Cardiomyopathy Flashcards
What is cardiomyopathy?
Primary disease of the myocardium.
What are the 4 different types of cardiomyopathy?
Dilated
Hypertrophic
Restrictive
Arrhythmogenic Right Ventricular (ARVC)
Describe the aetiology of cardiomyopathy
Majority are IDIOPATHIC
List 4 features of dilated cardiomyopathy
Sx of HF: fatigue, dyspnoea
Arrhythmias
Thromboembolism
FH of sudden death
List 10 causes of dilated cardiomyopathy
Post-viral myocarditis Alcohol Drugs (e.g. doxorubicin, cocaine) Familial Thyrotoxicosis Haemochromatosis Peripartum or postpartum HTN AI Congenital (x linked)
List 6 causes of restrictive cardiomyopathy
Amyloidosis Sarcoidosis Haemochromatosis Scleroderma Loffler’s eosinophilic endocarditis Endomyocardial fibrosis
List 5 features of restrictive cardiomyopathy
Dyspnoea Fatigue Arrhythmias Ankle or abdominal swelling FH of sudden death
What are the signs and symptoms of restrictive cardiomyopathy similar to?
Constrictive pericarditis
List 12 signs of dilated cardiomyopathy
Raised JVP Displaced apex beat Functional mitral + tricuspid regurgitations 3rd heart sound Tachycardia AF Hypotension Pleural effusion Oedema Jaundice Hepatomegaly Ascites
List 7 signs of restrictive cardiomyopathy
Raised JVP Kussmaul Sign: paradoxical rise in JVP on inspiration due to restricted filling of the ventricles Palpable apex beat 3rd heart sound Ascites Ankle oedema Hepatomegaly
What 3 common ECG features are seen in all types of cardiomyopathy?
Non-specific ST changes
Conduction defects
Arrhythmias
What may be seen on CXR in cardiomyopathy?
Cardiomegaly
Signs of heart failure: pulmonary oedema
What is seen in the ECG of restrictive cardiomyopathy?
Low voltage complexes
Which investigation is diagnostic and can differentiate between types of cardiomyopathy?
Echocardiography
What is seen on echocardiography in dilated cardiomyopathy?
Dilated ventricles with global hypokinesia + low ejection fraction
MR, TR, LV thrombus
What is seen on echocardiography in hypertrophic cardiomyopathy?
Ventricular hypertrophy (asymmetrical septal hypertrophy)
What is seen on echocardiography in restrictive cardiomyopathy?
Non-dilated non-hypertrophied ventricles
Atrial enlargement
Preserved systolic function
Diastolic dysfunction
Granular/ sparkling appearance of myocardium in amyloidosis
List 3 other investigations that may be performed in patients with cardiomyopathy
Cardiac Catheterisation: help exclude coronary disease
Endomyocardial Biopsy
Pedigree or Genetic Analysis
What is dilated cardiomyopathy?
Dilated, flabby heart of unknown cause.
Thickening of inner layer of heart chambers, heart muscles stretch + weaken
Impairment of contractility (systolic function)
What is restrictive cardiomyopathy?
Cardiac cells replaced with abnormal tissue e.g. scar tissue, causing muscle walls of ventricles to become stiff so relaxation (filling) phase is abnormal.
Impairment of compliance (diastolic function)
Describe the pathophysiology of arrhythmogenic cardiomyopathy
Autosomal dominant with variable expression
RV myocardium replaced with fatty + fibrofatty tissue
Give 3 presentations of arrhythmogenic cardiomyopathy
Palpitations
Syncope
Sudden cardiac death
What ECG abnormalities can be seen in arrhythmogenic RV cardiomyopathy ?
T wave inversion in V1-3
Epsilon wave in ~50%= terminal notch in QRS complex
What is seen on echo in arrythmogenic RV cardiomyopathy?
Often subtle in early stages
Enlarged, hypokinetic right ventricle with a thin free wall
What can be seen on MRI in arrhthmogenic RV cardiomyopathy?
Fibrofatty tissue
What is the management of arrhythmogenic RV cardiomyopathy?
Sotalol
Catheter ablation to prevent VT
Implantable cardioverter-defibrillator
What is Naxos disease? What triad of features characterises this?
Autosomal recessive variant of Arrhythmogenic RV cardiomyopathy
ARVC
Palmoplantar keratosis
Woolly hair
What is hypertrophic cardiomyopathy?
Autosomal dominant disorder of muscle tissue caused by defects in genes encoding contractile proteins
How may HOCM present?
Asymptomatic
Exertional dyspnoea
Angina
Syncope
Sudden death
Describe the syncope experienced in HOCM
Typically following exercise
Due to sub aortic hypertrophy of the ventricular septum, resulting in functional aortic stenosis
What is sudden death in HOCM usually due to?
Ventricular arrhythmias
List 5 signs of hypertrophic cardiomyopathy on examination
Jerky carotid pulse
Double apex beat
Systolic murmurs
Systolic thrill at LLSE
Large ‘a’ waves
Which murmurs may be heard in HOCM?
ESM: due to LV outlflow tract obstruction. Increases with Valsalva manoeuvre, decreases on squatting
Pansystolic: due to systolic anterior motion of the mitral valve - mitral regurgitation
Give 3 features of ECG in hypertrophic cardiomyopathy
Ventricular hypertrophy
Non-specific ST segment + T wave abnormalities, progressive T inversions
Deep Q waves in leads II + III
HOCM is associated with which 2 conditions?
Friedreich’s ataxia
Wolff-Parkinson White
What are the findings on echo in HOCM?
MR SAM ASH
Mitral regurgitation (MR)
Systolic anterior motion (SAM) of the anterior mitral valve leaflet
Asymmetric hypertrophy (ASH)
What is used in the management of HOCM?
ABCDE
Amiodarone
B-blockers or Verapamil for Sx
Cardioverter defibrillator
Dual chamber pacemaker
(Endocarditis prophylaxis)
List 3 drugs that must be avoided in HOCM
Nitrates
ACEi: reduce afterload which may worsen LV outflow tract gradient
Inotropes
