cardiomyopathy

Question 1

cardiomyopathy

Answer 1

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

Question 2

Cardiomyopathies either are confined to the heart or are a part of ??

Answer 2

generalized systemic disorders, often leading to cardiovascular death or progressive heart failure-related disability

Question 3

Cardiomyopathy: 3 Categories + 2

Answer 3

DILATED

HYPERTROPHIC

RESTRICTIVE

ARRHYTHMOGENIC RV (don’t know as much)

ISCHEMIC – not focused upon in this lecture (most common in developed world, CAD)

Question 4

Dilated cardiomyopathy

Congestive, DCM, IDC

Answer 4

most common cause of cardiomyopathy in the world

Dilatation and impaired contraction of the left or both ventricles.
hypokinetic left ventricle, and systolic dysfunction

Question 5

Hypertrophic cardiomyopathy (IHSS, HCM, HOCM, ASH)

Answer 5

Left and/or right ventricular hypertrophy, often asymmetrical, which usually involves the inter ventricular septum
with or without left ventricular obstruction

Question 6

Restrictive (Infiltrative)

cardiomyopathy

Answer 6

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

Question 7

Arrhythmogenic right ventricular cardiomyopathy (ARVD)

Answer 7

Progressive fibro-fatty replacement of the right ventricle

Question 8

Dilated Cardiomyopathy: Background

Answer 8

CONGESTIVE presentations
Can be *inherited*
True cause: *unknown*
CHAMBER ENLARGEMENT
IMPAIRED SYSTOLIC FUNCTION:
    right, left, or biventricular
Myocyte injury and fibrosis

Question 9

Wide spectrum of causes of Dilated Cardiomyopathy (DCM)

Answer 9

Idiopathic (typically viral)
Genetic ( 20-30%) 
Inflammatory (infectious and noninfectious) 
Toxic (etOH most common, chemo agents) 
Metabolic
Neuromuscular
Major ischemic episode

Question 10

DCM Incidence / Prognosis

Answer 10

Prevalence is 36/100,000 population
3rd most common cause of heart failure
Middle age, men > women
Annual mortality: 12% !
Most frequent cause of heart transplantation
DCM accounts for approximately 10,000 deaths and 46,000 hospitalizations per year in the US
Complete recovery is rare

Question 11

DCM primary vs secondary

Answer 11

Primary (idiopathic) is a disease of unknown etiology that principally affects the myocardium leading to LV dilation and systolic dysfunction

Secondary causes include ischemia, alcoholic, peripartum, post-infectious, viral

Question 12

DCM causes2

Answer 12

Toxin induced:
       alcohol
       anthracycline
       cobalt
       catecholamine

Radiation

Infectious:
viral / HIV
parasitic

Metabolic:
        starvation
        thiamine deficiency
                  (beriberi)
        thyrotoxicosis

Sarcoidosis

Hemochromatosis

Peripartum/postpartum
cardiomyopathy

Genetic

Question 13

heart failure dx

Answer 13

done clinically, no definitive lab tests

Question 14

DCM: Symptoms/Signs of heart failure

Answer 14

Pulmonary congestion (left heart failure) dyspnea (rest, excertional, nocturnal), orthopnea

Systemic congestion (right heart failure) edema, nausea, abdominal pain, nocturne

Cardiac exam reveals -> S3, S4 and murmur of TR and/or MR

Low CO
Hypotension, tachycardia, tachypnea
Fatigue and weakness

Question 15

DCM: arrhythmia

Answer 15

Atrial fibrillation, conduction delays, complex PVC’s, sudden death

Question 16

DCM dx

Answer 16

CXR (enlarged heart, CHF)

EKG (tachycardia, A-V block, LBBB, NSSTT changes, PVC’s)

Echo (left ventricular dilation, global hypo kinesis, low EF)

Myocardial biopsy, rare

Cardiac catheterization (R/O CAD):
-if age >40, ischemic history, high risk profile, abnormal ECG

Question 17

DCM tx

Answer 17

(*lifestyle*)
Limit activity based on functional status 
Salt restriction 
Fluid restriction
Initiate medical therapy

Question 18

DCM med therapy

Answer 18

Diuretics (don’t increase longevity, improve symptoms, except loop-spironolactone)

the following improve mortality:
Beta Blockers, ACE/ARB

Hydralazine/nitrate combination if cannot tolerate ACE/ARB

Spironolactone if EF less than 35% with Class III/VI HF

Anticoagulation prn (EF less than 30%, history of LV thrombus or embolic events)

