Cardiomyopathy Flashcards
What is cardiomyopathy?
Heart muscle disease
What are the main types of cardiomyopathy?
Hypertrophic
Dilated
Restrictive
Myocarditis
What is dilated cardiomyopathy?
When all four chambers of the heart enlarge
What are the causes of dilated cardiomyopathy?
Primary: Idiopathic Genetic mutation Infection Inflammatory Alcohol Drugs Post partum Tropical disease Haemaochromatosis Sarcoid
Secondary to pathological insult to myocardium
How does dilation occur in DCM?
New sarcormere are added in series and chamber grow larger leaving the walls thin compared to large chamber size - therefore less muscle used for contraction
How does DCM lead to congestive heart failure?
Less contraction -> less SV -> biventricular congestive heart failure as heart cant pump blood out to lungs and body efficiently -> systolic HF
What are the symptoms of DCM?
Progressive dyspnoea Fatigue Orthopnoea Paroxysmal nocturnal dyspnoea (PND) Ankle swelling Weight gain (fluid overload) Cough
What should be looked for in the clinical history for DCM?
PMH:
Illness, HPT, vascular disease, thyroid, neuromuscular disease
SH:
Travel, alcohol, job
What are clinical signs of DCM?
Poor perfusion Thready pulse Elevated JVP Displaced apex S3 and S3 MR murmur Pulmonary, sacral and ankle oedema Pleural effusion Thready pulse Acites Hepatomegally
What are the conditions that DCM commonly presents with?
Heart failure Cardiac arrhythmias Conduction defects Thromboembolism Sudden death
How can DCM cause regurgitation of AV valves?
Dilation of chambers, stretches valve so that they cant close all of the way during systole
MR can produce holosystolic pressure and S3 sound due to blood slamming against ventricle wall during diastole
How can DCM cause arrhythmia?
Stretching of muscle wall can irritate myocytes important for conduction
What investigations should be carried out for DCM?
ECG CXR BNP level FBC U+E Echo CMRI Coronary angiogram Biopsy depending on type of type of cardiomyopathy
What are general measures to treat DCM?
Correct anaemia
Remove exacerbating factors i.e. drugs
Reduce Na intake
What are specific measures to treat DCM?
ACEi
ATII blockers
Diuretics
B blockers
Spionolactone
Anticoagulants as required (thrombus formation common in DCM)
What are surgical measures to treat DCM?
Cardiac transplant
Sudden cardiac death risk with defibrillator implant
What is restrictive cardiac myopathy?
Myocardium become stiffer and less compliant - which means less blood enters the ventricle during diastole as they ventricles do not stretch to allow more blood in
How does RCM cause heart failure?
Stiffer myocardium means ventricles don’t stretch during diastole -> less blood pumped out -> diastolic heart failure
What are the causes of RCM?
Amyloidosis, sarcoid Familial, forms of HCM, diabetic Haemachromatosis Fabry disease Fibrosis, carcinoid, radiation, drug effects
How do amyloid cause RCM?
Misfolding of proteins which make it insoluble and can deposit in tissue
How does sarcoids cause RCM?
Forms granulomas in myocardium
Why does bi-atrial dilation occur in RCM?
Less filling of ventricles means extra blood in atrium
How does haemachromatosis cause RCM?
Too much iron from the diet is absorbed and it builds up in the myocardium
What investigation should be carried out for RCM?
ECG CXR BNP FBC U+Es - sarcoid and haemachormatosis Auto-Antibodies for sclerotic (hardening) connect tissue diseases Cardiac biopsy - amyloid Fabry - low plasma galactosidase A activity Echo CMRI
How does radiation cause RCM?
radiation generate reactive oxygen species in the myocardium which causes inflammation -> myocardial fibrosis
What can an ECG show in RCM?
Smaller QRS complexes due to restricted ventricular contraction
What is fabry disease?
Genetic disease a deficiency of the enzyme alpha-galactosidase A (a-Gal A) that causes a buildup of a type of fat in tissue
What are general measure for RCM?
Limited diuretic use B blockers Anticoagulats SCD risk assessment with IC implant Cardiac transplant Treat iron overload if due to amyloid or sarcoid
What is hypertrophic cardiomyopathy?
When walls are thick, heavy and hypercontractile - new sarcomeres are added in parallel
Usually affect Left ventricle
How does HCM lead to heart failure?
Larger muscles take up more room so less blood fills into the ventricle
Myocardium stiff and less compliant so less blood fills ventricle due to reduced stretch
Less EDV -> decreased SV -> diastolic heart failure
What genetic inheritance is HCM?
Autosomal dominant