Cardiomyopathies

Question 1

What is cardiomyopathy

Answer 1

Myocardial disorder in which THE HEART MUSCLE IS STRUCTURALLY AND FUNCTIONALLY ABNORMAL in the absence of coronary artery disease, hypertension, valvular disease, and congenital heart disease

Question 2

What are the three main types of CM

Answer 2

Hypertrophic
Dilated
Restrictive

Question 3

Dilated cardiomyopathy pathophysiology DCM

Answer 3

Symmetric left ventricular LV dilation
Impaired LV systolic function
Can involve the right ventricle RV

Not caused by CAD, valvular disease or HTN

Question 4

DCM presentation both symptoms and possible PE findings

Answer 4

Symptoms: 
EXERTIONAL INTOLERANCE WITH SOB, FATIQUE
Chest pain
Palpitations, presyncope/syncope
Peripheral edema
Abdominal pain

Posible PE findings:
Mitral/tricuspid regurg murmur 
S3 gallop
Increased jugular venous pressure
Basal crackles

Question 5

Etiologies of DCM

Answer 5

IDIOPATHIC
Familial
Infectious
Peripartum
Alcoholism
Drugs
[tachycardia-mediated
Endocrine dysfunction
Nutritional deficiencies

Question 6

What is the most common etiology of DCM

Answer 6

Idiopathic DCM up to 50% (no identifiable cause

Primary indication for cardiac transplantation

Question 7

Familial DCM criteria and important points

Answer 7

(+) FH of DCM in 2+ relatives

Seen >= 50% of patients with idiopathic DCM

Cardiac protein antibodies present in 30%

Question 8

Infectious DCM important points

Answer 8

Myocarditis= inflammatory process 2 degree to infectious or non-infectious causes

Viral-most common in US
Parasitic (changes DZ) most common cause of DCM in Latin America
Present w/ viral sxs and/or heart failure sxs
Endomyocardial biopsy is GOLD STANDARD for diagnosis

Question 9

Peripartum DCM risk factors and presentation

Answer 9

>30 African descent
Multiparous
H/o preeclampsia, eclampsia, postpartum HTN
Maternal cocaine abuse
>4wks use of oral tocolytic 

Presentation
Generally 36 wks of gestation - 5 mos. post-partum
Dyspnea, orthopnea, PND, pedal edema, cough, hemoptisis

*diagnosis: Inc. BNP, Echo= EF <45

Question 10

Alcoholic DCM risk factors, presentation, prognosis

Answer 10

Risk factors: 6 drink/day for 5-10 years

Presentation: HF symptoms, signs of long-term EtOH use

Prognosis: abstinence can lead to significant improvement in heart failure
If continue EtOH = poor prognosis, mortality 50% at 3-6 years

Question 11

Cocaine-related DCM

Answer 11

Toxic effects on myocardium

Causes myocarditis

Question 12

Chemotherapy-induced DCM

Answer 12

Anthracyclines
-including adriamycin (doxarubicin) -> breast CA, sarcoma and lymphoma

Early detection improves outcomes
Can present with HF symptoms

*obtain baseline echo to measure EF and continue to monitor

Question 13

Tachycardia mediated DCM cause and treatment

Answer 13

Caused by sustained rapid ventricular rates (130-200)
May follow any type of chronic cardiac arrhythmia
Treatment of tachycardia/arrhythmia reverses myocardial dysfunction

Question 14

Endocrine dysfunction DCM

Answer 14

Diabetes
-initially myocardial fibrosis and diastolic dysfunction(restrictive CM) later stages systolic dysfunction

Thyroid dysfunction
-thyroid hormone can alter preload, afterload, heart rate and contractility

Pheochromocytoma
-adrenal tumor secreting catecholamines

*cardiac dysfunction usually can be reversed by correction of endocrine disorder

Question 15

Nutritional deficiencies DCM

Answer 15

Thiamine B1 deficiency
-consider in alcoholic or malnourished pt (diarrhea, dialysis, poor intake)

