Cardiomyopathy Flashcards by Conor Hughes

What are the different types of cardiomyopathy?

Hypertrophic,
dilated,
restictive
myocarditis

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Name the different types of pericardial disease?

-Pericarditis and effusion +/- tamponade

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What is dilated cardiomyopathy?

Structural and functional discription– the ventricular function is impaired

Can be primary or secondary

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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

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What gene can cause dilated cariomyopathy?

SCN5A

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What chambers of the heart are effected?

-Can be one but often all chambers functionally impaired

Thrombosis not uncommon

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What is the incidence of dilated cardiomyopathy?

5-8 in 100,000

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The symptoms for dilated cardiomyopathy?

Progressive#
Slow onset
Dyspnoea
Fatigue
Orthopnoea
PND
Ankle oedema
Weight gain
Cough

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What is seen in the PMH of someone with dilated cardiomyopathy?

Systemic illness, travel, HT, vascular disease, thyroid,neuromuscular disease

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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

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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?

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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

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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

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What is the prognosis of dilated cardiomyopathy?

-Generally poor and often influenced by the cause where know

(worst by HIV)
(best by peripartum)

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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

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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

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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

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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

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Whjat is the prevalence of hypertrophic cardiomyopathy?

1:500

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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

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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

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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

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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