cardiomyopathy Flashcards
types
- hypertrophic
- arrhythmogenic
- dilated
what do they affect of the heart
mechanical or electrical function of the heart
what are the secondary causes
- infiltrative
- storage related (haemochromatosis)
- drugs (alcohol)
- neuromuscular
- nutritional
- autoimmune (systemic lupus)
- cancer therapy
what are some causes of hypertrophic cardiomyopathy
- aortic stenosis
- hypertension
what is the most common cause of sudden death in young people
hypertrophic cardiomyopathy
what kind of inheritance is hypertrophic cardiomyopathy
familial autosomal dominant
what are some mutations that cause hypertrophic cardiomyopathy
- genes encoding sarcomeric proteins
- ß-myosin heavy chain MYH7
- myosin bingin protein C
- troponin T and I
- alpha myosin heavy chain
where does hypertrophic cardiomyopathy involve
interventricular septum
what can ventricular ejection cause
mitral regurgitation
what are some characteristics of a mutation in troponin T
- mild hypertrophy
- high risk of sudden death
when does hypertrophy due to myosin binding protein manifest
not until sixth decade or later
symptoms of hypertrophic cardiomyopathy
- asymptotic
- chest pain
- SOB
- syncope
- arrhythmias
- sudden death
signs of hypertrophic cardiomyopathy
- double apical pulsation
- jerky carotid pulse
- ejection systolic murmur
- pan systolic murmur
- fourth heart sound
why is there a jerky carotid pulse in hypertrophic cardiomyopathy
rapid ejection and sudden obstruction to left ventricular outflow
why is there ejection systolic murmur in hypertrophic cardiomyopathy
due to left ventricular outflow obstruction late in systole
why is there pan systolic murmur in hypertrophic cardiomyopathy
due to mitral regurgitation
what investigations are done for hypertrophic cardiomyopathy
- ECG
- echo
- CMR
- genetic analysis
what is seen in ECG of hypertrophic cardiomyopathy
- left ventricular hypertrophy
- ST and T wave changes
- abnormal Q waves
what is seen in echo of hypertrophic cardiomyopathy
- asymmetric left ventricular hypertrophy
- vigorously contracting ventricle
- patterns of hypertrophy
what is diagnostic for hypertrophic cardiomyopathy
echo
what can CMR detect in hypertrophic cardiomyopathy
- hypertrophy
- abnormal myocardial fibrosis
treatment of hypertrophic cardiomyopathy
treatment of symptoms and prevent sudden death
what are the risk factors of sudden death in hypertrophic cardiomyopathy
- massive left ventricular hypertrophy
- family history of sudden death
- non-sustained ventricular tachycardia
- prior unexplained syncope
- abnormal blood pressure on exercise
what is given if low risk of sudden death in hypertrophic cardiomyopathy
amiodarone
what is given if high risk of sudden death in hypertrophic cardiomyopathy
ICD insertion
what is given for chest pain and dyspnoea in hypertrophic cardiomyopathy
beta blockers and verapamil
alone or in combo
what is given for left ventricular outflow tract obstruction in hypertrophic cardiomyopathy
disopyramide
what drugs should be avoided in hypertrophic cardiomyopathy
vasodilators as they may aggravate left ventricular outflow or cause refractory hypotension
where does the arrhythmogenic cardiomyopathy affect
right ventricle
what are the myocytes replaced with in arrhythmogenic cardiomyopathy
fatty or fibro-fatty replacements
what does fibrosis-fatty replacement lead to in arrhythmogenic cardiomyopathy
ventricular arrhythmia
what mutations are there in arrhythmogenic cardiomyopathy
in genes coding for desmosomal proteins
what is Naxos disease associated with
palmoplantar keratoderma and wooly hair
what is Naxos disease due to
mutation in junctional plakoglobin
what is Carvajal syndrome due to
mutation in desmoplakin
what are the 2 recessive forms of arrhythmogenic cardiomyopathy
- Naxos disease
- Carvajal syndrome
symptoms of arrhythmogenic cardiomyopathy
- asymptomatic
- syncope
- sudden death
- signs of right heart failure
what investigations are done for arrhythmogenic cardiomyopathy
- ECG
- echo
- CMR
- genetic test
what is seen on ECG of arrhythmogenic cardiomyopathy
- T wave inversion in precordial leads related to right ventricle
- right uncle branch block
- delayed depolarisations
what is seen on echo of arrhythmogenic cardiomyopathy
right ventricular dilatation and aneurysm formation
left ventricular dilatation
what is seen on CMR of arrhythmogenic cardiomyopathy
can assess right ventricle more accurately
can demonstrate fibro-fattty infiltration
what can be the gold standard for arrhythmogenic cardiomyopathy
genetic testing
what is first line treatment for arrhythmogenic cardiomyopathy
beta blockers
what is treatment for symptomatic arrhythmias 1
amiodarone or sotalol
what is done for life-threatening arrhythmogenic cardiomyopathy
ICD
what is dilated cardiomyopathy characterised by
dilatation of ventricular chambers
and
systolic dysfunction with preserved wall thickness
what kind of inheritance is dilated cardiomyopathy
autosomal dominant
what are causes of sporadic dilated cardiomyopathy
- myocarditis
- toxins
- autoimmune
- endocrine
- neuromuscular
what does dilated cardiomyopathy present with
- heart failure
- cardiac arrrhythmias
- condition defects
- thromboembolism
- sudden death
what investigation are done for dilated cardiomyopathy
- CXR
- ECG
- echo
- CMR
- coronary angiography
- biopsy
what is seen on CXR for dilated cardiomyopathy
cardiac enlargement
what is seen on ECG for dilated cardiomyopathy
- non specific ST and T wave changes
- sinus tachycardia
- arrhythmias
what is seen on echo of dilated cardiomyopathy
- dilatation of left and/or right ventricle with poor contraction function
what is seen on CMR of dilated cardiomyopathy
can show other causes of left ventricular dysfunction
or
abnormal myocardial fibrosis
what are treatment options of dilated cardiomyopathy
- cardiac resynchronisation
- ICDs
- cardiac transplantation
what is the appearance of left ventricular non-compaction
sponge like appearance of left ventricle
where does left ventricular non-compaction affect
apical portion of the left ventricle
how is left ventricular non-compaction diagnosed
- echo
- CMR
- left ventricular angiography
what is Primary restrictive non-hypertrophic cardiomyopathy
rare condition in which there is normal or decreased volume of both ventricles with bi-atrial enlargement, normal wall thickness, normal cardiac valves and impaired ventricular function
what condition are associated with primary restrictive non-hypertrophic cardiomyopathy
- amyloidosis
- sarcoidosis
- Loefflers endocarditis
- end-myocardial fibrosis
what investigations are done for primary restrictive non-hypertrophic cardiomyopathy
- CXR
- ECG
- echo
- CMR
- cardiac catheterisation
- biopsy
what is seen on echo of Primary restrictive non-hypertrophic cardiomyopathy
- symmetrical myocardial thickening
- notmal systolic ejection fraction
- imparted ventricular filling
- myocardium is speckled
- tramlines on M mode echo
treatment for Primary restrictive non-hypertrophic cardiomyopathy
no specific treatment
what treatment is sometimes done for Primary restrictive non-hypertrophic cardiomyopathy
cardiac transplantaiton
what is effective for familial amyloidosis
liver transplantation
