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
Exam findings for hypertrophic cardiomyopathy?
- Can be none
- Notched pulse patterns
- Irreg pulse if in AF or ectopy
- Double impulse over apex , thrills and mumurs, often dynamic, lVOT mumur will increase with valsalva and decrease with squatting
- JVP can be raised in very restrictive filling
How do you assess hypertrophic cardiomyopathy?
- ECG, Often abnormal but a few are normal where phenotype is poorly expressed in genotype +ve individuals
- Echo
- CMRI
- Risk stratification for SCD may need ICD (implantable cardioverter defibrillator)
What are the general measures to be taken when someone has hypertrophic cardiomyopathy?
- avoid heavy exercise
- Avoid dehydration
- Explore first degree family members
- consider genetic testing
What are the specific measures required for hypertrophic cardiomyopathy?
-Drugs to try to enhance relaxation
-If AF anticoagulants
-Obstructive form? - Surgical or alcohol septal ablation
-ICD if required(Image result for icd medical abbreviation
An implantable cardioverter-defibrillator )
What is myocarditis?
Acute or chronic inflammation of the myocardium
-associated with pericarditis
What can myocarditis generate and impair?
impair? |Function, conduction
generate? arrhythmia
What can myocarditis eventually take on the appearance of?
dilated cardiomyopathy
What is the prevalence of myocarditis?
8-10 per 100000
Describe the pathology of myocarditis?
Infiltration of inflammatory cells into the myocardial layers, reduced function and heart failure,
What are the symptoms of myocarditis?
-Heart failure with fatigue , SOB , CP in 26% (chest pain)
-
How do you assess myocarditis?
-ECG usually abnormal
-Biomarkers ofter elevated but not falling in a pattern consistent
with MI
-Echo, can get RWMA (regional wall motion abnormality)
-CMRI can see Oedmea
-Low threshold for biopsy
-Viral threshold for biopsy
-Viral DNA PCR
-Auto antibodies
-Step Antiobodies
-Lyme B Burgdorferi
-HIV
What are the general measures for myocarditis?
- Support with treatment of heart failure and support for brady and tachy arrythmias
- Immunotherapy if biopsy or other LX point to a specific diagnosis
- Stop toxic agent or drug exposure
What is the prognosis for myocarditis?
-30% recovery fully but 20% mortality at 1 year and 56% by 4 years
At 11 years those still alive are 93% transplant free
What is the pericardium?
Reflected linning over the epicardium (the visceral pericardium) and the parietal pericardium that is the inner portion of the exterior sac around the heart and proximal great vessels
What pericarditis?
- Inflammation of the pericardial layers with or without myocardial involvement
- Substantial number of causes
What are the majority of cases of pericarditis caused by?
- Viral
- idiopathic
- Bacterial
- Post MI
- Perforation
- Dissection of proximal aorta
- Neoplasia
What are the symptoms of pericarditis?
Chest pain with pleuritic features and postural features, sitting forward usually improves
-Fever
What are the signs for pericardial disease?
Temp up pericardial rub JVP raised low BP muffled heart sounds High fever and very unwell despite no effusion may suggest bacterial
What are the investigations to prove pericardial disease?
ECG and echo, Troponin may be raised if myocardial involvement too
What can be seen on ECG with pericardial disease?
widespread ST changes and PR depression of pericarditis
What general measures should be taken with pericardial disease?
-Viral is conservative
-Idiopathic gets colchicine and limited use of NSAIDs
Bacterial must be drained even if small effusion and antimicrobials, high death rate
-If large effusion present and haemodynamics then drain that mofo
What is pericardial effusion?
-May be haemodynamically significant =tamponade or not
causes pericarditis
What are the symptoms of pericardial disease?
fatigue, SOB, Dizzy with low BP, occasionally chest pain
What are the signs of pericardial effusion?
- Pulsus paradoxus
- JVP raised
- low BP
- RUb/ muffled HS
- Pulmonary Oedema is very rare in pericardial effusions/ tamponade
How do you image pericardial effusion?
urgent echo CWR show large cardiac shadow
How do you treat pericardial effusion?
Drain
-persistent effusion needs a surgical pericardial window made to allow flow to abdomen
What show in ECG in a large effusion?
-electrical alternans
QRS complex big then small
What causes constrictive pericarditis..
Idiopathic radiation post surgery autoimmune renal failure sarcoid
What is constrictive pericarditis?
impaired filling although most of the time myocardium is fine
Symptoms of constrictive pericarditis?
fatigue SOB cough
Signs of constrictive pericarditis?
signs of more right heart failure with oedema, ascites, high JVP jaundice, hepatomegaly, AF, TR, pleural effusion, pericardial knock
How do you assess constrictive pericarditis?
echo and right heart cath to differentiate from restrictive cardiomyopathy which can be very difficult
What is the treatment for constrictive pericarditis?
diuretics
pericardectomy
