31. Cardiomyopathy, Myocarditis and Pericarditis Flashcards
Define cardiomyopathy.
- Group of diseases affecting the heart muscle
- heart muscle becomes enlarged, thick and rigid
What are 3 types of cardiomyopathy?
- dilated cardiomyopathy
- hypertrophic cardiomyopathy
- restrictive cardiomyopathy
What is dilated cardiomyopathy?
- characterised by ventricular dilation and ventricular function is impaired
- symptoms and signs of ventricular failure
- term used to describe dilated and poorly functional left ventricle where aetiology is uncertain/ idiopathic (which excludes ventricular dysfunction secondary to ischaemic or valvular heart disease or hypertension)
Is dilated cardiomyopathy a primary problem?
Can be; or is the end result of any pathological condition of the myocardium
What is the aetiology of dilated cardiomyopathy? (6)
- genetic and familial, muscular dystrophy
- Inflammatory: infectious, autoimmune, postpartum, tropical disease
- Toxic: drugs, exogenous chemical, endocrine, alcohol
- Injury: cell loss, scar replacement
- haemaochromatosis (too much iron absorbed)
- sarcoid (red and swollen granulomas appear on skin)
What gene is responsible for genetic/familial dilated cardiomyopathy?
SCN5A gene; can be linked to autosomal dominant, recessive, x linked and mitochondrial genes
What chambers are dilated in dilated cardiomyopathy?
Usually l. ventricle but most often all chambers dilated and functionally impaired
Is thrombosis common in dilated cardiomyopathy?
Can be present
Is dilated cardiomyopathy reversible?
- Some cases can be reversible or partly reversible
- most are progressive and irreversible
What is the incidence of dilated cardiomyopathy?
5-8/100,000
What are the symptoms of dilated cardiomyopathy? (7)
- progressive, slow onset dyspnoea
- fatigue, tiredness, lethargy (lack of energy)
- orthopnoea (dyspnoea at rest or when lying down)
- post natal depression
- ankle swelling/ oedema
- weight gain of fluid overload
- cough
What past medical history may indicate higher likeliness of dilated cardiomyopathy? (6)
- systemic illness
- travelling
- hypertension
- vascular disease
- thyroid disease
- neuromuscular disease
What parts of social history should be considered when diagnosing dilated cardiomyopathy? (2)
- alcohol intake
2. occupation
What is found on examination in a patient with dilated cardiomyopathy? (14)
- poor superficial perfusion
- thready pulse
- atrial fibrillation (irregular pulse)
- shortness of breath at rest
- narrow pulse pressure
- JVP elevated +/- TR waves
- displaced apex beat
- S3 and S4 and mitral regurgitation murmur heard often
- pulmonary oedema
- pleural effusions
- ankle oedema
- sacral oedema
- acites (accumulation of fluid in abdominal cavity)
- hepatomegally
What investigations are done for dilated cardiomyopathy? (12)
- repeated ECG
- chest x ray
- N termial pro Brain natriuretic peptide
- Full blood count: U+E (urea and electrolytes)
- echocardiogram
- CMRI (cardiac magnetic resonance imaging)
- coronary angiogram
- sometimes myocardial biopsy depending on time course of cardiomyopathy
- cardiac catherisation (x ray of heart taken)
- exercise testing (O2 consumption by resp. gas analysis)
- L.ventricular angiography
- coronary arteriography (excludes ischaemic heart disease as diagnosis)
What are general treatment measures for dilated cardiomyopathy?
- correct anaemia
- remove exacerbating drugs (e.g. NSAIDs)
- correct any endocrine disturbance
- advise on fluid and salt intake (to reduce it)
- advise on managing weight to identify fluid overload
- heart failure (nurse referral)
What are more specific treatment measures for dilated cardiomyopathy? (8)
- ACEI, ATII blockers, diuretics
- Beta blockers (e.g. metoprolol, bisoprolol, carvedilol)
- Spironolactone (K sparing diuretic, prevents excess salt absorption)
- anticoagulants as required
- SCD (sudden cardiac death) risk assessment with ICD or CRT-D/P implant (cardiac resynchronisation therapy device/pacemaker)
- cardiac transplant (especially for young patients)
- digoxin and amiodarone
- long acting nitrates
What is the treatment and management of dilated cardiomyopathy largely based on?
based on treatment of heart failure
What is the general prognosis for dilated cardiomyopathy?