Implantable defibrillators if EF less than 35% on optimal medical therapy

Question 19

DCM more invasive tx

Answer 19

Cardiac transplantation:  
-DCM is most common indication for cardiac transplantation
Survival after transplant is 
80% one year
70% 5 years

Left Ventricular Reduction Procedures
LV-reshaping

Question 20

Hypertrophic Cardiomyopathy (HCM): Background

Answer 20

Genetic disease characterized by hypertrophy of the left ventricle with marked variable clinical manifestations morphologic and hemodynamic abnormalities

Prevalence 2:1000 (more than DCM?), younger pts
concentric or localized

Question 21

the heart on HCM

Answer 21

Small LV cavity, septal hypertrophy which can be asymmetric (ASH), systolic anterior motion of the mitral valve leaflet (SAM), +/- obstruction of left ventricular outflow with low stroke volume, but elevated EF

Question 22

HCM manifestations

Answer 22

Hyperdynamic systolic function
LVH and LV outflow obstruction
Diastolic dysfunction
Leads to pulmonary congestion

Question 23

HCM Dynamic LV outflow tract obstruction

Answer 23

Outflow tract gradient (>30 mm Hg), considered severe if >50 mm Hg (occurs in 25-30% of cases leading to name hypertrophic obstructive cardiomyopathy)

the original “classic” feature
we now know that it is absent in about half of the patients, and the severity of the obstruction varies greatly in those who do have it

Question 24

HCM complications

Answer 24

Dynamic LV outflow tract obstruction
Diastolic dysfunction
Impaired diastolic filling,  filling pressure
Myocardial ischemia
Mitral regurgitation
Arrhythmias

Question 25

HCM The causes of obstruction:

Answer 25

- narrowed left ventricular outflow tract due to hypertrophied interventricular septum - anterior displacement of the mitral valve leaflets during systole (SAM- systolic anterior motion of the mitral valve).

Question 26

why heart hypertrophies

Answer 26

heart tries to squeeze harder against restriction of outflow tract

Question 27

HCM: The severity of obstruction increases with:

Answer 27

- any maneuver that increases the force of contraction (exercise, positive inotropic agents) - any maneuver that decreases filling of the ventricle (dehydration, squatting-->standing, tachycardia, valsava)

Question 28

nomenclature HCM

Answer 28

Idiopathic Hypertrophic Subaortic Stenosis (IHSS) Hypertrophic Obstructive Cardiomyopathy (HOCM) Assymetric Septal Hypertrophy (ASH) Muscular Subaortic Stenosis (MSS) Hypertrophic Cardiomyopathy (WHO)

Question 29

most common presentation HCM

Answer 29

hypertrophy of inter ventricular septum

Question 30

HCM clinical presentation

Answer 30

Asymptomatic: Echo finding only ``` Symptomatic: Dyspnea in 90% Angina pectoris in 75% Fatigue, pre-syncope, syncope, risk of SCD Palpitation, PND, CHF, dizziness Atrial fibrillation, thromboembolism SUDDEN CARDIAC DEATH ```

Question 31

HCM Presentation (2)

Answer 31

* Ischemic chest pain*: secondary to increased myocardial oxygen demand - *not necessarily from CAD* (coronary aa not able to be filled due to hypertrophy) * SYNCOPE: LV outflow decreased * Exertional Syncope: increasing LV demand increases the level of obstruction

Question 32

Major Findings in HCM

Answer 32

* EJECTION MURMUR at LSB (systolic, sounds like mitral regurg) * EKG: LVH, abnormal Q’s * Valsalva: decreased LV volume * INCREASES the murmur * Standing: same as above *Squatting: Increased LV volume DECREASES the murmur (preload increases)

Question 33

HCM PE

Answer 33

systolic murmur best heard between the apex and left sternal border - increases in intensity with maneuvers that decrease preload (Valsalva maneuver) does not radiate to the carotid arteries

Question 34

HCM dx

Answer 34

CXR – mostly normal routine blood-work – unremarkable EKG – usually shows marked LVH *Echocardiogram* – is the diagnostic test of choice

