Cardiomyopathy Flashcards
(54 cards)
What is hypertrophic obstructive cardiomyopathy (HOCM)?
An autosomal dominant disorder of muscle tissue caused by defects in the genes encoding contractile proteins.
What is the estimated prevalence of HOCM?
1 in 500.
What is the most common cause of sudden cardiac death in the young?
Hypertrophic obstructive cardiomyopathy (HOCM).
What are the most common genetic defects associated with HOCM?
Mutations in the gene encoding β-myosin heavy chain protein or myosin-binding protein C.
What type of dysfunction is primarily seen in HOCM?
Predominantly diastolic dysfunction.
What are the characteristic findings on biopsy for HOCM?
Myofibrillar hypertrophy with chaotic myocyte disarray and fibrosis.
List common symptoms of HOCM.
- Exertional dyspnoea
- Angina
- Syncope
- Sudden death
- Arrhythmias
- Heart failure.
What causes syncope in HOCM?
Subaortic hypertrophy of the ventricular septum, resulting in functional aortic stenosis.
What are the common pulse and heart sounds associated with HOCM?
- Jerky pulse
- Large ‘a’ waves
- Double apex beat.
What type of murmurs are associated with HOCM?
- Ejection systolic murmur
- Pansystolic murmur.
What is the mnemonic for echo findings in HOCM?
MR SAM ASH.
What does MR stand for in the echo findings mnemonic for HOCM?
Mitral regurgitation.
What does SAM stand for in the echo findings mnemonic for HOCM?
Systolic anterior motion of the anterior mitral valve leaflet.
What does ASH stand for in the echo findings mnemonic for HOCM?
Asymmetric hypertrophy.
What ECG findings are commonly seen in HOCM?
- Left ventricular hypertrophy
- Non-specific ST segment and T-wave abnormalities
- Deep Q waves.
What are the management options for HOCM?
- Amiodarone
- Beta-blockers or verapamil
- Cardioverter defibrillator
- Dual chamber pacemaker
- Endocarditis prophylaxis.
List drugs to avoid in HOCM.
- Nitrates
- ACE-inhibitors
- Inotropes.
What is arrhythmogenic right ventricular dysplasia (ARVD)?
A condition where right ventricular myocardium is replaced by fatty and fibrofatty tissue.
What percentage of ARVD patients have a mutation in desmosome components?
Around 50%.
What is a common ECG finding in ARVD?
T wave inversion in leads V1-3.
What is the epsilon wave in ARVD?
A terminal notch in the QRS complex found in about 50% of patients.
What are the common presentations of ARVC?
- Palpitations
- Syncope
- Sudden cardiac death.
What is the classic triad of Naxos disease?
- ARVC
- Palmoplantar keratosis
- Woolly hair.
What are the classic causes of dilated cardiomyopathy?
- Alcohol
- Coxsackie B virus
- Wet beri beri
- Doxorubicin.