6
- generally poor
- often influenced by other causes of cardiomyopathy due to;
1. peripartum (weakness of heart muscle in last month of gestation to 5 months after delivery)
2. idiopathic
3. due to ischaemic heart disease
4. due to doxorubicin therapy (chemotherapy drug)
5. due to infiltrative myocardial disease
6. due to HIV infection
What is restrictive and infiltrative cardiomyopathy?
- less common (most uncommon cardiomyopathy)
- physiology of filling and myocyte relaxation capacity (heart isn’t able to relax)
- systolic function may or may not be impaired
- presentation almost identical to constrictive pericarditis except constrictive pericarditis can successfully be treated by surgery whereas management of restrictive cardiomyopathy is largely supportive
- diastolic dysfunction with restricted ventricular relaxation
- systolic function and contraction usually well maintained until late when ventricular dilatation and dysfunction occurs
What is restrictive and infiltrative cardiomyopathy related to?
- 50% related to specific clinical disorder
- rest remains unknown (idiopathic)
What is the aetiology of restrictive and infiltrative cardiomyopathy?
- non-infiltrative disease (non-accumulation)
- infiltrative disease (accumulation)
- storage disease
- endomyocardial diseases
What non-infiltrative disease lead to restrictive cardiomyopathy? (5)
- familial
- forms of HCM (hypertrophic cardiomyopathy)
- scleroderma (hardening connective tissue)
- diabetic
- pseudoxanthoma elasicum
What infiltrative disease lead to restrictive cardiomyopathy? (2)
- amyloid
2. sarcoid
What storage disease can lead to restrictive cardiomyopathy? (2)
- haemachromatosis (excess iron taken up by body)
2. Fabry disease (dysfunctional metabolism pf certain lipids)
What endomyocardial disease can lead to restrictive cardiomyopathy? (4)
- fibrosis
- carcinoid
- radiation
- drug effects
What is the pathology of restrictive cardiomyopathy?
- the inability to fill well a ventricle whose wall has reduced compliance
- relaxation of ventricle wall is an active process that needs functioning intact myocytes since it isn’t passive (myocytes need to coordinate)
What are the clinical signs and symptoms of restrictive cardiomyopathy? (8)
- dyspnoea
- exercise intolerance
- signs of r. ventricle dysfunction
- raised JVP
- peripheral oedema
- hepatomegaly
- ascites (fluid in abdominal/ peritoneal cavity)
- pulmonary oedema seen in late disease stage
What basic investigations need to be done to diagnose restrictive cardiomyopathy? (11)
- repeated ECG (noting LBBB and other conduction defects)
- chest x ray
- N termial pro Brain Natriuretic Peptide
- full blood count: urea + electrolytes (look out for sarcoid and haemachromatosis)
- auto antibodies for sclerotic CT diseases
- amyloid needs non-cardiac biopsy
- fabry: low plasma alpha galactosidase A activity
- echocardiogram
- Cardiac MRI (CMRI)
- myocardial biopsy
- cardiac catherisation
What are the general and specific treatment measures for restrictive cardiomyopathy? (7)
- limited diuretic use (as filling pressures will cause problems)
- Beta blockers
- ACEIs
- anticoagulants
- Sudden cardiac death risk assessment with ICD or CRT/P (cardiac resynchronisation therapy/pacemaker) implant
- cardiac transplant
- treatment for fluid retention and arrhythmias
In which 2 conditions can iron overload occur which can happen in restrictive cardiomyopathy?
- amyloid
- Fabry’s
(specific treatment available but endomyocardial fibrosis has little treatment available)
What is the prognosis for restrictive cardiomyopathy?
Unless reversible, then poor prognosis
What is hypertrophic cardiomyopathy?
- characterised by unexplained, usually patchy hypertrophy of the l.ventricular and sometime r. ventricular myocardium
- variable degree of fibrosis in myocardium
- myocardial cell disarray and disruption of of myofibrillar components within hypertrophied myocytes
- impaired relaxation and systolic function is usually adequate with some functional abnormality
Can hypertrophic cardiomyopathy be a primary cause?
Yes; can occur without secondary causes of ventricular hypertrophy such as aortic stenosis or systemic hypertension
Which portion of the heart is does hypertrophic cardiomyopathy usually localise in?
In the upper section of the intrerventricular septum and anterior free wall of the l.ventricle
What is the prevalence of hypertrophic cardiomyopathy?
Relatively high at 1:500
How many genes associated with hypertrophic cardiomyopathy?
1500 genes identified