Cardiomyopathies (HF pt 2)

Question 1

True or false: AHA classification is evolving, overlap

Answer 1

True

Question 2

Define primary and secondary CM

Answer 2

Primary CM – disease process primarily confined to the heart
Secondary CM – describes conditions in which cardiac involvement is part of systemic condition

Question 3

List 3 etiologies of secondary CM

Answer 3

Genetic
Hypertrophic cardiomyopathy is the most common primary CM
Mixed
Dilated CM – genetic or acquired, typically presents like HFrEF
Restrictive CM is less common, often associated with systemic disease
Acquired variants CM
Peripartum and stress induced

Question 4

True or false: Regardless of phenotype and etiologies, most common CM present with similar symptoms as HF: i.e., fatigue, DOE, orthopnea, PND, Dependent edema, pre-syncope, syncope, and/or cardiac ischemia (ACC stage C, NYHA class 2-4)

Answer 4

True

Question 5

How do you Dx cardiomyopathies?

Answer 5

Same as traditional HF:
1) History and exam
2) ECG and Echocardiogram

Question 6

Tx of CM may include what?

Answer 6

1) Appropriate HF GDMT
2) Appropriate activity restriction
3) Treating underlying issue (Sarcoidosis ….. Steroids)

Question 7

What testing is there for cardiomyopathies?

Answer 7

Genetic testing family members; an evolving screening practice

Question 8

“Common things happen commonly but are not really all that common” describes what?

Answer 8

Hypertrophic cardiomyopathy (HCM)

(most common cause of sudden cardiac death in young adults)

Question 9

Hypertrophic cardiomyopathy (HCM):
1) Is it inherited?
2) How common is it?

Answer 9

1) Inherited, autosomal dominant
-8 genes found associated with HCM
2) Asymptomatic prevalence in adults 1: 200 – 500
-Symptomatic prevalence undefined

Question 10

What is the most common cause of cardiac sudden death in young patients? What is the mortality rate?

Answer 10

HCM; 10% morality in children after diagnosed with HCM

Question 11

Hypertrophic cardiomyopathy (HCM):
1) Leads to LVH, especially of the ___________________.
2) Septal thickening predominates and may cause LV outflow tract ______________ (LVOTO) &/or MV ___________

Answer 11

1) ventricular septum
2) obstruction; distortion

Question 12

HCM:
Septal thickening predominates and may cause LV outflow tract obstruction (LVOTO) &/or MV distortion. This is called what?

Answer 12

Termed hypertrophic obstructive cardiomyopathy (HoCM)

Question 13

____% of patients with HCM have LVOTO

Answer 13

75%

Question 14

When do you have a high index of suspicion of HCM?

Answer 14

Consider in children and adults:
1) FHx HCM
2) Heart murmur
3) Abnormal EKG

Question 15

Hypertrophic cardiomyopathy:
1) When may it be found?
2) What Sx does it often present with?

Answer 15

1) Incidental finding after finding new heart murmur or abnormal EKG
2) Sx assoc. with exertion: Dyspnea, palpitations; Syncope; Chest pain

Question 16

HCM presentation:
1) Atypical CP is associated with what?
2) What is the worst way for HCM to first present?

Answer 16

1) Meal, dehydration, or exertion
2) Sudden cardiac death

Question 17

Detected cardiac abnormalities associated with HCM include what?

Answer 17

1) LVOTO
2) Mitral regurgitation
3) Diastolic dysfunction (HFpEF)
4) Myocardial ischemia
5) Arrhythmias – up to 50% have paroxysmal AF
6) Autonomic dysfunction

Question 18

Initial work up if you suspect HCM should include what?

Answer 18

1) 3 generation family history
2) Dynamic cardiac exam (squatting, valsalva)
3) 12 lead ECG
4) Echo or cardiac MR
5) Prolonged cardiac monitor (Zio patch, etc.)

Question 19

HCM: describe genetic counseling

Answer 19

1) Pretest genetic counseling:
-Multiple genes associated, multiple pathologic variants
-By provider experienced in cardiac geneti

Question 20

Describe an HCM assoc. murmur on exam

Answer 20

1) Pre-load dependent murmur
2) Murmur decreased with squatting (increased LV preload)
3) Murmur increased with standing or Valsalva (decreased LV preload) (opposite of Aortic Stenosis)

Question 21

What do Obstructive HCM murmur and aortic stenosis murmur have in common?

Answer 21

ULSB systolic murmur

Question 22

Describe what the following will do internally and how it will present if a pt has Obstructive HCM murmur:
1) Upright posture/Valsalva maneuver:
2) Squatting

Answer 22

1) Decreased LV volume (decreased preload and stroke volume)
-Increases outflow obstruction and murmur intensity
2) Increased LV preload
-Decreases subaortic obstruction and murmur intensity

Question 23

Describe what the following will do internally and how it will present if a pt has aortic stenosis:
1) Standing up/Valsalva maneuver
2) Squatting

Answer 23

1) Decrease LV volume (decreased preload and stoke volume)
-Decrease murmur intensity
2) Increased LV preload; increased murmur intensity

Question 24

Describe EKG changes in HCM.

