Cardiomyopathy, myocarditis and pericarditis Flashcards
What is cardiomyopathy?
group of diseases of the myocardium which affects the mechanic or electrical function of the heart
What kinds of cardiomyopathy affect the mechanic function of the heart?
Hypertrophic Arrhythmogenic Right ventricular Dilated Restrictive
What kinds of cardiomyopathy affect the electrical function of the heart?
Conduction system disease
Ion channelopathies e.g. long QT
Describe dilated cardiomyopathy
Dilated left ventricle (poorly contracts)
All chambers can be dilated though
Can result from myocarditis
What can cause dilated cardiomyopathy?
Genetic and familial DCM: SCN5A, MD
Inflammatory/infectious
Toxic - alcohol
Injury/cell loss/scarring - sarcoidosis
What gene is responsible for familial dilated cardiomyopathy?
SCN5A - autosomal dominant inheritance
Muscular dystrophy can also cause it
What are the symptoms of DCM?
Dyspnoea Fatigue Orthopnoea PND Ankle oedema, weight gain from fluid Cough Embolism from mural thrombi Arrhythmias Signs of biventricular heart failure
What should you make sure to check during a history for suspected DCM?
PMH - systemic illness, travel, vascular disease
FH - familial? MD?
SH- alcohol, job
What would be seen on examination on those with DCM?
Poor superficial perfusion Thready pulse, irregular if in AF SOB at rest Narrow pulse pressure Elevated JVP pressure Displaced apex S3 and S4 Pulmonary oedema Pleural effusion Ankle oedema Sacral oedema Ascites Hepatomegaly
What investigations would be carried out on those with suspected DCM?
CXR - cardiac enlargement ECG - LBBB Cardiac imaging - CMRI, angiogram BNP ECHO
What general treatments are given to those with DCM?
Correct anaemia - iron tablets Remove exacerbating drugs Correct endocrine disturbance (thyroid) Reduce salt and fluid intake Weight management
What treatments are given to those with DCM?
BP - ACEIs, ARBs, diuretics Beta blockers Spironolactone Anticoagulants ICD if patients have later stage HF Heart transplant if severe
Describe restrictive cardiomyopathy
Rigid myocardium restricts ventricular filling, features similar to constrictive pericarditis. Can see gross bi-atrial dilation in an attempt to counteract this
What is the most common cause of restrictive cardiomyopathy?
Amyloidosis
List some causes of restrictive cardiomyopathy
Non infiltrative - familial, diabetes
Infiltrative - amyloidosis, sarcoid
Storage - haematochromatosis, Fabry dis.
Endomyocardial - fibrosis, carcinoid, toxicity
Describe some investigations for RCM?
ECG - LBBB, other defects CXR BNP Bloods Autoantibodies for sclerotic disease Amyloid - non-cardiac biopsy Fabry - low a-galactosidase A activity ECHO Biopsy
How do you diagnosis RCM?
Cardiac catheterization
Endomyocardial biopsy
What test would confirm Fabry disease being the cause of RCM?
Fabry - low a-galactosidase A activity
What test would confirm amyloidosis being the cause of RCM?
Amyloid - non-cardiac biopsy
How do you treat RCM?
NO DIURETICS Beta blockers and ACEIs Anticoagulants as required Treatments for iron overload ICD Cardiac transplants
Describe hypertrophic cardiomyopathy
Marked ventricular hypertrophy in the absence of abnormal loading conditions such as hypertension and valvular disease. Leads to loss of compliance which impairs diastolic filling, reducing stroke volume
What are most cases of hypertrophic cardiomyopathy caused by?
Autosomal dominant mutations in genes encoding sarcomeric proteins e.g. troponin I and beta myosin
What is the most common cause of sudden cardiac death in young people?
Hypertrophic cardiomyopathy
What can result from septal hypertrophy due to hypertrophic cardiomyopathy and mitral valve defect?
Let ventricular outflow tract obstruction (LVOT)
What are the symptoms of hypertrophic cardiomyopathy?
Fatigue
Dyspnoea
Anginal like chest pain
Exertional pre-syncope or syncope related to arrhythmias or LVOT
What complications are associated with hypertrophic cardiomyopathy?
Sudden death Atrial and ventricular arrhythmias Thromboembolism Infective endocarditis Heart failure
Describe the clinical signs of hypertrophic cardiomyopathy
Jerky carotid pulse Pansysolic murmur from mitral regurgitation Ejection systolic murmur - LVOT Notched pulse pattern Double impulse over apex LVOT murmur increased with valsalva Decreased LVOT when squatting Raised JVP
What assessments will be carried out for hypertrophic cardiomyopathy?