Question 35

HCM – EKG Findings

Answer 35

``` Abnormal in 85-90% of cases LVH, Strain pattern Abnormal ST-T’s, giant T wave inversions Abnormal Q’s, Bundle Branch Block Left atrial enlargment Ventricular arrhthymias ```

Question 36

ECHO findings HCM (don't know in detail)

Answer 36

Left ventricular hypertrophy >1.3 cm (usually >1.5 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)

Question 37

HCM: MORE ECHO

Answer 37

LVH usually develops between 5-15 years of age in HCM A normal ECHO in a young child does not R/O the diagnosis **Serial ECHOs are recommended up to the age of 20 yr where there is a family history of HCM**

Question 38

HOCM

Answer 38

Clinical presentation from infancy to old age Variable clinical course 25 % of cohort achieve normal longevity Annual mortality 3% in referral centers probably closer to 1% for all patients Course may be punctuated by adverse clinical events: sudden cardiac death, embolic stroke, and consequences of heart failure Sustained V-Tach and V-Fib: most likely mechanism of syncope/ sudden death

Question 39

HOCM2

Answer 39

Risk of SCD higher in children, may be as high as 6% per year, majority have progressive hypertrophy Accounts for 36% of deaths in athletes younger than 35 years Clinical deterioration usually is slow Poor prognosis in males, young age of onset, family Hx of SCD, Hx of syncope, exercise induced hypotension (worst) Progression to DCM occurs in 10-15%

Question 40

Risk Factors for Sudden Cardiac Death: HCM

Answer 40

``` Young age (younger than 35 years) “Malignant” family history of sudden death Aborted sudden cardiac death Sustained VT or SVT Non-sustained VT on holter monitoring Atrial fibrillation Dilated left ventricle NYHA classes III or IV ```

Question 41

HCM management

Answer 41

-careful family history focused on sudden cardiac death -exercise testing to determine the presence of exercise-induced LVOT gradient -counseling regarding avoidance of strenuous exercise, avoidance of dehydration -all first-degree family members should be periodically screened with an echocardiogram – yearly between ages 12-18, every 5 years thereafter -consider genetic testing       

Question 42

HCM tx

Answer 42

Beta-adrenergic blockers (atenolol, metoprolol, sotalol, etc) Calcium channel blockers (amlodipine, diltiazem, etc) Anti-arrhythmics – Amiodarone, disopyramide Pacemakers (ICD) / Defibrillators (AICD) *Myomectomy (resection of septum) *Alcohol septal ablation (controlled MI through septal perforator perfusing basal septum) wall thinning decreases in LVOTO Transplantation

Question 43

HCM tx: B-blockers

Answer 43

- first-line therapy, clinical improvement >50% - 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 please NOTE that in hypertrophic cardiomyopathy, as opposed to dilated cardiomyopathy, we are using beta- blockers for their negative inotropic effect

Question 44

Septal | Myectomy

Answer 44

drip etOH next to septum, part of septum dies, reducing hypertrophy

Question 45

HCM Course of Illness

Answer 45

Usually, signs and symptoms increase with age *Leading cause of death: *sudden death in asymptomatic patients*

Question 46

Restrictive Cardiomyopathy (RCM): Background

Answer 46

*Rare in West: can affect both ventricles *Systolic function usually normal especially *early* in the disease process Small ventricular cavity size *Diastolic dysfunction* (can't fill) Elevated ventricular filling pressures LA is huge compared to LV; dilates in response to pressure

Question 47

RCM more background

Answer 47

Hallmark: abnormal diastolic function Rigid ventricular wall with impaired ventricular filling Bear some functional resemblance to constrictive pericarditis Importance lies in its differentiation from operable constrictive pericarditis Much less common then DCM or HCM outside the tropics, but frequent cause of death in Africa, India, South and Central America and Asia primarily because of the high incidence of endomyocardial fibrosis in those regions

Question 48

Classification of Restrictive Cardiomyopathy

Answer 48

Myocardial ``` *Non-infiltrative*: Idiopathic Familial Hypertrophic Scleroderma* ``` Infiltrative: Amyloid (most common) Sarcoid Gaucher’s Storage disease: Hemochromatosis* Glycogen storage disease Fabry’s ``` Endomyocardial: Endomyocardial fibrosis Hypereosinophilic syndrome Carcinoid Metastatic malignancy Radiation Chemotherapy toxicity ```

Question 49

Physiology of RCM

Answer 49

Rigid myocardium-->inc. diastolic ventricular pressure-->Venous congestion-->Jugular venous distention Hepatomegaly Ascites Rigid myocardium-->dec. ventricular filling-->dec. CO-->weakness, fatigue

