Cardiomyopathy Flashcards
What is cardiomyopathy classified into
Hypertrophic
Dilated
Restrictive
A lot of overlap so new way to classify
What are new classification
Genetic
Secondary cause
Acquired
Mixed
What are genetic causes
Autosomal dominant
Mutation in B-myosin / protein C
What do genetic mutation cause
HCM
Arrythmogenic R ventricular cardiomyopathy - replaced fatty tissue
What does HCM cause
Leading cause of cardiac death in the young
How do you Dx
Genetic test
ECG - arrythmia
ECHO - MR / hypertrophy
Cardiac MRI
What is the treatment
Defibrillator
What are secondary causes
Drugs
- Alcohol / thiamine deficiency
- Doxorubin (chemo)
Endocrine
- DM
- Thyrotoxicosis
- Acromegaly
Infection
- Coxsackie
Infiltrative
- Amyloidosis
- Haemochromatosis
- Sarcoidosis
- SLE
Other
- Myotonic dystrophy
- Post RT
- Endocarditis
What are acquired causes
Post partum
Tokotsubo
Who is at greater risk of post partum
Older women
Greater parity
Multiple
What is tokotsubo
Stress induced transient ballooning of myocardium
Follows severe stress such as family death / break up
How does tokotsubo present
ACS Chest pain HF features ST elevation Normal angiogram Rx = supportive
What is HCM
Inappropriate ventricular hypertrophy
Causes impaired relaxation
Systolic usually preserved
Eventually reduced LVOT and reduced CO
What is associated with HCM
WPW
Freidreich’s ataxia
What are the symptoms of HCM
Asymptomatic Sudden cardiac death Chest pain Angina SOB Palpitations Syncope Fatigue Cardiac failure
What causes syncope
Arrhythmia or LVOT / AS
What are signs of HCM
Jerky pulse AF Double impulse over apex Ejection systolic murmur increases valsalva Thrill Raised JVP
What are RF for HCM
FH sudden cardiac death
Maternal DM
How do you Dx HCM
ECG ECHO MRI Angiogram Genetic test
What are ECHO findings
MR SAM ASH
Mitral regurgitation as mitral valve doesn’t close properly
Systolic anterior motion of MV
Asymmetric hypertrophy
What are ECG findings
LVH ST and T wave abnormality Q waves AF WPW VT Ectopics
VT = cause of death
How do you treat HCM
Avoid heavy exercise
Anti-coagulation if AF
Surgical resection / ablation
ABCDE
What is ABCDE in HCM
Anti-arrhythmia - amiadarone + anti-coagulant if AF BB / verapamil for Sx Cardiac defibrillator - ICD Dual chamber pacemaker Endocarditis - watch
What should you avoid
Digoxin
INotrope
Nitrates
As increase contractility of heart
What are complications of HCM
Sudden death LVOT Arrhythmia HF AF MR
What is most common cardiomyopathy
Dilated
What is dilated
Enlarged heart
Often all chambers - LV>RV
Ventricular function impaired, systolic dysfunction resulting in reduced CO and increased EDV
Leads to HF
What causes dilated
Mixed Genetics Idiopathic Infection - Coxsackie / HIV / viral Toxins - alcohol / Doxcirubin (chemo) / cocaine High BP Inflammation - SLE Infiltration - amyloid / sarcoid / hamochromatosis Post partum Endocrine - Hyperthyroid
What gene
DCN SCN5A
DMD
How does dilated cardiomyopathy present
Classic HF signs Progressive SOB Fatigue Pulmonary oedema Cough Palpitations Arrhythmia - AF / VT RVF Emboli Mitral and tricuspid regurgitation - pan systolic
What are the signs of dilated cardiomyopathy
Systolic murmur Tachycardia Hypotension Poor perfusion Thread pulse Elevated JVP Displaced apex S3+S4 Pleural effusion Ascites Hepatomegaly as RHF develops
What is important in the history
Travel Systemic illness High BP / vascular Thyroid Neuromuscular
What investigations do you do
Bloods ECG CXR ECHO Angiogram BNP Biopsy
What does ECG show
LBBB
Tachy
What does ECHO show
Low ejection
Global dilatation
What does CXR show
Cardiomegaly
Oedema
How do you treat
Treat cause if known Remove exacerbating drugs e.g. NSAIDs Reduce fluid and salt intake ACEI, diuretic, BB, spironolactone Anti-coagulate if indicated Treat HF + arrhythia VTE prophylaxis SCD assessment
What are the complications of dilated
VTE
Heart failure
Renal failure
Sudden cardiac death
What is restrictive cardiomyopathy
Relaxation affected
Unable to fill ventricle
Systolic may or not be impaired
What causes restrictive
Form of HCM Haemochrmatosis Amyloid - biopsy Sarcoid - ACE Scleroderma - Ab Fibrosis Radiation Drugs Malignancy DM Storage disease - low plasma galactodise
What are the symptoms of restrictive
Present like constrictive pericarditis Back pressure leads to RVF Oedema Increased JVP with X and Y descent Hepatomegaly Ascites
How do you treat restrictive
Treat cause BB Anti-coagulant Transplant Specific treatment for iron / storage SCD assessment
What has limited use in restrictive
ACEI/ diuretic
Low filling pressure so worsen