Cardiomyopathies

Question 1

3 major types of cardiomyopathy

Answer 1

1. dilated
2. hypertrophic
3. restrictive

Question 2

Cardiomyopathies often result in symptoms of heart failure through the following mechanisms
• - ___ dysfunction • - __ dysfunction • - ___ obstruction to blood flow
from the heart

Answer 2

Cardiomyopathies often result in symptoms of heart failure through the following mechanisms
• - Systolic dysfunction • - Diastolic dysfunction • - Dynamic obstruction to blood flow
from the heart

Question 3

Systolic dysfunction – the myocytes have decreased ___ function

Diastolic dysfunction – the myocytes contract ___ however cannot fully __. This leaves the ventricles “___” and unable to fill properly

Answer 3

Systolic dysfunction – the myocytes have decreased contractile function (weak squeeze)
Diastolic dysfunction – the myocytes contract normally however cannot fully relax. This leaves the ventricles “stiff” and unable to fill properly

• creates higheer pressures of the heart/ventricle.

Question 4

what diseases are excluded from cardiomyopathies

Answer 4

• valvular heart disease (CMs only affect myocytes)
• congenital heart disease
• coronary artery disease (ischemic cardiomyopathy)

Question 5

outline the changes in heart structure in DCM

Answer 5

DCM=dilated cardiomyotpathy. there is an enlargement of the ventricular chambers, resulting in systolic impairment because of weaker contraction– LV walls think out

Question 6

DCM causes

Answer 6

primary: idiopathic, genetic peripartum

seconary; infection (myocarditis, HIV, hepatitis, sepsis)

• toxins (alcohol, cocaine, chemotherapy)

Question 7

myocarditis inflammation often is due to ____. it is usually self limiting but can develop into ___. Biopsy shows lymphcytes and cell ___. Patients generall present with symptoms and signs of ___ ___

Answer 7

myocarditis inflammation often is due to INFECTION. it is usually self limiting but can develop into DCM. Biopsy shows lymphcytes and cell NECROSIS. Patients generall present with symptoms and signs of HEART FAILURE

Question 8

treatment for myocarditis

Answer 8

standard HF treatment (recall; Acei, ARB, ARNI, Betablockers, diuretics, aldosterone inhibitors). Additional therapies may include corticosteroids, IVIG, antiviral agents

Question 9

risk factors for peripartum cardiomyopathy

Answer 9

• older maternal age
• African descent
• multiple pregnancies

Question 10

pathophysioology of DCM

Answer 10

Question 11

recall these symptoms from previous lectures (HF lecture). In addition to theese signs and symptoms, what heart sounds/aspects would DCM cause?

Answer 11

DCM is a structural disease that results in a dilated ventricle

dilated ventricle = increased size = diffuse/displaced APEX

• holosystolic murmur of the MR or the TR depending on which side is affected– the ventricle is so big and the valve cusps no longer touch each other, resulting in a murmur.

Question 12

Most common cause of sudden death in atheletes

Answer 12

HCM: hypertrophic cardiomyopathy

• often familial (autosomal dominant) resulting from mutation of the sarcomeric cproteins.

Question 13

HCM pathophysiology

Answer 13

thickened myocytes contract well but are “stiff” and cannot relax –> problems filling the ventricle –> high left ventricular diastolic pressure

• given the myocyte disarray, these patients are also at risk for ventricular arrhthmias

Question 14

what is HOCM

Answer 14

hypertrophic OBSTRUCTIVE cardiomyopathy. Results in impaired LV outflow because the growth causes obstruction of the valves.

Question 15

HOCM is ___ hypertrophy (usually the __ side) of the ventricular septum causing narrowing of the ___ outflow tract.

• for blood to flow out of this narrowed area throguh the ___ valve, it must speed up (___ effect) which creates an area of low pressure
• the anterior mitral valve is subsequnetly sucked into this low pressure area, thereby making the obstruction worse and does not allow the MV to close properly, causing __ ___ in addution to the obstruction.

Answer 15

HOCM is ASYMMETRIC hypertrophy (usually the LEFT side) of the ventricular septum causing narrowing of the LV outflow tract.

• for blood to flow out of this narrowed area throguh the AORTIC valve, it must speed up (VENTURI effect) which creates an area of low pressure
• the anterior mitral valve is subsequnetly sucked into this low pressure area, thereby making the obstruction worse and does not allow the MV to close properly, causing MITRAL REGURGITATION in addution to the obstruction.

Question 16

IN HOCM, there are different hemodynamic changes with which the low pressure area/gradient is formed. this pressure gradient is worsened by :

Answer 16

low preload

• low afterload
• increased contractility

Question 17

how would valvular aortic stenosis affect the S2 sound?

Answer 17

doft S2. A2 portion of S2 is usually the louded but with stenosis, it’s sticky and it wont “slam” as hard.

• pulsus parvus et tardus is also a sign as well as a late peaking systolic murmur

Question 18

what heart sound will HOCM produce

Answer 18

an S4 sound: s4 indicates ventricular stiffness during filling. think about it– it’s stiff because in HOCM, there is hypertrophic obstruction.

• HOCM will also produce prominent systolic ejection pattern murmur at LLSB which varies with certain maneuvers
• spiking dome pulse

Question 19

maneuver that can bring out the HOCM murmur

Answer 19

Vaslsalva

Question 20

treatment of HCM

Answer 20

Question 21

RCM results in abnoramlly ___ ventricular walls. This results in ___ dysfunction. The most common cause is amyloidosis

Answer 21

abnormally rigid but not necessarily htick ventricular walls. results in abnormal filling and thus DIASTOLIC DYSFUNCTION (INCREASE PRESSURE (IN ATRIA BECAUSE IT HAS TO COMPENSATE TRYING TO GET BLOOD INTO STIFF VENTRICLE)– systolic function is preserved until end stage

• most common cause is amyloidosis.

Question 23

causes of restrictive CM

Answer 23

• idiopathic
• scleroderma
• amyloidosis
• hemochromatosis
• glycogen storage disease
• endomyocardial disease

Question 24

cardiac amyloid plaques is a large cause for ___ cardiomyoapthy.

• is results in reduced ventricular ___ and thus an increased LV ____ ____ pressure.

it increases ___ venous pressure and decreases stroke volume and cardiac output

Answer 24

cardiac amyloid plaques is a large cause for RESTRICTIVE cardiomyoapthy.

• is results in reduced ventricular COMPLIEANCE and thus an increased LV END DIASTOLIC pressure.

it increases PULMONARY venous pressure and decreases stroke volume and cardiac output

Question 25

Question 26

treatment for restriction CM

Answer 26

honestly it s apoor prognosis with limited treatment

• symptom management (often diuretics
• transplant if candidate