Cardiomyopathy Flashcards
What is cardiomyopathy?
Morphologically and functionally abnormal myocardium
What is the difference between and secondary cardiomyopathy?
Primary - Pathology predominantly involves the heart
Secondary due to other causes
Primary Cardiomyopathy:
There are 5 types - list them?
You will go through each one
Dilated cardiomyopathy
Hypertrophic cardiomyopathy
Restrictive cardiomyopathy
Arrhythmogenic right ventricular cardiomyopathy
Obliterative cardiomyopathy
PC - Dilated cardiomyopathy:
What does this lead to? - 2
THIS IS AUTOSOMAL DOMINANT so ask about FH
Inv:
What sort of things would you see on an ECG?
What further investigations need to be done to assess heart function? - 2
What imaging need to be done to exclude coronary artery disease?
BIOPSY may be needed if it is acute and causes HF.
Management:
- How are symptoms of HF managed?
- What may be implanted for arrhythmia’s?
- FINAL Rx IS TRANSPLANT
Coronary angiography
Poor systolic function
Ventricular dilatation
- Non-specific ST segment and T-wave changes
- Sinus tachycardia
- Arrhythmias
- Atrial fibrillation, VT
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CXR - shows enlargement
ECHO:
- Shows dilatation
- Shows valve insufficiency
Cardiac MRi could also be used!
ACE inhibitors and diuretics
ICD
PC - Hypertrophic cardiomyopathy:
Over how many mm is classed as myocardial hypertrophy?
How does this affect the ventricles?
Who does it commonly cause sudden death in?
THIS IS ONLY DIAGNOSED IF THERE IS NO OTHER CAUSATIVE HAEMODYNAMIC FACTOR (e.g. HTN, aortic valve diseases)
It is autosomal dominant with incomplete penetrance. What does incomplete penetrance mean?
> 15 mm
Smaller ventriclar vol
Young athletes
It skips a generation
PC - Hypertrophic cardiomyopathy:
What can HC be split into?
Obstructive and Non-obstructive
PC - Hypertrophic cardiomyopathy:
What does apical hypertrophy mean?
You get asymmetrical SEPTAL hypertrophy with or without obstruction.
They develop 4 interrelated processes:
- Left Ventricular Outflow Obstruction
- Mitral regurgitation
- Diastolic dysfunction
- Myocardial ischemia
What 2 things causes LV outflow obstruction?
What causes mitral regurg? What will patients complain of?
Diastolic dysfunction - what dos this mean? why does this cause exertion SOB?
Myocardial ischaemia - what is important to rule out before classifying this under hypertrophic cardiomyopathy?
(2) Systolic anterior motion (SAM) of mitral valve (Leaflets of mitral valve move towards enlarged septum during systole)
At the apex of the heart is where the enlargement is
(1) Asymmetrical hypertrophy of interventricular septum (ASH)
Systolic anterior motion of the mitral valve leaflets
Impaired ventricular relaxation
As the ventricles are not able to fill properly leading to impaired CO and raised pulmonary venous pressure
Occurs without atherosclerotic coronary artery disease
PC - Hypertrophic cardiomyopathy:
Symptoms - 3
Signs - what is seen on XR? - what is seen on ECG?
It may also just lead to SUDDEN death!
Examination:
- What may you notice when feeling the apex beat?
- They get a jerky carotid pulse. What does this mean?
- They get a jugular venous pulse due to decreased ventricular compliance!
- What extra heart sound may you hear? (SEEN IN GENERAL CARDIOMEGALY)
- Why do they get mitral regurg?
CP
SOB
Syncope with exertion - can’t maintain CO
Pul oedema
Cardiac arrhythmias (AF)
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Forceful atrial contraction
Short upstroke and prolonged systolic ejection
S4
Secondary to SAM (systolic anterior motion)
PC - Hypertrophic cardiomyopathy:
ECG:
- What are the septal leads?
- What sort of save is seen on ECG?
Echo - used to find everything else:
- Over how many mm is classed as hypertrophy of the left ventricle?
CMR can also be used. What does it stand for?
What can be done to fully confirm the diagnosis?
Septal Q waves
V1-V2
LVHJ»_space; 15MM
Cardiac Magnetic Resonance (CMR
Endomyocardial biopsy – shows fibrosis and will allow you to find out what the underlying pathology is
PC - Hypertrophic cardiomyopathy:
Medical Management:
Aim to treat symptoms and prevent sudden death!
Beta-blockers:
- How does this help? - 2
Verapamil:
- How does this help?
What is amiodarone used for?
Diuretics is used to relieve the symptoms of HF.
Why should vasodilators not be used?
- Increase ventricular diastolic filling/ relaxation
- # Decrease myocardial oxygen consumption
Augments ventricular diastolic filling/ relaxation
Anti-arrhythmias
Vasodilators may aggravate left ventricular outflow obstruction
PC - Hypertrophic cardiomyopathy:
Surgical Management:
What would be used to control the arrthymias to prevent sudden cardiac death? (Used in combo with meds)
Surgical septal myomctomy - what is it?
Alcohol can be used to ablate some of the septum. What are 2 complications of this because of the toxicity of the alcohol?
ICD - Implantable Cardioverter Defibrillator
Remove small portion of upper septum
+/- mitral valve replacement
- Complete heart block – toxicity
- Large myocardial infarctions – 2* to alcohol
PC - Hypertrophic cardiomyopathy:
How do differentiate HCM vs athlete heart?
Athlete’s heart is symmetrical!!!!!
Restrictive cardiomyopathy:
How does this lead to heart failure?
It can be either primary (idiopathic) or secondary. What diseases causes infiltration into the myocardium? - 5
Restrictive CM and constrictive pericarditis can present quite similarly.
Kussmaul’s sign is definitely seen in pericarditis. What is it?
What difference is seen on echo?
Impaired ventricular filling due to diatstolic insufficiency
Ventricles stiff and rigid
Increased tension of ventricular filling
Amyloidosis Sarcoidosis Haemochromatosis Scleroderma Glycogen storage disease of the heart
Kussmaul’s signis a paradoxical rise in jugular venous pressure (JVP) on inspiration, or a failure in the appropriate fall of the JVP with inspiration. It can be seen in some forms of heart disease and is usually indicative of limited right ventricular filling due to right heart dysfunction.
CP would have a normal wall thickness, whereas in RCM, it is thickened
Restrictive cardiomyopathy:
What are the main 2 symptoms?
Due to difficulty filling the right ventricle, what are 4 manifestations of this?
What extra heart sound is head with any type cardiomyopathy?
Fatigue
SOB
Increased JVP
Hepatic enlargement
Ascites
Oedema
S4
Restrictive cardiomyopathy:
ECG:
- What should you notice on the ECG if it is RESTRICTIVE?
CXR:
- 2 signs?
What 2 other imaging can be used?
What final thing can be done?
There is no specific Rx:
- Manage HF and embolic manifestations
Low voltage QRS, ST and T-wave changes
Cardiomegaly
Pulmonary venous congestion
Echo
CMR
Endomyocardial biopsy – shows fibrosis and will allow you to find out what the underlying pathology is
