Cardiomyopathy Flashcards

1
Q

What is a cardiomyopathy and what can it lead to?

A

Disease of heart muscle making it harder to pump blood to body

can lead to heart failure

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

What main types of cardiomyopathy are there?

A

Dilated

Hypertrophic

Restrictive

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

What is dilated cardiomyopathy?

A

progressive
usually irreversible

causing contractile dysfunction with heart failure

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

What are the causes of dilated cardiomyopathy?

A

25% familial

secondary causes : heart valve disease
after child birth
thyroid disease

myocarditis
alcoholism 
autoimmune
ingestion of drugs
mitocondrial disorders
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

What is the pathophysiology of dilated cardiomyopathy?

A

Enlarged L ventricle

–> lower EF, increase in V wall stress and ESV

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

What are the early compensatory mechanisms in dilated cardiomyopathy?

A

increase in heart rate and tone of peripheral vascular system

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

What hormones or peptides increase in circulation due to dilated cardiomyopathy?

A

Neurohumoral activation of renin-angiotensin aldesterone system and an increase in CATECHOLAMINES in system

levels of NATRIURETIC peptides

these increase as part of the compensatory mechanisms however these can become overhwlemed and heart will fail

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

How will a person with dilated myopathy present?

A
Dyspnoea 
Murmur
Fatigue
angina
pulmonary congestion
low cardiac output 
displaced apex beat, S3 or systolic
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

What investigations are done for dilated cardiomyopathy?

A
Genetic (familail)
viral serology
ECG
chest C ray
Cardiac cathetar
cardiac MRI/CT
exercise stress test
echocardiography
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

What diet modifications should be made for dilated myopathy?

A

Fluid and Na+ restriction

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

If the underlying cause of dilated cardiomyopathy is sarcoidosis / myocarditis what treatment is given?

A

Immunosuppressants

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

If the underlying cause of dilated cardiomyopathy is haemochromatosis what treatment is given

A

Phlebotomy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

When would you lower ACE inhibitor dose for heart failure?

A

If the px develops cough, hypertension, renal dysfunction

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Wha is given for arrhythmias based on dilated cardiomyopathy?

A

Amiodarone

Dofetillide

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

What is hypertrophic cardiomyopathy?

A

Increase in left ventricular wall thickness that is not solely explained by abnormal loading conditions

Genetic - autosomal dominant in 50% of cases

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

Why is hypertrophic cardiomyopathy the leading cause of sudden cardiac death in children and adolescents?

A

Most px are asymptomatic however this means the first clinical manifestation may be sudden death from ventricular tachycardia or fibrillation

17
Q

Where does the hypertrophy usually occur in the left ventricle?

A

Although it can occur in any region of LV

usually involved interventricular septum which obstructs the flow of blood from smaller LV to the aorta

18
Q

How is the diastolic function in hypertrophic cardiomyopathy px?

A

abnormal function, impairs ventricular filling and increases filling pressure

these px have abnormal calcium kinetics, subendocardial ischaemia

19
Q

How may a px with hypertrophic cardiomyopathy present?

A
  • Sudden cardiac death
  • S3
  • ejection systolic murmur
  • Gallop
  • Syncope
  • presyncope
  • congestive heart failure
  • dizziness
  • palpitations
  • angina
20
Q

What investigations are done hypertrophic cardiomyopathy?

A

Haemoglobin levels - anaemia worsens chest pain and dyspnea

Brain natriuretic peptide (BNP), troponin T levels: Elevated BNP, NT-proBNP, and troponin T levels are associated with a higher risk of cardiovascular events, heart failure, and death

Echocardiography
Chest Xray
Cardiac MRI

21
Q

How is HCM managed?

A

The symptoms are managed with B blocker, the side effects of which are reduced by verapamil

add disopyramide if symptoms persist

if there are refactory symptoms then undergo mechanical therapy

22
Q

What mechanical treatments are avaliable for HCM?

A

?PM with short AV delay

if further invasive is required due to refractory symptoms –> Septal myectomy or ablation

23
Q

What is restrictive cardiomyopathy?

A

diagnosis is based on establishing the presence of restrictive ventricular filling

characterized by:
-diastolic dysfunction with restrictive ventricular physiology

  • systolic function remains normal.
  • Atrial enlargement occurs due to impaired ventricular filling during diastole
  • but the volume and wall thickness of the ventricles are usually normal.
24
Q

What may cause RCM?

A

idiopathic, familial (has been related to troponin I or desmin mutations, the latter often in association with a skeletal myopathy),

systemic disorders:
- haemochromatosis, - amyloidosis
- sarcoidosis
- Fabry's disease
- carcinoid syndrome
- scleroderma, anthracycline toxicity
or previous radiation.
25
Q

What is the pathophysiology of RCM?

A

stiff myocardium, this can be due to infiltrative cardiomyopathies

the stiffening causes diastolic dysfunction:

the stiffness causes –> rise in V pressure –> diastolic filling terminates abruptly at the end of rapid filling

ventricle filling volume is reduced leading to reduced cardiac output

26
Q

What are infiltrative cardiomyopathies?

A

Infiltrative cardiomyopathies are characterised by deposition of abnormal substances (ie, amyloid proteins, noncaseating granulomas, iron) within the heart tissue.

27
Q

What may advance cases of RCM present with?

A

Adverse remodelling may lead to systolic dysfunction and ventricular arrhythmias in advances cases

28
Q

How will a px with RCM present?

A

comfortable whilst seated due to fluid in abdomen or lungs

frequently have ascites and pitting edema in lower extremeties

Weight loss
cardiac cachexia

Liver enlarged and full of fluid which may be painful

Easy bruising, periorbital purpura, macroglossia, and other systemic findings, such as carpal tunnel syndrome, (consider amyloidosis)

Increased jugular venous pressure is present.

pulse volume decreased, alongside SV + CO

29
Q

How to manage RCM?

A

Heart failure management: including angiotensin-converting enzyme inhibitors or angiotensin receptor II blockers, diuretics and aldosterone inhibitors should be initiated in patients with reduced LV

  • Antiarrhythmic Therapy
  • Immunosuppression- Steroids
  • Pacemaker
  • Cardiac transplantation