Cardiomyopathy Flashcards

1
Q

Case presentation

A

19 y/o Male, presents to your office with complaints of palpitations and occasional chest pain especially when he has been practicing for his football team

Fam Hx: Uncle passed away at age 25, Cousin has heart problem

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2
Q

Definition of cardiomyopathy

A

a heterogeneous group of diseases of the myocardium associated with mechanical &/or electrical dysfunction that usually (but not invariably) exhibit inappropriate ventricular hypertrophy or dilatation and are due to a variety of causes that frequently are genetic. Cardiomyopathies either are confined to the heart or are a part of generalized systemic disorders, often leading to cardiovascular death or progressive heart failure-related disability

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3
Q

Dilated cardiomyopathy

A

Dilatation and impaired contraction of the left or both ventricles (chamber enlargement)

  • Ventricular dilation, hypokinetic left ventricle, and systolic dysfunction
  • can be inherited
  • congestive presentation
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4
Q

Hypertrophic cardiomyopathy

A

Left and/or right ventricular hypertrophy, often asymmetrical, which usually involves the interventricular septum.

-Inappropriate myocardial hypertrophy, with or without left ventricular obstruction

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5
Q

Restrictive cardiomyopathy

A

Restricted filling and reduced diastolic size of either or both ventricles with normal or near-normal systolic function.

-Abnormal ventricular filling with diastolic dysfunction

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6
Q

Arrhythmogenic right ventricular cardiomyopathy

A

Progressive fibro-fatty replacement of the right ventricle

-Fibroadipose replacement of right ventricle

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7
Q

Common causes of Dilated Cardiomyopathy

A
  1. Idiopathic
  2. Toxin induced (alcohol, anthracycline, cobalt, catecholamines)
  3. Radiation
  4. Infection (viral, HIV, parasite)
  5. Metabolic (starvation, thiamine deficiency like beriberi, thyrotoxicosis)
  6. Sarcoidosis
  7. Hemochromatosis
  8. Peripartum/postpartum
  9. Genetic
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8
Q

Dilated cardiomyopathy incidence

A
  • most common of all
  • myocyte injury and fibrosis
  • Third most common cause of heart failure
  • Middle age, men > women
  • Annual mortality: 12% !
  • Most frequent cause of heart transplantation
  • complete recovery is rare
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9
Q

S/S of Dilated cardiomyopathy

A
  1. Pulmonary congestion-left heart failure: dyspnea (rest, excertional, nocturnal), orthopnea
  2. Systemic congestion- right heart failure: edema, nausea, abdominal pain, nocturia
  3. Hypotension (low CO), tachycardia, tachypnea, fatigue and weakness
  4. Arrhythmia
    Atrial fibrillation, conduction delays, complex PVC’s, sudden death
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10
Q

During dilated cardiomyopathy cardiac exam shows

A

S3, S4 and murmur of TR and/or MR

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11
Q

CXR

A

shows enlarged heart, CHF

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12
Q

Treatment for dilated cardiomyopathy

A
  • Limit activity based on functional status
  • Salt restriction
  • Fluid restriction
  • Diuretics
  • Beta Blockers, ACE/ARB (improve mortality)
  • Hydralazine/nitrate combination if cannot tolerate ACE/ARB
  • Spironolactone if EF
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13
Q

Dilated cardiomyopathy is the most common indication for ________

A

cardiac transplantation
Survival after transplant is
80% one year
70% 5 years

-or can do left vent reshaping

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14
Q

People with Hypertrophic Cardiomyopathy (HCM) also have:

A
  • Small LV cavity
  • septal hypertrophy (huge)
  • systolic anterior motion of the mitral valve leaflet +/- obstruction of left ventricular outflow
  • low stroke volume
  • elevated EF
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15
Q

Usually patients with Hypertrophic Cardiomyopathy (HCM) are

A

very young

may lead to MI, mitral regurgitation or arrhythmias

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16
Q

What type of dysfunction is Hypertrophic Cardiomyopathy and what does it lead to?

A

Diastolic dysfunction (impaired filling, increased filling pressure)

Leads to pulmonary congestion (have LV outflow obstruction and hyper dynamic systolic function bc of so much muscle, especially septum)

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17
Q

“Classic” feature of hypertrophic cardiomyopathy is

A

Dynamic left ventricular outflow tract obstruction

absent in about half of people and obstruction varies greatly

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18
Q

The severity of LV outflow obstruction increases with

hypertrophic obstructive= HOCM

A

**exercise
-positive inotropic agents
volume depletion

Anything that decreases filling:

  • *sudden assumption of upright posture
  • tachycardia
  • *Valsalva maneuver
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19
Q

S/S of hypertrophic cardiomyopathy

A
  • Asymptomatic (Echo finding only)
  • Dyspnea in 90%
  • Angina pectoris in 75% (ISCHEMIC CHEST PAIN)
  • Fatigue, pre-syncope, SYNCOPE (incr. risk of SCD)
  • Palpitation, PND, CHF, dizziness
  • Atrial fibrillation, thromboembolism
  • SUDDEN CARDIAC DEATH
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20
Q

