Cardiomyopathies Flashcards by Tim Montrief

What are the three major types of cardiomyopathies?

Dilated
Restrictive
Infiltrative (Restrictive Infiltrative)

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What % of Dilated cardiomyopathies have diminished systolic dysfunction?

100%

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What leaflet motion is present in the mitral valve for dilated cardiomyopathy?

Carpentier Class 3b = Restricted leaflet motion in systole

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What is a Type I Carpentier Mitral Valve Motion?

Normal Motion of Mitral Valve Leaflets

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What is a Type II Carpentier Mitral Valve Motion?

Increased mobility

Flail leaflet

Ruptured cord

Billowing

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What is a Type IIIa Carpentier Mitral Valve Motion?

Restricted motion during sysole and diastole

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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

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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

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What is the most common substance in infiltrative cardiomyopathy?

Beta Amyloid in Amyloidosis

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What % of patients with infiltrative cardiomyopathy have diastolic dysfunction?

100%

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What is the most common cardiomyopathy?

Dilated Cardiomyopathy

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What are the major etiologies of dilated cardiomyopathy?

Idiopathic
Peripartum
Infections
Genetic (Muscular Dystrophy)
Toxins

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What infections can cause a dilated cardiomyopathy?

Post Viral (Coxsackie B virus, Chagas Disease)

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What toxins can cause dilated cardiomyopathy?

Alcohol

Doxorubicin

Cobalt

Snake Bites

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What is the mortality rate of dilated cardiomyopathy for 2 years?

50%

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What is the mortality rate of dilated cardiomyopathy for 5 years?

75%

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What percent of dilated cardiomyopathy improve?

25%

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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** 1. Anterior motion of Mitral Valve 2. 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?

1. During systole, valve abruptly opens 2. Valve stays open Resembles a "box"

What is seen here?

1. Aortic valve leaflets open, but close pre-maturely due to LVOT proximally **\*Coarse fluttering of the aortic valve leaftlets with premature closure\*** 2. Proximal obstruction pushing the leaflet open

What are the two major etiologies of Restrictive infiltrative cardiomyopathy?

1. Primary 2. Secondary

What are the **three** major **primary** etiologies of Restrictive infiltrative cardiomyopathy?

1. Loeffler's Hypereosinophilic Endocarditits 2. Endomyocardial Fibrosis 3. Idiopathic

What are the **six** major **secondary** etiologies of Restrictive infiltrative cardiomyopathy?

1. Amyloidosis 2. Sarcoidosis 3. Glycogen Storage Diseases 4. Hemochromatosis 5. Drugs (Antrhocyclines, Ergotamines, Methysergide, Serotonin) 6. 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 E_M in RICM vs. Constrictive Pericarditis?

E_M\< 8 cm/sec in RICM E_M \>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