Restrictive And Other Cardiomyopathies Flashcards
Restrictive cardiomyopathy is also known as what?
Infiltrative CMO
What does RCMO refer to?
Increased resistance to LV filling due to increased myocardial stiffness
What is the main cause of RCMO?
Abnormal infiltration, storage or fibrosis within the myocardium
What is the cause of non-infiltrative RCMO?
Idiopathic, familial, hypertrophic (can cause restrictive physiology), scleroderma, diabetic (fibrosis)
What is the cause of infiltrative RCMO?
Amyloidosis, sarcoidosis, Hurler’s disease, Loeffler’s disease
What is the cause of storage disease RCMO?
Hemochromatosis, Fabry disease, glycogen storage disease
What is the cause of endomyocardial RCMO?
Endomyocardial fibrosis, carcinoid heart disease, radiation, toxic effects from anthracycline (chemo)
What is the etiology of infiltrative diseases related to RCMO?
Disease of heart muscle that is secondary to disease/disroder that produces histologic changes to cardiac muscle
What kind of diastolic function is seen with RCMO?
HFnEF (backwards failure)
What symptoms are seen with Left CHF?
Dyspnea, orthopnea, paroxysmal nocturnal dyspnea, fatigue, cough, weight gain
What symptoms are seen with right CHF?
Jugular venous distension, hepatomegaly, peripheral edema, ascites, anasarca
What echocardiographic findings are seen with RCMO?
Normal LV cavity, increased wall thickness, bi-atrial enlargement, small pericardial effusion
What things will you see on an ECG with RCMO?
Atrial and ventricular arrhythmias, low voltage QRS, conduction defects
What things will you see on a chest xray with RCMO?
Cardimegaly, pulmonary congestion/effusion
What things will you see on a cardiac MRI with RCMO?
May demonstrate infiltration
What are medical tx for RCMO?
Treat underlying etiology, diuretics, ACE inhibitors, anticoagulants, anti-arrhytmics, pacemaker
What are surgical tx for RCMO?
Very limited, cardiac transplant
What is the most common type of RCMO?
Infiltrative CMO - amyloidosis
Why is infiltrative CMO amyloidosis misdiagnosed in early stages as LVH?
Due to hypertension
What does infiltrative CMO amyloidosis do?
Produces a stiff myocardium which prevents filling
What are the four classifications of amyloid disease?
Primary/idiopathic, secondary, familial, senile
What is secondary amyloid disease associated with?
Inflammatory disorders like rheumatoid arthritis, tuberculosis, Chron’s disease
What is senile amyloid disease also known as?
Transthyretin (TTR) type CMO
What is a very important finding for amyloid?
LVH on 2D plus low voltage ECG from myocardium
Pseudo-infarction pattern with amyloid shows as what on the ECG and is caused by what?
Abnormal Q waves and is caused by myocardial fibrosis
How does the myocardium appear with amyloid?
Granular (speckled)
What are echo features of amyloid?
- Concentric LVH and RVH
- Wall motion abnormalities
- Asymmetrical septal thickening
- Mild-moderate bi-atrial enlargement
- Pericardial effusion
- Evidence of pulmonary hypertension
The greater the wall thickness the more severe the what?
The more severe the diastolic dysfunction
A measure of >15 mm in wall thickness suggests what for patients?
Restrictive filling pattern
What parameters suggest increased filling pressures?
Mitral E/A: >2.0
Decel time: >150 ms
TDI e’ septal: <5 cm/s
E/e’ Ratio: >15
What late changes with amyloid may make RCMO indistinguishable from DCMO?
LV dilation, LVEF reduced, LA enlargement
What is sarcoidosis?
Multiple granulomatous disease which involves the heart in 25% of patients
With sarcoidosis, granulomas infiltrate what?
Lungs, lymph nodes, liver, spleen, skin, parotid glands, heart
What are the echo features of sarcoidosis?
- Pericardial effusion
- Increased anterior RV thickness >5 mm
- Pulonary HPTN
- RV dsfx due to PAH
- MR/TR
- Diastolic dysx
What are echo features of early sarcoidosis?
Increased wall thickness
What are echo features of late sarcoidosis?
- Aneurysmal dilation of the LV
- Segmental wall thinning
- LV dilation and reduced EF
- Wall motion abnormalities
What is hemochromatosis?
Iron storage disease affecting multiple organ system that results in organ dmage and malfunction
What is primary hemochromatosis?
Inherited (metabolic syndrome: excessive absorption of iron from diet and reduced elimination)
What is secondary hemochromatosis?
Iron overload due to repeated blood transfusions in patients with chronic anemia, prolonged haemodialysis, alocoholic liver disease
What is the clinical pentad of hemochromatosis?
- CHF
- Cirrhosis
- Impotence
- Diabetes
- Arthritis
How does ECG appear with hemochromatosis?
Afib, low voltage, SVTs, atrioventricular conduction defects
Describe primary hemochromatosis?
Genetic, male to female 3:1, affects men >40, women >50
Describe secondary hemochromatosis?
Chronic liver disease, multiple blood transfusions
What are the echo features of hemochromatosis?
Features related to dilated CMO, increased wall thickness (RVH, LVH), preserved systolic function
What are endomyocardial syndromes also known as?
Hypereosinophilic syndromes
What do endomyocardial syndromes evolve from?
Infiltration of eosinophils to thrombosis and scarring, with risk of embolism
Endomyocardial syndromes refer to persistently elevated blood eosinophil counts due to:
- Idiopathic
- Malignant
- Reactive
- Infectious
What is restriction due to with endomyocardial syndromes?
Fibrotic tissue that lines the myocardium (decreased LV function leads to CHF, and then death)
What is ARVD?
Arrhythmogenic RV cardiomyopathy
Carcinoid heart disease not only affects valves of the right heart but also what?
Right heart endocardium
What is LV non-compaction?
Genetic LV wall thickening with deep recesses between trabeculae
How does the compaction process normally occur?
Epicardium to endocardium and base to apex
Where is the last part of the endocardium to compact?
Apical (where non-compaction is seen)
How will EF appear with dilated cardiomyopathy?
Mod/sev decreased
How will EF appear with hypertrophic cardiomyopathy?
Normal
How will EF appear with restrictive cardiomyopathy?
Normal
How will LV diastolic fx appear with dilated cardiomyopathy?
Abnormal
How will LV diastolic fx appear with hypertrophic cardiomyopathy?
Abnormal
How will LV diastolic fx appear with restrictive cardiomyopathy?
Abnormal
How will LV mass appear with dilated cardiomyopathy?
Increased (due to dilation)
How will LV mass appear with hypertrophic cardiomyopathy?
Asymmetric LVH variable
How will LV mass appear with restrictive cardiomyopathy?
Concentric LVH
Which chambers will dilate with dilated cardiomyopathy?
All 4
Which chambers will dilate with hypertrophic cardiomyopathy?
LA and RA if MR
Which chambers will dilate with restrictive cardiomyopathy?
LA and RA
