cardiomyopathies and myocarditis Flashcards
what is the most common cardiomyopathy ?
dilated congestive cardiomyopathy (DCM)
what is the pathology in dilated cardiomyopathy ?
progressive dilatation of all chambers , a big heart with very big chambers
what type of dysfunction is associated with dilated cardiomyopathy ?
systolic dysfunction
what are the causes of dilated cardiomyopathy ?
non genetic
genetic
idiopathic
what are the causes of DCM?
a bunch of stuff can cause cardiac dilatation
Alcohol abuse
Beriberi
Coxsackie
Cocaine
Chagas disease
Doxorubicin
peripartum cardiomyopathy
takutsobo cardiomyopathy
tachycardia cardiomyopathy
what are the genetic causes of DCM ?
Duchenne and Becker muscular atrophy
what are the treatments of DCM ?
conventional management of heart failure with thee option of cardiac resynchronization therapy and ICDs for patients with a score 3 in thee NYHA
cardiac transplantation
what is the pathology in hypertrophic cardiomyopathy ?
-massive myocardial hypertrophy without ventricular dilatation ( asymmetrical hypertrophy of the interventricular septum )
what is the echo finidings in hypertrophic cardiomyopathy ?
banana like ventricular cavity
what type of dysfunction is associated with HCM?
diastolic dysfunction
what is the gross picture of HCM ?
asymmetrical septal hypertrophy
asymmetrical left ventricular hypertrophy
what are the causes of HCM ?
distinct genetic disorder, where there is a defect in one of the genes encoding cardiac contractile proteins
is also a storage disease found in babies to diabetic mothers
what are the genes affected in HCM?
heavy chain myosin
what are the effects of HCM
commonest cause of sudden cardiac death in young during vigorous exercise
syncopal attacks on exercise
what is the treatment for HCM?
according to risk stratification
ICD insertion for patients with high risk of sudden death
risk is lower ? amiodarone
what is the most common cause of sudden death in adolescent during vigorous exercise ?
hypertrophic cardiomyopathy
what is the pathology in restrictive cardiomyopathy ?
infiltration of the myocardium resulting in a firm myocardium and eventually failure of relaxation
what are the causes of restrictive cardiomyopathy ?
RASH
radiation fibrosis
amyloidosis
sarcoidosis
hemochromatosis
what is amyloidosis ?
extracellular deposition of amyloid substance mainly of walls of blood vessels
what stain can be used with amyloid ?
congo red stain
what is seen on clinical examination with restrictive cardiomyopathy ?
1- elevated JVP with diastolic collapse - friedreich’s sign
2- elevation of venous pressure with inspiration (Kussmmaul’s sign )
right ventricular hypertrophy
what is the treatment of restrictive cardiomyopathy ?
medical
prevention of progression
cardiac transplantation
what is myocarditis ?
primary inflammation of the myocardium
what are the causes of myocarditis ?
may be :
infectious causes
non-infectious causes
unknown
what are the infectious causes of myocarditis ?
cardioselective viruses (adenovirus) non-selective viruses non-viral causes
what are the non-infectious causes of myocarditis ?
immune reaction
post-viral
rheumatic heart disease
SLE
what are the effects of myocarditis ?
-wide spectrum from totally asymptomatic to acute heart failure to sudden cardiac death via arrhythmia
what is the treatment of myocarditis ?
- supportive therapy
- appropriate antimicrobials
how to differentiate between cardiomyopathies and other cardiac problems ?
myocardial disease due to intrinsic defect if it is due to HTN, coronary artery disease or valvular heart disease or inflammation its not cardiomyopathy
what are the signs in HCM ?
double apical pulsation
jerky carotid pulse
ejection systolic murmur
pansystolic murmur
what are the signs seen in HCM ?
jerky carotid pulse
double apical pulsation
what are the manoeuvres that decrease afterload ?
standing
valsalva
what manoeuvres increase afterload ?
squatting
what type of murmur is associated with left ventricular outflow obstruction ?
ejection systolic murmur
what are the common causes of LV outflow obstruction ?
aortic stenosis
HCM
what happens as a result of the systolic anterior motion which happens in HCM ?
mitral regurg - pansystolic murmur
s4 cannot coexist with what heart condition ?
atrial fibrillation
when is s4 heard ?
with LV hypertrophy
what investigation can be used to differentiate between HCM and infiltrative cardiomyopathies ?
CMR
in patients with HCM who have significant left ventricular outflow obstruction , what is the management ?
dual-chamber pacing
plus disopyramide
what class of drugs should be avoided in HCM ?
vasodilators
what waves are specifically seen in arrhythmogenic right ventricular cardiomyopathy ?
epsilon waves
what is the first line treatment for patients with non life threatening arrhythmias ?
beta blockers
management for refractory or life threatening arrhythmia ?
ICD
diagnostic testing for arrhythmogenic right ventricular cardiomyopathy ?
genetic testing
what is the presentation of takotsubo CM ?
middle aged woman
history of stressful event
apical ballooning
what are the features of peripartum cardiomyopathy ?
affects women in the llast trimesster
or within 5 mmonths of preegnancy
what condition is peripartum cardiomyopathy associated with ?
preeclampsia
what is the prognosis of peripartum CMM ?
half of the patients will recover to normal function within 6 months
other half may develop heart failure and sudden death
what is the cause of tachycardia cardiomyopathy ?
prolonged periods of supraventricular or ventricular tachycardia
what is the management for tachycardia cardiomyopathy ?
cardioversion and ablation
what is characteristic for cardiac amyloidosis ?
voltage mass-mismatch
ground glass appearance of the myocardium
macroglossia
bruising around the eye
what is the novel therapy for patients with familial amyloidosis ?
tafamidis
liver transplantation
what is the appearance of left ventricular noncompaction ?
congenital cardiomyopathy
sponge like appearance of the left ventricle and excessive trabeculations
what is the next diagnostic step for patients with amyloidosis ?
TTE
