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