Carnitine deficiency
-impairs oxidation of fatty acids, resulting in lipid accumulation in myocete cytomplasm

*cardiac dysfunction usually can be reversed by correction of nutritional deficiency

Question 16

DCM diagnostics

Answer 16

EKG:
-non-specific changes -> sinus tachycardia, left bundle branch block, atrial/ventricular arrhythmia

CXR:
-cardiomegaly (globular heart), pleural effusions

Labs:
-BNP, TSH, CBC, CMP, (+/-) endomyocardial biopsy

ECHO***

• dilated ventricle, reduced LV systolic function
• exclude unsuspected valve disease

Question 17

DCM management

Answer 17

Treat or remove underlying cause

• d/c alcohol or cocaine use
• evaluate chemo dosing

Lifestyle modification
Close f/u

Question 18

DCM med management

Answer 18

ACE inhibitors **1st line**
-reduce afterload by vasodilation, BP reduction 
Diuretics
-used for volume overload/reduce preload
B-blockers
-decrease oxygen demand
Digoxin
(+) inotrope
Failure to respond to first line treatments
Used in afib, fatigue, dyspnea

Question 19

DCM management

Answer 19

Antiarrhythmics
-amiodarone if afib

Anticoagulation
-if afib, EF <30%, h/o thromboemboli, presence of mural thrombi (on wall of ventricle)

IMPLANTED CARDIOVERTER DEFIBRILLATOR
CARDIAC TRANSPLANTATION

Question 20

Hypertrophic cardiomyopathy pathophysiology

Answer 20

LEFT VENTRICULAR HYPERTROPHY (LVH) IN THE ABSENCE OF A CAUSE

• diastolic filling abnormalities
• small LV cavity

Leading cause of sudden cardiac death (SCD) in young persons

Question 21

HCM etiology

Answer 21

Familial

Question 22

What is obstructive HCM

Answer 22

Factors WORSENING left ventricular outflow tract (LVOT) obstruction:

Tachycardia
Hypovolemia
Standing
Valsalva
\+ inotropes
Diuretics
Vasodilators 

My words: mitral valve is close to septum and gets stuck. This causes regurg, and obstructed aortic outflow

Can be asymptomatic until exertion

Question 23

HCM presentation

Answer 23

Asymptomatic
Found on echo
Death

Symptomatic
Dyspnea on exertion
Exertional angina 
Fatigue
Pre-syncope, syncope
Palpitations

Question 24

HCM presentation continued

Answer 24

POTENTIAL PE FINDINGS W/ LVOT OBSTRUCTION
Brisk, bifid carotid pulse
Audible S4
Crescendo-decrescendo systolic ejection murmur

INCREASES with valsalva and standing
DECREASES with squat and isometric handgrip

Question 25

HCM diagnostics

Answer 25

EKG - LV hypertrophy w/ strain - increased incidence of both supraventricular and ventricular arrhythmias * ECHO* - INCREASED LV WALL THICKNESS >= 15mm is diagnostic - asymmetric septal hypertrophy - systolic anterior motion of mitral valve Stress/exercise echo: -risk stratification assess LVOT

Question 26

Sudden cardiac death risk factors

Answer 26

Prior sustained ventricular tachycardia (VT) Non-sustained VT Young age <30 years Family history of sudden death due to HCM Massive LVH (wall thickness >= 30mm) Unexplainable syncope, especially in young patients Brady arrhythmias

Question 27

HCM and physical activity restriction

Answer 27

Avoid most competitive sports | Avoid high-intensity sports

Question 28

HCM management

Answer 28

Asymptomatic: ->no prophylactic therapy Symptomatic: BETA BLOCKERS BB or NON-DIHYDROPYRIDINE CALCIUM CHANNEL BLOCKERS (Nd-CCB) - (-) inotrope -may improve chest pain and dyspnea Diuretics w/ caution if significant LVOT obstructin and symptoms Manage arrhythmias ICD implantation if high risk for SCD, sustained ventricular tachyarrhythmia

