Restrictive CM

Question 1

What is heart failure with reduced EF abbreviated as?

Answer 1

HFrEF

HFrEF stands for Heart Failure with reduced Ejection Fraction.

Question 2

What is the ejection fraction (EF) percentage for HFrEF?

Answer 2

EF < 50%

An ejection fraction of less than 50% indicates reduced heart function.

Question 3

What percentage of heart failure patients are estimated to have HFrEF?

Answer 3

Est. 50%

Approximately half of heart failure patients fall under this category.

Question 4

What is heart failure with preserved EF abbreviated as?

Answer 4

HFpEF

HFpEF stands for Heart Failure with preserved Ejection Fraction.

Question 5

What is the ejection fraction (EF) percentage for HFpEF?

Answer 5

EF > 50%

An ejection fraction of greater than 50% indicates preserved heart function.

Question 6

What percentage of heart failure patients are estimated to have HFpEF?

Answer 6

Est. 50%

Approximately half of heart failure patients fall under this category.

Question 7

What is Restrictive Cardiomyopathy (RCM)?

Answer 7

Increased resistance to ventricular filling due to increased myocardial stiffness (decreased ventricular compliance)

RCM is characterized by the heart’s inability to fill properly due to stiffness of the heart muscle.

Question 8

How is ventricular compliance related to Restrictive Cardiomyopathy?

Answer 8

Inversely related

As myocardial stiffness increases, ventricular compliance decreases, making it harder for the heart to fill with blood.

Question 9

What may be associated with Restrictive Cardiomyopathy?

Answer 9

Elevated ventricular diastolic pressure and increased atrial pressure

These pressures can indicate how well the heart is filling and functioning during diastole.

Question 10

What is the global systolic function in Restrictive Cardiomyopathy?

Answer 10

May be preserved

Despite the filling issues, the heart’s ability to pump blood during systole can remain normal.

Question 11

What are the classifications of Restrictive Myopathy?

Answer 11

• Non-infiltrative
• Infiltrative

Includes idiopathic and other causes like familial, HCM, and scleroderma.

Question 12

What is a key characteristic of idiopathic restrictive myopathy?

Answer 12

Unknown cause

Idiopathic means that the exact cause of the myopathy is not identified.

Question 13

Name one type of infiltrative restrictive myopathy.

Answer 13

• Amyloid
• Sarcoid
• Storage Dz
• Hemochromatosis
• Endomyocardial
• Fibrosis
• Hypereosinophilic
• Carcinoid heart Dz
• Metastatic CA
• Radiation
• Chemotherapy

Amyloid is the most common infiltrative cause, associated with abnormal protein production in the bone marrow.

Question 14

What does hemachromatosis indicate in restrictive myopathy?

Answer 14

High ferritin (iron) levels

Hemochromatosis is characterized by excessive iron accumulation in the body.

Question 15

What is the most common cause of infiltrative restrictive myopathy?

Answer 15

Amyloid

Amyloid is associated with abnormal protein produced in the bone marrow.

Question 16

What autoimmune disorder is associated with infiltrative restrictive myopathy?

Answer 16

Sarcoid

The cause of sarcoid is unknown.

Question 17

What is another name for fibrosis in restrictive myopathy?

Answer 17

Poverty Dz

Fibrosis refers to the thickening and scarring of connective tissue.

Question 18

True or False: Chemotherapy can cause infiltrative restrictive myopathy.

Answer 18

True

Chemotherapy is known to have various side effects, including cardiac complications.

Question 19

Fill in the blank: A condition characterized by high eosinophil levels leading to restrictive myopathy is called _______.

Answer 19

Hypereosinophilic

Hypereosinophilia can lead to tissue damage and organ dysfunction.

Question 20

What are systemic diseases with cardiac manifestations?

Answer 20

Disease processes that affect a number of tissue and organ systems.

Types include hemolytic, infiltrative storage, systemic rheumatic, hereditary, and connective tissue disorders.

Question 21

Define hemolytic disorder: hyper eosinophilic syndrome.

Answer 21

Overproduction of eosinophils, which are white blood cells; also known as Lafler endocarditis.

Commonly affects young individuals aged 20 to 50 years.

Question 22

What are the cardiac manifestations of hyper eosinophilic syndrome?

Answer 22

Dilated or restrictive cardiomyopathy, endocardial fibrosis, mural thrombus, TIA/CVA.

TIA stands for transient ischemic attack and CVA stands for cerebrovascular accident.

Question 23

What is hemochromatosis?

Answer 23

An iron storage disease that can affect multiple organs, most common in men; it is a primary hereditary disease.

It results in dilated restrictive cardiomyopathy.

Question 24

What are the cardiac manifestations of hemochromatosis?

Answer 24

Dilated restrictive cardiomyopathy with deposits most in the sub epicardium.

Treatment options include phlebotomy and iron chelation therapy.

Question 25

Define cardiac amyloidosis.

Answer 25

A multisystem disease with extracellular deposition of amyloid protein in various organs, leading to tissue damage and organ malfunction. ## Footnote Affected organs include the kidney, heart, liver, nerve, skin, and tongue.

Question 26

What are the signs of right heart failure during a physical examination?

Answer 26

Jugular venous distention, peripheral edema, hepatomegaly, ascites, and widespread swelling. ## Footnote Ascites refers to abdominal swelling.

Question 27

What are common EKG findings in cardiac amyloidosis?

Answer 27

Arrhythmias, low-voltage QRS complexes, increased wall thickness, left bundle branch block. ## Footnote These findings are hallmarks of cardiac amyloidosis.

Question 28

What are the echo findings in cardiac amyloidosis?

Answer 28

Heart failure with preserved EF, varying degrees of diastolic dysfunction, symmetric thickening of LV walls greater than 1.5 cm, unexplained thickening of the RV, and decreased cardiac output. ## Footnote Atrial enlargement and pericardial effusion may also be observed.

Question 29

What are the medical treatments for cardiac amyloidosis?

Answer 29

Diuretics, ACE inhibitors, anticoagulation, antiarrhythmic drugs, bone marrow stem cell transplant, cardiac resynchronization therapy, ICD, pacemaker. ## Footnote ICD stands for implantable cardioverter-defibrillator.

Question 30

What is the surgical treatment for cardiac amyloidosis?

Answer 30

Heart transplant.

Question 31

What is the significance of global longitudinal strain (GLS) in cardiac amyloidosis?

Answer 31

GLS can identify a unique pattern seen only in cardiac amyloidosis, termed apical sparing. ## Footnote This pattern shows near normal strain values at the apex with low strain values at the base and midsection of the left ventricle.

Question 32

Fill in the blank: The approach to restrictive cardiomyopathy includes determining the LV wall thickness, LV global/segmental systolic function, and grade of LV diastolic dysfunction, as well as assessing _______.

Answer 32

RV size, wall thickness and function.

Question 33

What may indicate high pulmonary artery systolic pressure in restrictive cardiomyopathy?

Answer 33

High left side filling pressures.