Cardiomyopathies and sutff Flashcards
What is the ventricular morphology of dilated cardiomyopathy?
Dilated LV with little hypertrophy
What is the ventricular morphology of arrhythmogenic RVCM?
dilated RV with sections of missing muscle.
What is the ventricular morphology of HCM?
Marked hypertrophy.
What is the ventricular morphology of RCM?
fibrotic/infiltrated myocardium
What are the symptoms of DCM?
Fatigue, weakness, dyspnea, orthopnea, paroxysmal nocturnal dyspnea
What are the symptoms of ARVCM?
R heart failure
Palpatations
Syncope
Sudden death
What are the symptoms of HCM?
Exertional dyspnea Angina Syncope A-Fib, V-Fib CHF Sudden Death (1.5%)
What are the symptoms of RCM?
Dyspnea
Fatigue
What presents in the physical exam for DCM?
pulmonary rales
s3
functional regurge
possible RV failure signs
What presents in the physical exam for ARVCM?
rhythm disturbances (ventricular arrhythmias)
What presents in the physical exam for HCM?
S4
Outflow obstruction: systolic murmur and mitral regurgitation
What presents in the physical exam for RCM?
Signs of RV failure
JVD
Hepatomegaly
Peripheral edema
What is the pathophysiology of DCM?
imparied systolic contraction
mural thrombi is common
What is the pathophysiology of ARVCM?
nothing.
What is the pathophysiology of HCM?
impaired diastolic relaxation
LV systolic function vigorous with dynamic obstruction of mitral valve during mid-to-late systole
What is the pathophysiology of RCM?
“stiff” LV
impaired diastolic relaxation
normal systolic function
What is the cardiac size for DCM?
Dilated/enlarged
What is the cardiac size for HCM?
normal or dilated
What is the cardiac size for RCM?
usually normal
What does the echo show for DCM?
dilated, poorly contractile LV
What does the echo show for HCM?
LV hypertrophy more pronounced in septum (acquired) or concentric (genetic)
Systolic anterior movement of MV with mitral regurge
What does the echo show for RCM?
Usually normal systolic contraction
Speckled appearance in infiltrative disorders
What does the histology show for DCM?
Interstitial fibrosis
replacement fibrosis
What does the histology show for ARVCM?
myocyte atrophy
fibrosis
fatty tissue
What does the histology show for HCM?
increased diameter of myocytes
abnormal arteries
myofiber disarray
What does the histology show for RCM?
interstitial fibrosis
What is the pathogenesis for DCM?
- often in 30s and 40s
- genetic, usually autosomal dominant
- in kids with DCM, often mitochondrial gene mutations
- infectious: coxsackie, adenovirus, parvovirus, HIV
- iron overload
- child birth
What is the pathogenesis for ARVCM?
- autosomal dominant with variable penetrance
- defective desmosomes > detachment of myocytes at intercalated discs > myocyte death and fibrosis
- disease occurs in bursts
What is the pathogenesis for HCM?
- heart thickening usually happens in kids
- autosomal dominant mutation of sarcomeric genes (B-myosin heavy chain, myosin binding protein C, Troponin T)
- *Troponin T is important**
What is the pathogenesis for RCM?
- genetics, involving Troponin I and desmin genes
- amyloidosis
- endomyocardial fibrosis
- Loeffler endomyocarditis: hypereosinophilia due to release of granulocytes
What is the treatment for ARVCM?
none (80% dies of arrhythmias, 20% die of CHF)
What is the treatment for HCM?
- implanted automatic defibrillator
- negative inotropic agents
- myocardial reduction with surgery or alcohol
What is the treatment for RCM?
Can be treated with platelet derived growth factor