Question 29

Surgery for HCM

Answer 29

Indicated for SYMPTOMATIC LVOT obstruction w/ advanced heart failure refractory to medical therapy Surgical septal myectomy EtOH ablation Mitral valve surgery (mitral valve replacement or plication)

Question 30

Restrictive cardiomyopathy RCM pathophysiology

Answer 30

``` Non-dilated, rigid ventricles Diastolic dysfunction Restrictive filling Preserved systolic function HFpEF R and L atrial enlargement ```

Question 31

RCM most common Etiology

Answer 31

Infiltrative - Amyloidosis -> most common - sarcoidosis Storage diseases -hemochromatosis Glycogen storage disease Other Idiopathic Scleroderma Fibrosis 2nd chemotherapy, radiation

Question 32

RCM presentation symptoms and PE

Answer 32

Symptoms: Right > left heart failure Edema, abdominal discomfort, ascites Angina, syncope, dyspnea PE *S3 gallop not heard in constrictive pericarditis Prominent JVP +/- kussmaul’s sign (distensión of jugular vein during inspiration) Possible MR/TR murmur (regurg)

Question 33

Cardiac amyloidosis presentation and diagnostic studies

Answer 33

Presentation: Elevated JVP Hepatomegaly, ascites Periorbital purpura with heart failure is pathognomonic (late finding) Diagnostic studies: Echo -> inc left ventricular wall thickness with diastolic dysfunction, later right ventricular diastolic dysfunction develops Endomyocardial biopsy —> definitive

Question 34

RCM diagnostic

Answer 34

EKG- non-specific findings *ECHO* Bi0atrial enlargement Normal to small ventricular cavity (amyloid may result in wall thickness) Nml Ef, abn filling CARDIAC MRI Chamber size, wall thickness, presence/absence of infiltration, inflammation, fibrosis Endomyocardial biopsy Diagnostic

Question 35

RCM Managment

Answer 35

``` TREAT UNDERLYING CAUSE LOW DOSE LOOP DIURETICS - decrease systemic and pulmonary congestion Heart transplant consideration Poor prognosis ```

Question 36

What is takotsubo cardiomyopathy (TCM)

Answer 36

“Ballooning” TRANSIENT LV systolic and diastolic dysfunction in the absence of attributable coronary artery disease (CAD) - preceded by emotional or physical trigger - typically occurs in postmenopausal women

Question 37

TCM presentation

Answer 37

-abrupt onset Present like acute coronary syndrome SUBSTERNAL CHEST PAIN, dyspnea, syncope Symptoms of heart failure

Question 38

TCM diagnostics

Answer 38

Labs: increased troponin, BNP EKG: St-segment elevation, deep anterior T-wave inversions (which gradually resolve) CXR: normal or pulmonary congestion Echo: decrease EF, possible LVOT obstruction, eval for thrombus Cardiac MRI: wall motion abnormalities

Question 39

TCM managment

Answer 39

IMMEDIATE: similar to any acute MI, including cath lab Resolve trigger At discharge -ASA, BB, and ACE-I until the LV fully recovers -(+) thrombus = anticoagulation for minimum of 3 months Long-term: depends on whether LV dysfunction persists Prognosis: most recover systolic function w/in 4 wks. May have variable EKG changes for several months following event

Question 40

Diagnostic criteria Takotsubo cardiomyopathy

Answer 40

1. There is transient hypokinesis, dyskinesia, or alpines is of the left ventricle 2. No obstructive coronary artery disease (CAD) or angiographic evidence of an acute plaque rupture 3. New EKG abnormalities (ST-segment elevation and/or T-wave inversion) or modestly elevated levels of cardiac troponin 4. Absence of pheochromocytoma or myocarditis