In HCM, ejection murmur is heard at

A

left sternal border

-does NOT radiate to the carotid arteries

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21
Q

HCM, the EKG will show

A

*LVH (Strain pattern) and *abnormal Q waves

may see giant T wave inversions or LA enlargement, bbb or arrhythmias

22
Q

In HCM, the valsava will

A

decrease LV volume and INCREASE THE MURMUR

-standing will do this too

23
Q

In HCM, squatting will

A

increase LV volume and DECREASE THE MURMUR

24
Q

Echo findings in HCM

A
  • Echocardiogram is best test
  • Left ventricular hypertrophy > 1.3 cm
  • Septal to posterior wall ratio > 1.3:1
  • Mitral regurgitation
  • Systolic anterior motion of the mitral valve (SAM)
  • Premature midsystolic closure of the aortic valve
  • Asymmetric septal hypertrophy (ASH)
  • Diastolic dysfunction
  • Left ventricular outflow tract obstruction (LVOT)
25
Q

Recommendation when there is a family history of HCM

A

Serial ECHOs are recommended up to the age of 20 yr where there is a family history of HCM

-LVH usually develops between 5-15 y/o

26
Q

Accounts for 36% of deaths in

A

athletes

27
Q

Management of HCM

A
  • careful family history focused on sudden cardiac death
  • counseling regarding avoidance of strenuous exercise, avoidance of dehydration
  • all first-degree family members should be periodically screened with an echo
28
Q

HCM treatment

A
  1. FIRST- Beta blockers
    - CCB
    - Anti-arrhythmics – Amiodarone, disopyramide
    - Pacemakers (ICD) / Defibrillators (AICD)
    * **Myomectomy (resection of septum)
    * **Alcohol septal ablation (controlled MI through septal perforator perfusing basal septum), leads to wall thinning which decreases LV outflow obstruction
29
Q

Beta blockers help HMC by

A

*NEGATIVE inotropic effect decreases outflow gradient
-decreased myocardial demand results in reduced
ischemia
-prolonged diastolic filling time results in improved LV
filling as well as improved coronary perfusion
-may have an antiarrhythmic effect

30
Q

IN HCM, usually, signs and symptoms _____ with
age
Leading cause of death: _____________

A

increase

sudden death in asymptomatic patients

31
Q

Restrictive Cardiomyopathy (RCM) background

A
  • rare in western world
  • can affect both ventricles
  • systolic fx usually normal (especially early on)
  • SMALL ventricular cavity size
32
Q

RCM is a ________ dysfunction and involves __________

A
  • DIASTOLIC DYSFUNCTION

- elevated vent filling pressures (small ventricular cavity size)

33
Q

RCM has a ________ with impaired ventricular filling

A

rigid ventricular wall

34
Q

RCM bears some functional resemblance to _________

A

constrictive pericarditis

-Importance lies in its differentiation from operable constrictive pericarditis

35
Q

RCM is much less common then DCM or HCM outside the tropics, but frequent cause of death in ________ primarily because of the high incidence of endomyocardial fibrosis in those regions

A

Africa, India, South and Central America and Asia

36
Q

Non-infiltrative causes of RCM

A

*Idiopathic
*Scleroderma
Familial
Hypertrophic

37
Q

Infiltrative causes of RCM

A

Amyloid (most common)
*Sarcoid
Gaucher’s

38
Q

Storage disease causes of RCM

A

Hemochromatosis
Glycogen storage disease
Fabry’s

39
Q

Endomyocardial causes of RCM

A

*Endomyocardial fibrosis
*Hypereosinophilic syndrom
*Radiation
*Chemotherapy toxicity
Carcinoid
Metastatic malignancy

40
Q

Rigid myocardium –> ______ diastolic vent pressure —> venous congestion –> ________

A

increased

-Jugular venous distention, Hepatomegaly, Ascites

41
Q

Rigid myocardium–> _____ ventricular filling –>

_____ CO –> ________

A

decreased
decreased
fatigue and weakness

42
Q

in RCM, Echo shows

A
  • *thick walls, reduced systolic fxn**
  • Abnormal mitral inflow pattern
  • Prominent E wave (rapid diastolic filling)
  • Reduced deceleration time (increased LA pressure)
43
Q

procedure that is commonly necessary in RCM

A

Right heart cath

-can help with endomyocardial biopsy

44
Q

Consider RCM in any patient with

A

R sided heart failure without evidence of either cardiomegaly or systolic dysfunction

45
Q

RCM diagnosis

A

possibly biopsy

46
Q

RCM treatment

A

CHF THERAPY

  • No Clear medical therapy
  • Treat reversible causes
  • Transplant maybe only option
47
Q

GOOD TABLE COMPARING 3 ON SLIDE 56

A

slide 56

48
Q

Arrhythmogenic RV Cardiomyopathy is characterized by

A

fibroadipose replacement of segments of the free wall of the right ventricle

49
Q

Arrhythmogenic RV Cardiomyopathy is predominately found in ____

A

young adults; cause of young adult sudden death

50
Q

Arrhythmogenic RV Cardiomyopathy- all patients who are symptomatic with arrhythmias get

A

ICD implantation