40 cardiomyopathy Flashcards
What is etiology of dilative cardiomyopathy
Genetic-
-Gene mutations autosomal dominant
- hemochromatosis ( or restrictive)
Duchenne muscular dystrophy
Secondary
Coronary artery disease
Atrial hypertension
Infection Coxackie b virus Rheumatic Chagas! - trypanazoma cruzi HIV
SLE
Cardiac toxic -, anthracyclins such as - doxorubicin,
Malnutrition
Thiamine deficiency - wet beriberi
Selenium deficiency
Radiation
Endocrinopathies- pheochromocytoma, acromegaly
Vascular heart disease
Aortic stenosis
Regurgitation
Patho physiology of DCM
Contractility of myocardium decreases
Frank sterling law activated
Preload increase end diastolic volume increase
Myocardial re modelling - eccentric hypertrophy and dilation of the ventricles
Reduced myocardial contraction and then ejection fraction
Clinical features of dilative cardiomyopathy
RHF
Exceptional Dyspnea
Ankle Edema
Ascitis
Jugular vein distending
Rales
LHF
Dyspnea
In - physical examination for DCM what do you do
Mitral valves or tricuspid regurgitation - systolic murmur
S3 gallop
Rales
What is the diagnostics of DCM
High BNP
Troponin and CK- MB
Echo - dilation
Reduced ejection fraction
Inferolateral hypokinesia
Chest x ray- cardiomegaly
Pulmonary oedema
ECG - AV BLOCK
LBBB
Reduced qrs voltage
Cardiac biopsy- mostly used to exclude other causes ———— Hemochromatosis Serum iron high Ferritin high Total iron binding capacity Low Transferrin saturation high Liver enzyme’s high
Genetic tests
Liver enzymes- Prussian blue
Pronounced hemosiderin
Bronze skin diabetes/ cirrhosis/ hepatomegaly / pituatory gland- hypogonadism/ arthralgia
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Trypanazoma cruzi
Acute phase - peripheral blood smears direct visualisation
Blood test. PCR t cruzi DNA
If intermediate and chronic phase- serological says to detect igG against t cruzi
Treatment or dilative CM
Treatment for heart failure- sodium restriction Ace Beta blockers Diuretics Digoxin
Chagas’ disease- benznidazole
Rheumatic heart disease- penicillin V
SLE - hydroxychoroquine
Addition of methotrexate or azathioprin
Glucocorticoids
If LVEF less than 35% ICD implantable cardioverter defib to prevent sudden cardiac death caused by VFIB
Heart transplantation if medical therapy fails
Complications of DCM
Global heart failure
Systemic thromboembolism
Ventricular tachycardia- VFIB
what are the different types of hypertrophic cardiomyopathy
obstructive hypertrophy - ventricular asymetry septal hypertrophy = blocks the outflow tract through the aorta
Nonobstructive HCM: without obstruction of the left ventricular outflow tract (LVOT)
only ventricular hypertrophy
- asymmetrical septal hypertrophy
- symmetric hypertrophy
- apical hypertrophy
HCM is one of the most frequent causes of?
sudden cardiac death in young patients, especially young athletes.
along side myocarditis
etiology of hypertrophic cardiomyopathy ?
Autosomal dominant with varying penetration mutations of the sarcomeric protein genes - beta myosin heavy chain, myosin binding protein C cardiac troponin t
pathophysiology of hypertrophic cardiomyopathy
non obstructive type end diastolic volume is reduced reduced stroke volume reduced cardiac output HOWEVER EJECTION FRACTION IS PRESERVED ( the blood in th
ventricles are pumped out)
obstructive -
same - reduced EDV
however there is outflow tract obstruction - all the blood in the ventricles is not pumped out
ejection fraction is low
clinical features of hypertrophic cardiomyopathy ?
symptoms worsens with exercise and use of certain drugs such as DIURETICS , HYDRALAZINE , ACEI / ARB, DIGOXIN
the non obstructive type is frequently asymptomatic
Obstructive :
exertional dyspnea / dyspnea on rest
tachycardia , palpitation
syncope , dizziness
chest pain
Hypotension
sudden cardiac death particularly in intense physical activity
what re the physical findings in hypertrophic cardiomyopathy ?
reversed split - expiration p2 then A2
late onset systolic ejection murmur - crescendo decrescendo in the obstructive type -at the apex of left sternal border
DOES NOT RADIATE
=apex or left sternal border
enhanced by valsalva
SAM(systolic anterior motion) - there is mitral valve regurgitation
S4 GALLOP
precordial lifting
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Pulsus bisferiens: LV outflow obstruction causes a sudden quick rise of the pulse followed by a slower longer rise
carotid artery spike and dome pulse
What is the diagnostics criteria for hypertrophic cardiomyopathy
LV hypertrophic more than 12mm? (15 or more in adults).
2) absence of other cardiac or systemic diseases that would explain the hypertrophy - such as hypertension or aortic stenosis
what are the modes of diagnosis in hypertrophic cardiomyopathy
TTA with doppler
- thickening of ventricular wall and septum
if LV wall thickness is or more than 30mm = high risk of sudden cardiac death
Systolic function normal
Diastolic function dysfunction
Left atrial enlargement
systolic anterior motion of the mitral valve
contact between the septum and mittal valve during systole
mitral r egurgitation
increase aortic pressure gradient
findings more specific to obstructive
- asymmetrical thickening of the inter ventricular septum
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ECG typical findings :
LVH sign - sokolow lyon index
asymmetrical septal hypertrophy deep Q wave in - inferior leads 2,3,avf and
lateral - 1 , avl and v4-v6
giant inverted t waves in PRECORDIAL LEADS
P mitrale
LBBB
associated arrhythmia - vtach , afib , atrial flutter
MOST HAVE ABNORMAL ECG
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Cardiac MRI
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genetic testing +cardiac biopsy
