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Cardiomyopathies (Dilated (Aetiology (Vascular HTN, Congenital AD or X…
Cardiomyopathies
Dilated
Pathophysiology
Dilated, flabby LV
LV cannot contract properly, reduced CO and HF,
can also lead to arrhythmias
Aetiology
Vascular
HTN
Congenital
AD or X-linked dilated CM
Haemachromatosis
Infection
Post-viral
Endocrine
Hyperthyroidism
Peri-post-partum
Epidemiology
RARE
Clinical presentation
SOB
Fatigue
Palpitations
Diagnosis
Examination
Tachycardia, hypotension, raised JVP,
Displaced apex (cardiomegaly)
Murmur (TR/MR)
Bibasal crackles (oedema)/reduced air entry (effusion)
Hepatomegaly
Investigations
Bedside
Obs (tachycardic, hypotensive)
ECG (tachycardia, poor R wave progression, T wave changes)
Bloods
FBC, U+E, LFTs, BNP (raised)
Genetic testing
Imaging
CXR: cardiomegaly, pulmonary oedema
ECHO: diagnostic; dilated ventricles, poor EF, valve disorder
History
PC/HPC: SOB, fatigue, reduced exercise tolerance, palpitations
PMH: known conditions, HTN, thyroid disease
DH: current meds, allergies
FH: cardiomyopathy, other heart disease
SH: living arrangements, occupation, school/nursery
Management
Medical
Diuretics
Indication: HF
E.g. furosemide
MOA: reduces fluid overload
ACEi
Indication: HF
E.g. ramipril
B-blocker
Indication: HF
E.g. carvediolol
Surgical
Biventricular pacing
Indication: severe disease
MOA: synergises contractions between the
LV and the RV
ICD
Indication: severe disease
MOA: defib in place as risk of sudden death
Heart replacement
Indication: severe disease
Conservative
Information, advice, support
Lifestyle
Prognosis
Variable
40% mortality in 2y
Hypertrophic
Clinical presentation
Asymptomatic
Chest pain
SOB
Syncope
Palpitations
Sudden death
Diagnosis
History
PC/HPC: chest pain, SOB, syncope
PMH: known conditions
DH: meds, allergies
FH: cardiomyopathy
SH: occupation, school, lifestyle
Examination
Jerky pulse, double apex beat,
thrill, murmur (ejection systolic)
Investigations
Bedside: obs, ECG (LVH, T wave inversion, arrhythmias)
Bloods: FBC, U+E, LFT, BNP, genetic testing
Imaging: CXR (cardiomegaly), ECHO (diagnostic)
Special tests: electrophysiology, exercise ECG
Pathophysiology
AD inherited condition or sporadic mutation
Genetics affect cardiac contractile proteins
Asymmetrical ventricle hypertrophy, thick non-compliant ventricles
Poor contraction, low CO and HF
Eventually leads to LV outflow obstruction
Management
Conservative
Information, advice, support
Lifestyle
Medical
B-blockers
Indication: chest pain/palpitations
E.g. propanolol
MOA: reduce HR
CCBs
Indication: chest pain, palpitations
E.g. cardio-specific e.g. verapamil
MOA: slow HR
Amiodarone
Indication: arrhythmia e.g, AF, VT
MOA: reduce risk sudden cardiac death
Anticoagulation
Indication: AF prophylaxis
Surgical
Septal myomectomy
Indication: severe disease
MOA: chemical or physical reformation
of outflow tract, improving SV/CO
ICD
Indication: high risk VT/sudden death
MOA: detects shockable rhythms and
electrical shock given
Epidemiology
RARE
Leading cause of sudden cardiac death
Can present at any age
Complications
HF
Arrhythmias
VTE
IE
Sudden cardiac death
Prognosis
6% mortality/y if <14y 3%/y if >14y
Restrictive
Aetiology
Metabolic
Haemachromatosis
Autoimmune
Amyloidosis
Sarcoidosis
Scleroderma
Eosinophilic endocarditis
Diagnosis
Examination
Cardio: raised JVP, Kussmaul's sign, diffuse apex beat, bibasal crackles (oedema), peripheral oedema
Abdo: hepaomegaly, splenomegaly, ascites
Investigations
Bedside
Obs: nil
ECG: RVH
Bloods
FBC, U+E, LFT, BNP
Imaging
CXR: cardiomegaly
ECHO: structural change
cardiac catheterisation: diagnostic (pressure changes, biopsy)
History
PC/HPC: chest pain, SOB, fatigue
PMH: known medical conditions (autoimmune)
DH: meds, allergies
FH: cardiac disorders
SH: occupation, lifestyle
Pathophysiology
Infiltration and sclerosis of myocardium
Stiff ventricle walls, unable to relax effectively
Reduced diastolic filling and contraction
Mostly affects RV and right heart
Clinical presentation
Chest pain
SOB
Fatigue
Epidemiology
RARE
Management
Conservative
Information, advice, support
Surgical
Transplant
Indication: severe disease
Prognosis
Very poor, can die <1y diagnosis
Definition
Group of disorders affecting
the heart muscle (myocardium)