Module 16 : Restrictive and Other Cardiomyopathies Flashcards
what is another term for restrictive cardiomyopathy RCMO
- infiltrative CMO
what does RCMO refer to
- disease other than ischemici heart disease which cause increased resistance to LV filling due to increased myocardial stiffness
- increased LVEDP
- increase mLAP
what is the main cause of RCMO
- abnormal infiltration, storage, or fibrosis within the myocardium
what are the 4 classes of RCMO
- non infiltrative
- infiltrative
- storage disease
- endomyocardial
what are the 2 main causes of non infiltrative RCMO
- HCMO
- diabetic CMO
what are the 2 causes of infiltrative RCMOq
- amyloidosis
- sarcoidosis
what is a main cause of storage disease RCMO
- GSD
what are the 2 endomyocardial causes of rCMO
- endomyocardial fobroelastosis
- carcinoid heart disease
what is the etiology of infiltrative RCMO
- disease of heart muscle secondary to a disorder that produces histological changes to cardiac muscle by depositing a substance inside or in between muscle cells
what 2 things may infiltrative RCMO lead to
- infiltration of myocardium
- restrictive filling pattern
what are the 3 clinical findings of RCMO
- diastolic dysfunction (HFnEF) backwards failure
- early stags normal systolic function
- arrhythmias
what are the echo findings in RCMO
- LV cavity size normal
- wall thickness increased
- bi atrial enlargments
- small pericardial effusion
what are the 5 roles of echo in RCMO
- asses chamber function and size
- degree of DD
- degree of MR or TR
- determine RVSP
- differentiate from constrictive pericarditis
what will the ECG look like with RCMO
- atrial and ventricular arrhythmias
- low voltage QRS
- conduction defects
what are the 5 medical treatments of restrictive CMO
- treat underlying aetiology
- diuretics
- ace inhibitors
- anticoagulnats
- anti arrhythmias
what are the 2 surgical treatments of RCMP
- pace maker / defibrilaors
- cardiac transplant
what is the most common type of RCMO
- amyloidosis
what is amyloidosis RCMO
- deposits of amyloid protein in between myocytes
- inderdiagnosed
is diagnosis of amyloid CMO easy or hard
- very difficult
what 5 other organs may be affected by amyloid CMP
- heart
- liver
- kidnyes
- spleen
- skin
what are the 4 classifications of amyloid disease
- primary idiopathic = more common in males
- secondary
- familial
- senile
what will amyloid CMO look like eon ECG
- low voltage ECG
- LVH plus low voltage ECG importnatn
- pseudo infarction
- afib
- ventricular arrhytmias
- conduction defects
what are the 4 medical treatments of amyloid CMO
- digitalis, digoxin
- diuretics
- vasodilators
- anticoagulants
what are the 4 surgical treatments of amyloid CMO
- bone marrow stem cell transplant
- pacemaker
- implantable device
- transplant
what are the 7 echo features of amyloid CMO
- granular speckled appearance
- concentric LVH and RVH
- WMA
- asymmetric septal thickening
- bi atrial enlargements
- pericardial effusion
- PHTN
what does an increase in wall thickness mean in regards to DD
- thick the wall = more severe diastolic dysfunction
what are the early changes in systolic function with amyloidosis
- LV systolic function normal and normal size
what are the late changes seen in systolic function with amyloidosis
LV may dilates
- LVEF reduce
what is sarcoidosis
- multiple granulomatous disease which involves the heart
- females more
- rare
what is theist common site for granulomas to infiltrate
- lungs
what are the clinical features of sarcoidosis
- progressive heart failure
- diffuse pulmonary fibrosis = PHTN
- death
- dysrhtymias
- 3rd degree AV blocks
what are the echo features of sarcoidosis
- pericardial effusion
- increase anterior RV thickness > 5mm
- PTHN
- RV dysfunction
- MR/TR
- DD
- late = dilation of LV, wall thinning, reduced EF, WMA
what is hemochromatosis and what are the 2 groups
- iron storage disease affecting multiple organ systems resulting in organ damage and malfunction
- primary, secondary
what is primary hemochromatosis
- inherited
what is secondary hemochromatosis
- iron overload due to repeated blood transactions with patients with chronic anemia, hemodialysis, alcoholic liver disease
what is the clinical pentad of hemochromatosis
- CHF
- cirrhosis
- impotence
- diabetes
- arthritis
what will the ECG look like with hemochromatosis
- AF
- low voltage
- SVTs
- AV blocks
what is another name for endomyocardial syndrome
- hypereosinphilic
- perisitant elevated blood eosinophil counts
what are the four causes of perisitant elevated blood eosinophil counts
- idiopathic
- malignant
- reactive
- infectious
what does endomyocardial syndrome evolve from
- infiltration of eosinophils to thrombosis
- restriction due to fibrotic tissues that lines myocardium decreased LV function and CHF then death
what is GSD
- excessive storage of glycogen in tissue
- hereditary
- heart becomes enlarged and globular in shape
- thickened RV LV and IVS
what are 4 other infiltrative CMOS
- arrhytmogenic RV dysplaisia
- carcinoid heart disease
- fabrys disease
- collagen disorders
characteristics of arrhytmogenic RV dysplasia
- genetiv
- fibrofatty
- RV systolic and diastolic dysfunction
- RV dilation RV small aneurysms
- RV trabeculation get bigger
- normal LV
- arrhytmias
characteristics of LV non compaction
- gentic
- LV wall thickening with deep recess between trabeculae
- hypokinesis
- arrhytmias
- heart failure
development of LV non compaction
- embryonic development
- before coronary arteries blood supplied by trebeculation in blood
- once coronaries developed the LV compass from epicardium to endocardium and base to apex
- LV non compaction is seen usually in apex
