Cardiomyopathies Flashcards
What are the three major types of cardiomyopathies?
- Dilated
- Restrictive
- Infiltrative (Restrictive Infiltrative)
What % of Dilated cardiomyopathies have diminished systolic dysfunction?
100%
What leaflet motion is present in the mitral valve for dilated cardiomyopathy?
Carpentier Class 3b = Restricted leaflet motion in systole
What is a Type I Carpentier Mitral Valve Motion?
Normal Motion of Mitral Valve Leaflets
What is a Type II Carpentier Mitral Valve Motion?
Increased mobility
Flail leaflet
Ruptured cord
Billowing
What is a Type IIIa Carpentier Mitral Valve Motion?
Restricted motion during sysole and diastole
What is the inheritance pattern for hypertrophic cardiomyopathy?
(Specific 3 things)
1. Autosomal Dominant
2. Variable Penetrance - Penetrance refers to the proportion of people with a particular genetic change (such as a mutation in a specific gene) who exhibit signs and symptoms of a genetic disorder
3. Variable expressivity - In genetics, expressivity is the degree to which a phenotype is expressed by individuals having a particular genotype
What arrythmogenic complications can arise from hypertrophic cardiomyopathy patients?
RBBB
(Will have pacemakers in place that depolarize right heart before left heart)
Septum depolarizes before lataral wall
This will decrease the outflow tract obstruction
What is the most common substance in infiltrative cardiomyopathy?
Beta Amyloid in Amyloidosis
What % of patients with infiltrative cardiomyopathy have diastolic dysfunction?
100%
What is the most common cardiomyopathy?
Dilated Cardiomyopathy
What are the major etiologies of dilated cardiomyopathy?
- Idiopathic
- Peripartum
- Infections
- Genetic (Muscular Dystrophy)
- Toxins
What infections can cause a dilated cardiomyopathy?
Post Viral (Coxsackie B virus, Chagas Disease)
What toxins can cause dilated cardiomyopathy?
Alcohol
Doxorubicin
Cobalt
Snake Bites
What is the mortality rate of dilated cardiomyopathy for 2 years?
50%
What is the mortality rate of dilated cardiomyopathy for 5 years?
75%
What percent of dilated cardiomyopathy improve?
25%
What end diastolic diameter of dilated cardiomyopathy predicts a higher mortality?
> 4 cm / m2
What pathology is seen with Type IIIa leaflet motion?
Rheumatic Heart Disease
Outside of Dilated cardiomyopathy, when will we see Type IIIb motion of the mitral valve?
Ischemic mitral insufficiency
More of a ventricular (papillary muscle) problem not a leaflet problem
What emptying velocity using pulse wave doppler on the left atrial appendage when is there likely to be no clot?
>40 cm/sec = Risk of low
>55 cm/sec = 100% negative predictive value
When is there a 100% Negative predictive value of thrombus in the left atrial appendage?
55-60 cm/sec
What wall is typically spared of hypertrophy in all subtypes of Hypertrophic cardiomyopathy?
Basal Inferolataral wall
AKA
Basal Posterior wall
Comment on the systolic function of Hypertrophic cardiomyopatahy.
Normal
Comment on the diastolic function of Hypertrophic cardiomyopatahy.
Grossly abnormal
What percentage of hypertrophic cardiomyopathy patients have obstructive physiology in the LVOT?
25%
What is seen here?
Y sign for LVOT obstruction
- Anterior motion of Mitral Valve
- Posterior direction of mitral insufficiency
What is seen here on CWD?
Late peaking dagger shaped CWD profile
Early in systole = No flow acceleration
Late in systole = Late peaking dagger shape
If you want to use M-mode to assess HOCM, what do you do?
Put cursor across Aortic Valve in Long Axis
Select M Mode
What is seen here in M-mode across aortic valve?
- During systole, valve abruptly opens
- Valve stays open
Resembles a “box”
What is seen here?
- Aortic valve leaflets open, but close pre-maturely due to LVOT proximally
*Coarse fluttering of the aortic valve leaftlets with premature closure*
- Proximal obstruction pushing the leaflet open
What are the two major etiologies of Restrictive infiltrative cardiomyopathy?
- Primary
- Secondary
What are the three major primary etiologies of Restrictive infiltrative cardiomyopathy?
- Loeffler’s Hypereosinophilic Endocarditits
- Endomyocardial Fibrosis
- Idiopathic
What are the six major secondary etiologies of Restrictive infiltrative cardiomyopathy?
- Amyloidosis
- Sarcoidosis
- Glycogen Storage Diseases
- Hemochromatosis
- Drugs (Antrhocyclines, Ergotamines, Methysergide, Serotonin)
- Radiation
Of the primary and secondary causes of Restrictive infiltrative cardiomyopathy, which is the most common?
Amyloidosis
What is the major pathophysiologic cause of amyloidosis causing Restrictive infiltrative cardiomyopathy?
infiltration of Beta Amyloid
How does the myocardium appear in amyloidosis?
Granular, Speckled or Starry Skied
What is the systolic and diastolic function of Restrictive infiltrative cardiomyopathy?
Diastolic dysfunction always
Systolic dysfunction
How do you distinguish between restrictive infiltrative cardiomyopathy and constrictive pericarditis?
RICM:
Restrictive transmitral inflow velocitites (E > A)
Reduced tissue doppler velocities
Constrictive Pericarditis:
Restrictive transmitral inflow velocitites (E > A)
Normal Tissue doppler velocities (even elevated sometimes)
Quantitatively, what is the EM in RICM vs. Constrictive Pericarditis?
EM < 8 cm/sec in RICM
EM >8 cm/sec in constrictive pericarditis
What determines prognosis in Restrictive infiltrative cardiomyopathy?
Wall Thickness
Diastolic Function
Systolic Function
Once symptoms occur in RICM, what is survival?
2-3 year survival <50%
What do amyloidosis patients require before heart transplantation?
Bone marrow biopsy to get rid of amyloid cells
How do you distinguish an unroofed septal perforator vs. VSD
Look at timing of Flow LV to RV
Flow during systole with VSD
Flow during diastole with Unroofed septal perforator
