal ST Segments
T wave inversion

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8
Q

echo hcm

A

LVH
LVOT obstruction
Exclude AS etc

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9
Q

scd

A

Commonest cause of SCD in young

Frequently exertional (sport)

Ventricular tachyarrhythmias

  • Disorganised myocyte architecture
  • Microvascular abnormalities
  • Ischaemic bursts
  • Replacement scarring

High risk: ICD

  • VT
  • FHx SCD
  • decrease BP
  • increase LVH
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10
Q

scd treatment

A

Symptoms
Beta blockers
Verapamil
Disopyramide
Septal ablation
Myectomy MV repair/replacement

Risk
ICD
Avoid exercise

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11
Q

dilated cardiomyopathy

A

Cardiac enlargement and systolic dysfunction

Common (25%) cause of heart failure

Idiopathic (>50% cases)

  • Genetic factors/family history
  • Viral myocarditis
  • Immunological abnormalities

Identifiable cause in <50% …

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12
Q

dilated cardiomyopathy history

A

Heart failure

  • Dyspnoea
  • Fatigue
  • Fluid congestion

systemic emboli

Family history
Viral illness
Other causes

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13
Q

DCM examination

A

Fluid congestion
- Raised JVP, ankle oedema

Cardiomegaly

S3, S4

Secondary MR, TR

Other causes*

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14
Q

HCM investigation

A

ECG (non-specific)

  • ST, T wave changes
  • SVT, VT
  • Heart block

CXR (non-specific)

  • Cardiolomegaly
  • Pulmonary venous congestion/oedema

Blood/other tests (specific)
- E.g. Iron, Copper, thyroid, B12

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15
Q

HCM treatment

A

Heart failure Rx
ACE inhibitors, beta blockers, diuretics
Device therapy
Biventricular pacing, ICD
Cardiac transplantation

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16
Q

restrictive cardiomyopathy

A

Group of heart muscle disorders characterised by …
Thickened, “stiff” myocardium
Reduced LV compliance
Diastolic dysfunction
±“systolic function” preserved

PRIMARY restrictive cardiomyopathy
SECONDARY …

17
Q

restrictive crdiomyoparthy

18
Q

history of rwstrictive

A

Congestive heart failure

  • Dyspnoea, fatigue
  • Fluid congestion

Symptoms of systemic disease (eg amyloid)

19
Q

restrictive examination

A

Mild cardiomegaly
S3 or S4
increase JVP

20
Q

restrctive ecg

A 
Low voltages (despite thick myocardium)
 Heart block
21
Q

restrictive echo

A

Small ventricular cavities
Ventricular hypertrophy
(impaired contraction)
Abnormal myocardial texture
increase thickness atrial septum, valves
Bi-atrial enlargement

22
Q

hypereosiniphilic heart disease (RCM) - Loeffler’s endocardial fibrosis

A

response to 1º or 2º eosinophilia

direct toxic effect on endocardium

  • free radical mediated
  • inflammatory phase: myocarditis
  • thrombotic phase
  • fibrotic phase: “RCM”
23
Q

rcm treatment

A

Heart failure drugs

Specific therapies

  • E.g. Hypereosinophilic RCM
  • Warfarin
  • Steroids
24
Q

arrythmogenic RV cardiomyopathy

A

Rare, familial

Diffuse/segmental RV…

  • Myocyte death
  • Fibrous/fatty replacement
  • Myocarditis

RV (LV) enlargement
- Systolic dysfunction

Ventricular arrhythmia
- May be presenting feature

25
Q

ARVC gives an epsilon wave

26
Q

ARVC echo and mri

A

Focal/diffuse RV…

  • echodensity
  • dilatation
  • dysfunction
27
Q

ARVC treatmenr

A

Avoid exercise (sport)
Defibrillator
Family screening

Cardiology (23 decks)

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