ECG - patterns of LVH
ECHO - LVH
CMRI - VH and fibrosis
Genetic testing for sarcomeric protein mutations
How do you treat hypertrophic cardiomyopathy?
Beta blockers + verapamil - reduce HR Amiodarone - anti-arrhythmic Anticoagulants Surgical resection/ septal ablation ICD
What general advice do you give to those with hypertrophic cardiomyopathy?
Avoid heavy exercise
Avoid dehydration
Encourage family to be tested
Regular follow ups to reappraise progress
Describe myocarditis
Inflammation of myocardium, which can impact on myocardial function, conduction (heart block) and generate arrhythmias
Late stages appears like DCM
List some causes of myocarditis
Infection - Coxsackie virus, diphtheria, RF Radiation Injury Lyme disease? HIV
What is the most common cause of myocarditis?
Coxsackie infection
List the symptoms of myocarditis
Fever Varying degrees of biventricular failure Cardiac arrhythmias and pericarditis Fatigue SOB Heart block?
Describe some investigations for myocarditis
ECG - ST changes ECHO CMRI - cardiac enlargement, oedema Viral DNA PCR Autoantibodies HIV Lyme B burgdorferi
How do you diagnose myocarditis?
Demonstrate increased serum viral titres and inflammation on cardiac biopsy (though these are rarely performed)
How do you treat myocarditis?
Treat HF and arrhythmia
Immunotherapy
Stop possible drug/toxic agent exposure
Bed rest
How much fluid is found in the pericardium normally?
50ml
What is pericarditis?
Inflammation of the pericardial layers with to without myocardial involvement
What causes pericarditis?
Infection - Coxsackie B, echovirus, HIV Tuberculosis Fungal infections Perforation/trauma Dissection of proximal aorta Neoplasia (breast, lung, leukaemia) Ureamia AI rheumatic disease
Describe symptoms of pericarditis
Lasts 1-2 weeks normally Sharp retrosternal chest pain Pleuritic features - pericardial rub Postural changes - sitting forward improves, lying back makes it worse Pain worse on inspiration Pain radiates to neck and shoulders Fever (infection)
What are the clinical signs of pericarditis?
Fever Pericardial rub Raised JVP Low BP Muffled heart sounds + increased JVP suggest effusion No effusion = bacterial infection
What investigations are used for pericarditis?
ECG - ST elevation (saddle shaped)
ECHO
Troponin increased if myocardium involved
How do you treat pericarditis?
Antimicrobials, NSAIDs
Drain large effusions/bacterial infected
Systemic corticosteroids if resistant
Why cant NSAIDs be used for pericarditis if an MI has happened a few days prior?
Increased risk of myocardial rupture
What are some complications of pericarditis?
Pericardial effusion
Chronic pericarditis (>6 months)
Tamponade
What can cause pericardial effusions?
pericarditis
hypothyroidism
What is pericardial tamponade?
Medical emergency, large amount go pericardial fluid restricts diastolic filling and causes a marked reduction in cardiac output
What are some symptoms of pericardial tamponade?
Fatigue
SOB
Dizzy with low BP
Occasionally chest pain
What are some clinical signs of pericardial tamponade?
Pulsus paradoxus Raised JVP Low BP Pericardial rub Muffled HS Pulmonary oedema Tachycardia KUSSMAULS SIGN
What is Kussmaul’s sign?
Elevated JVP which paradoxically increases with inspiration (sign of pericardial tamponade)
Seen in pericarditis
What investigations are used for pericardial tamponade?
CXR - large heart shadow, globular heart
ECG
ECHO - echo free space around heart
How do you treat pericardial tamponade?
Emergency pericardiocentesis
Fluid analysed for neoplastic cells, infection
If recurs, excise pericardial “window”
Describe constrictive pericarditis
Heart becomes encased with fibrotic pericardial sac, preventing adequate diastolic filling of ventricles
What causes constrictive pericarditis?
Intrapericardial haemorrhage in surgery Radiation AI RF Sarcoid disease
What are the clinical signs of constrictive pericarditis?
Jugular venous distension Oedema Hepatomegaly Ascites Kussmauls sign Atrial fibrillation Pericardial knock
What are the symptoms of constrictive pericarditis?
Fatigue SOB Cough Jaundice VERY similar to right HF and restrictive cardiomyopathy
What investigations are used for constrictive pericarditis?
CXR - normal heart, pericardial calcification
CT/MRI - pericardial thickening/calcification
ECHO - diff from restrictive cardiomyopathy
How do you treat constrictive pericarditis?
Careful/limited diuretics
Surgical removal of pericardium