Question 50

RCM: Clinical Manifestations

Answer 50

Symptoms of right and left heart failure Jugular Venous Pulse Echo-Doppler: Abnormal mitral inflow pattern Prominent E wave (rapid diastolic filling) Reduced deceleration time ( LA pressure)

Question 51

RCM (2): Presentation

Answer 51

* Again: CHF…S3 * EKG: can be normal or nonspecific * ECHO: to exclude other CHF causes * thick walls, reduced systolic fxn * Doppler study: can demonstrate diastolic abnormality * Right heart cath: commonly necessary - can help with endomyocardial biopsy

Question 52

Consider RCM in any patient with ??

Answer 52

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

Question 53

RCM dx tx??

Answer 53

possibly biopsy Treatment: CHF therapy (same as DCM) - No Clear medical therapy - Treat reversible causes - Transplant maybe only option

Question 54

slide 56

Answer 54

overview

Question 55

Arrhythmogenic RV Cardiomyopathy

Answer 55

Characterized by fibroadipose replacement of segments of the free wall of the right ventricle (fibrosis of RV causing significant ventricular arrhythmias) Familial and progressive Predominately found in young adults Cause of young adult sudden death ICD implantation in ALL patients who are symptomatic with arrhythmias ICD implantation vs anti-arrhythmic meds in asymptomatic patients

Question 56

FIRST AID

Answer 56

pg 296

Question 57

amyloidosis

Answer 57

pt has heart failure, EKG is low voltage: amyloid infiltrates decrease e-conduction

Question 58

FA: dilated cardiomyopathy

Answer 58

Most common cardiomyopathy (90% of cases). Often idiopathic or familial. ABCCCD: Other etiologies include chronic Alcohol abuse wet Beriberi Coxsackie B virus myocarditis chronic Cocaine use Chagas disease Doxorubicin toxicity, hemochromatosis, sarcoidosis, peripartum cardiomyopathy

Question 59

FA: DCM findings

Answer 59

HF, S3, systolic regurgitant murmur, dilated heart on echocardiogram, balloon appearance of heart on CXR

Question 60

FA: DCM tx

Answer 60

``` Na+ restriction ACE inhibitors β-blockers diuretics digoxin ICD (defibrillator) heart transplant ```

Question 61

FA: DCM pathophys

Answer 61

``` Systolic dysfunction ensues. Eccentric hypertrophy (sarcomeres added in series) ```

Question 62

case: HOCM

Answer 62

dx with ECHO: significant gradients outflow obstruction ``` what to do? defibrillator in heart no more competitive sports B-blocker stay hydrated genetic testing for rest of fam monitor every 6 mos may tolerate sx if want to continue competitive sports ```

Question 63

FA: HCM

Answer 63

60–70% of cases are familial, autosomal dominant (commonly a β-myosin heavy-chain mutation). Can be associated with Friedreich ataxia. Causes syncope during exercise and may lead to sudden death in young athletes due to ventricular arrhythmia.

Question 64

FA: HCM findings

Answer 64

S4, systolic murmur. May see mitral regurgitation due to impaired mitral valve closure.

Question 65

FA: HCM tx

Answer 65

cessation of high-intensity athletics use of β-blocker or non-dihydropyridine Ca2+ channel blockers (e.g., verapamil) ICD if patient is high risk.

Question 66

FA: HCM pathophys

Answer 66

Diastolic dysfunction ensues. Marked ventricular hypertrophy, often septal predominance. Myofibrillar disarray and fibrosis. Obstructive hypertrophic cardiomyopathy (subset)—asymmetric septal hypertrophy and systolic anterior motion of mitral valve Ž outflow obstruction Ž dyspnea, possible syncope.

Question 67

FA: RCM/infiltrative cardiomyopathy major causes

Answer 67

sarcoidosis, amyloidosis, postradiation fibrosis, endocardial fibroelastosis (thick fibroelastic tissue in endocardium of young children), Löffler syndrome:(endomyocardial fibrosis with a prominent eosinophilic infiltrate), and hemochromatosis (dilated cardiomyopathy can also occur).

Question 68

FA: RCM pathophys

Answer 68

Diastolic dysfunction ensues. Can have low- voltage ECG despite thick myocardium (especially amyloid)