Cardiomyopathy Flashcards
What are the different types of cardiomyopathy?
- Hypertrophic,
- dilated,
- restictive
- myocarditis
Name the different types of pericardial disease?
-Pericarditis and effusion +/- tamponade
What is dilated cardiomyopathy?
Structural and functional discription– the ventricular function is impaired
Can be primary or secondary
What is the aetiological backround of dilated cardiomyopathy?
Ischaemia and valvular causes
- Genetic
- Inflammatory, infectious, autoimmune, postpartum
-Toxic, drugs, exogenous chemicals ,endocrine
injury, cell loss,scar replacement
What gene can cause dilated cariomyopathy?
SCN5A
What chambers of the heart are effected?
-Can be one but often all chambers functionally impaired
Thrombosis not uncommon
What is the incidence of dilated cardiomyopathy?
5-8 in 100,000
The symptoms for dilated cardiomyopathy?
- Progressive#
- Slow onset
- Dyspnoea
- Fatigue
- Orthopnoea
- PND
- Ankle oedema
- Weight gain
- Cough
What is seen in the PMH of someone with dilated cardiomyopathy?
Systemic illness, travel, HT, vascular disease, thyroid,neuromuscular disease
What can be seen on examination
Poor superficial perfusion thready pulse irrreg in AF SOB at rest Narrow pulse pressure JVP elevated displaced apex s3 and s4 MR mummur often pulmonary oedema pleural effusion ankle and sacral oedema acites hepatomegaly
What is the basic evaluation for cardomyopathy?
- Repeat ECG noting LBBB if present (left bundle branch block)
- CXR
- Nterminal pro brain Natriuetic peptide
- Basic bloods FBC, U+E
- Echo
- CMRI, probably best imaging modality
- Coronary angiogram
- Sometimes biopsy depending on time course of cardiomyopathy?
How do you GENERALLY treat dilated cardiomyopathy?
- Correct anaemia
- Remove exacerbating drugs eg NSAIDS
- Correct any endocrine disturbances
- Advise on fluid and salt intake reduce it
- Advise on managing weight to identify fluid overload
- HF nurse referral
What are the specific measures taken to treat dilated cardiomyopathy?
- ACE, ATII blockers, diuretics
- Beta blockers
- Spironlactone
- Anticoagulants as required
- SCD risk assessment with ICD or CRT-D/P implant
- Cardiac transplant
What is the prognosis of dilated cardiomyopathy?
-Generally poor and often influenced by the cause where know
(worst by HIV)
(best by peripartum)
Describe the pathology of restrictive and infiltrative cardiomyopathy?
- Inability to fill well a ventricle whose wall has reduced compliance
- Relaxation of the ventricular is an active process that needs functioning intact myocytes, it is not passive
Causes gross bilateral atrial dilation
What are the causes of restrictive and infiltrative cardiomyopathy?
50% idiopathic
non infiltrative= familial forms of HCM, scleroderma, diabetic pseudoxanthoma elasticum (fragmentation and mineralisation of elastic fibres)
Infiltrative= Amyloid, sarcoid
Stroage diseases -= Haemachromatosis, Fabry disease
Endomyocardial diease = Fibrosis, carcinoid radiation,drug effects
Specific measures for treating restrictive and infiltrative cardiomyopathy?
-Limited diuretics as low filling pressure will cause problems
-Beta blockers limited ACE1 use
-Anticoagulants as required
-SCD Risk assessment with ICD or CRT-D/P implant
Cardiac transplant
What can be used for basic evaluation of restrictive and infiltrative cardiomyopathy?
-Repeated ECG noting LBBB
CXR
N terminal pro Brain NAtriuetic peptiode
-Basic bloods FBC, U+E
-Auto antibodies for sclerotic CT disease
-Amyloid needs non cardiac biopsy to help establish the diagnosis
-Fabry;Low plasma alpha galactosidases A activity
-Echo
-CMRI, Probably best imaging modality
-Biopsy more helpful but still has false negative rates
Whjat is the prevalence of hypertrophic cardiomyopathy?
1:500
How many genes are said to be responsible for hypertrophic cardiomyopathy?
about 1500
Sarcomemere defect
autosomal dominant but has variable expression and incomplete penetrance
50% but how expressed is not know until time passes
Describe the pathology of hypertrophic cardiomyopathy?
- Myocyte hypertophy and disarray
- Can be generalised or segmental wall thickness> 14mm or > 12mm in primary relative
- Can be apical, septal or generalised
- IMpaired relaxation so behaves in restricted manner
- Septal hypertrophy this interfere with mitral valve defect leads to LVOT (left ventricular outflow tract) obstruction
- coronary arteries also affected
What are the symptoms of hypertrophic cardiomyopathy?
_asymptomatic for many
- fatigue
- Dyspnoea#-Anginal like chest pain
- Exertional pre-syncope, syncope related to arrhythmias or LVOT obstrcution