Cardiomyopathy Flashcards
What are the 3 types of cardiomyopathy?
Restrictive - Stiffening of Myocardium
Hypertrophic - hypertrophy of myocardium
Dilated - Dilation of ventricles
What causes DCM? [4] What is the most common cause
Complication and prognosis?
- Genetics e.g. Muscular Dystrophy
- Doxorubicin/alcohol/chemicals
- Coxsackie B virus
- Idiopathic*
Cx: sudden cardiac death
Pro: 40% mortality in 2y
How does DCM present?
Symptoms [6]
Signs [8]
- Fatigue
- Dyspnoea/Orthopnea/PND
- Peripheral edema
- Cough
- LHF: Weak Pulses, pleural effusion
- RHF: Raised JVP, Ascites, jaundice, hepatomegaly
- Tachycardia, hypotension
- S3 gallop, mitral/tricuspid regurg
- Displaced apex beat
DCM investigations and results
4 first line
2 others
- Bloods: FBC, U&E and creatinine (low Na+ indicates poor prognosis), LFT, BNP confirms HF
- ECG: tachycardia, non-specific T wave changes, poor R wave progression, LVH. LBBB
- Echo: dilated hypokinetic heart with low ejection fraction (may have MR, TR or mural thrombus)
- CXR - Pulm congestion + Cardiomegaly
Cardiac MRI
Endomyocardial biopsy - Visibally stretched fibres
How do we treat DCM? [6]
Bed rest Diuretics, ACEi, BB (reduce strain) Digoxin (increase contractility) Warfarin (reduce thrombus risk) Biventricular pacing, ICD Heart transplant
What causes RCM? [7]
Amyloidosis Sarcoidosis Haemochromatosis Fibrosis - MI, drugs, radiation, idiopathic Genetic mutations causing familial Diabetes Loffler's endocarditis
How does RCM present? [5]
Symptoms are similar to constrictive pericarditis, SOB, fatigue
Right ventricular failure predominates
- Cough, Chest Pain
- Oedema, Ascites, Hepatomegaly
- Raised JVP
- Tachycardia
- Audible S3/4
How do we test for RCM? [6] which one is gold standard
*Right ventricular biopsy: gold standard with +ve Congo red staining, may show sarcoidosis, amyloidosis
Serum Fe (Haemochromatosis)
ECHO - biatrial enlargement and patchy fibrosis from infiltrative disease
MRI (ddx from constrictive pericarditis)
CXR (pulm congestion + normal heart size)
ECG (AF)
Note: normal heart size and normal ejection fraction in ECHO
How do we treat RCM? Treat the cause Rx 3 Devices 1 Definitive treatment 1
Rx
- Warfarin (AF)
- B-blockers - ACEI - Diuretic -> Reduce strain on heart
- Amiodarone (arrhythmia’s)
ICD
Heart Transplant
What is THE cause of HCM? [3]
Pathogenesis [3]
Familial hypertrophic cardiomyopathy
Autosomal dominant
Strong genetic components
Leading cause of sudden cardiac death in the young
Missense mutations [1] of beta-myosin chains [1] which affect sarcomeric proteins [1]
How does HCM present?
Symptoms [7]
Signs [5]
Asymptomatic until the valve is occluded or the heart cant pump enough blood anymore:
- Fatigue
- Chest Pain, angina
- Dyspnoea
- Palpitations
- Tachycardia
- Presyncope
- Exertional Arrythmias
- Notched/bifid Pulse
- Raised JVP
- Audible S4
- Double apex beat
- Systolic ejection murmur worse on valsalva
How do we test for HCM?
6 investigations
ECG ECHO CXR Cardiac catheterisation (MR) Exercise testing with respiratory gas mask (risk stratification) Genetic Testing Biopsy - myocyte dissaray
How do we treat HCM? Rx 4 Lifestyle modification 1 Devices 1 Surgical methods 3
Lifestyle mods: reduce exercise/stress
Rx:
Warfarin + Beta blockers (reduce contractility)
Verapamil
Amiodarone
Devices: Dual chamber pacing (ICD)
Surgical: 1. Septal myomectomy/ablation (reduces outflow gradient) 2. Surgical mitral repair 3. Heart transplant
What should we see on ECG for HCM? [4]
LVH Progressive T wave inversion Deep Q waves (inferior and lateral leads) AF, WPW Ventricular ectopic, VT
What would we see on ECHO for HCM? [4]
- asymmetrical septal hypertrophy
- non-dilated small LV cavity with hyper contractile posterior wall
- mid-systolic closure of aortic valve
- systolic anterior movement of mitral valve
