WCS8 Cardiology inherited cardiac diseases Flashcards
(30 cards)
types of inherited cardiac diseases
- inherited cardiomyopathies
- inherited disorders of rhythm and conduction
- familial hypercholesterolemia
Are VSD, ASD, WPW inherited cardiac diseases?
no. they are congenital
types of inherited cardiomyopathies (morphological classification)
- hypertrophic
- dilated
- restricted
- arrhythmogenic right ventricular disease
types of inherited disorders of rhythm and conduction
- Long QT syndrome
- Brugada syndrome
- CPVT
- PCD
feature of cardiomyopathies
inappropriate ventricular hypertrophy or dilation
–> mechanical and/or electrical dysfunction
morphological abnormality in hypertrophic cardiomyopathy
inappropriate LVH +/- LVOT obstruction
morphological abnormality in dilated cardiomyopathy
LV dilation
Systolic dysfunction
morphological abnormality in arrhythmogenic RV cardiomyopathy
fibroadipose infiltrate
morphological abnormality in restrictive cardiomyopathy
abnormal LV filling
diastolic dysfunction
Pathophysiology of HOCM
- LVOT obstruction
- diastolic dysfunction
- myocardial ischemia
- MR
Risk factors for sudden death in HCM
High risk - cardiac arrest - sustained vtach moderate to high risk - FH of premature sudden death - extreme LVH - abnormal exercise blood pressure - unexplained syncope - NSVT low risk - mild LVH others - afib - mi - LVOT obstruction - high risk mutation - intense physical exertion
Hallmark of RCM
rigid ventricular walls with ventricular filling dysfunction
–> normal systolic but abnormal diastolic function
secondary causes of hcm
amyloidosis
pheochromocytoma
metabolic disease
secondary causes of rcm
amyloidosis
hemochromatosis
endomyocardial fibrosis/disease
condition with functional resemblance as rcm
constrictive pericarditis
causes for DCM
- viral
- valvular
- toxin
- ischemic
- infiltrative
- idiopathic
complications for DCM
- heart failure
- sudden death
Pathogenesis of DCM
- Genetic factors –> DNA mutation –> altered gene products –> altered myocardial function –> dcm
- genetic factors –> MHC –> alteration of immune system –> t cell dysfunction autoantibodies –> altered myocardial function –> DCM
- viral infection –> myocarditis –> interference with immune response –> tcell dysfunction autoantibodies –> altered myocardial function –> dcm
- viral infection –> myocarditis –> viral persistence –> altered myocardial function –> dcm
LQTS hallmarks
QT prolongation on ECG
propensity to Vtach
causes of LQTS
congenital - ion channelopathies (sporadic, Jervell& Lange-Neilson, Romano-ward)
acquired
- drug - abx, antiarrhythmic, psychotropics
- myocardial (ischemia, MVP, myocarditis),
- metabolic disturbance - DM, hyperparathyroid, hypothyroid, pheo,
- electrolyte abnormalities,(hypoK,Ca, Mg)
- malnutrition,
- CNS - hemorrhage, trauma, cva, encephalitis
Pathophysiology of LQTS
reduced repolarization current/ enhanced depolarization current –> prolonged ventricular repolarization (QT)
How to differentiate LQT from LQTS
- Schwartz score clinical syndrome recognition (fh, symptoms, ecg)
- exercise testing
- genetic identification
Treatment of LQTS
- B-blockers
- lifestyle advices
- left cervicothoracic stellectomy
- icd implantation
- pacemaker implantation
Brugada syndrome hallmarks
- ST elevation (coved/saddle-back type) in right precordial leads +/- apparent RV conduction block
- heart grossly structurally normal
- propensity for life-threatening Vtachs