Answer 24

1) LVH or normal:
-Axis deviation
-Voltage criteria
-Septal Q waves inf/lat
2) QRS LAD: + in Lead I; - in lead aVF
3) Voltage criteria LVH:
-Deep S in V1or 2 +
-Tall R is V5 or 6
= 35+ mm

Question 25

List some DDxs for LVH

Answer 25

Chronic HTN Athletic cardiac wall remodeling Aortic valve dysfunction Systemic disease Amyloidosis Sarcoidosis

Question 26

Describe Echocardiogram (TTE) in HCM

Answer 26

LV hypertrophy End diastolic LV wall thickness > 15 mm or > 13 mm in patient with known gene variant or + Fhx of HCM > 2 STD above mean for age in kids Diastolic dysfunction/HFpEF Mitral valve regurgitation

Question 27

True or false: HCM Tx is Team based at specialized centers

Answer 27

True

Question 28

Describe how to Tx asymptomatic HCM

Answer 28

Routine monitoring and imaging: 1) Repeat EGK, Echo, prolonged cardiac monitoring -Kids: q 1-2 years -Adults q3-5 years -All 1st degree family members of patients with HCM require the same monitoring/imaging --If family member dx in childhood – q 1-2 years, dx as adults – q 3-5 years

Question 29

HCM: 1) What is the first line Tx for symptomatic patients? Why? 2) What is the second line?

Answer 29

1) B blockers Decrease heart rate and LVOTO vs. bradycardia 2) Nondihydropyridine CCB as alternative but not add-on

Question 30

HCM Tx: Diuretics, ACEi/ARBs may increase __________ secondary to vasodilation and decreased afterload

Answer 30

LVOTO

Question 31

Echo shows NO LVOTO; how do you Tx?

Answer 31

Loop diuretic if volume overloaded If EF < 50% ….treat as HFrEF, avoid hypotension

Question 32

How do you Tx if If symptomatic despite BB or CCB?

Answer 32

May add on disopyramide (Norpace) or septal reduction therapy: a) Disopyramide must be combined with AV nodal blocking agent or may result in AF if used alone d) Septal reduction therapy by myectomy or alcohol ablation improves symptoms but not disease progression

Question 33

True or false: ICD reduce cardiac sudden death in those at risk

Answer 33

True

Question 34

Describe exercise in stable HCM

Answer 34

Moderate intensity safe & recommended Studies show similar burden of ventricular arrhythmias in patients engaged in competitive sport as those who are not Because risk of cardiac sudden death is similar at rest or during competitive sports, vigorous exercise and sports may be reasonable with shared decision making

Question 35

Describe occupation and HCM

Answer 35

CDL – allowed if no ICD or major risk factor for cardiac sudden death Commercial pilots – allowed if low risk HCM and treadmill test @ 85% max heart rate (MHR)

Question 36

Describe pregnancy and HCM

Answer 36

Generally safe in stable HCM 25% experience cardiac symptoms Sudden cardiac death during gestation and delivery rate BB, except Atenolol, considered safe Adverse outcomes ~ 3-4% whether vaginal delivery or C-section

Question 37

What is DCM and what causes it? When does it occur?

Answer 37

Insult leading to weakness of myocardial wall, thinning. and dilation Leading indication for heart transplant Can occur at any age, most common in 40’s and 50’s

Question 38

Describe the imaging of DCM

Answer 38

Symptoms suggestive of volume overload – edema, JVD, hepatic congestion, S3/S4, arrhythmias, and thromboembolic Diagnosis – most patients symptomatic at time of diagnosis ECG –non-specific Sinus tach and other arrhythmias CXR – cardiomegaly, pulmonary edema, pleural effusion Echo – dilated LV, HFrEF

Question 39

Describe Tx of DCM

Answer 39

GDMT based on HF stage (often HFrEF) BB ACEi or ARB Symptom control Diuretics Digoxin Consider OAC ICD – defibrillator with low EF%

Question 40

Restrictive cardiomyopathy: 1) Is it common? Explain 2) How is it defined? Explain

Answer 40

1) Least common of major CM – 2-5% of cases -Northern European men 2) By physiologic function rather than anatomic (HCM or DCM) -Myocardium infiltrated with abnormal tissue leading to fibrosis, wall stiffness, increased diastolic pressure, leading to reduced preload (diastolic dysfunction) – think HFpEF -Amyloidosis, sarcoidosis, XRT, and scleroderma among common etiologies

Question 41

Restrictive cardiomyopathy: 1) How does it present? 2) What if it's LHF?

Answer 41

1) Often symptoms of RHF: Ascites and peripheral edema JVD Pulmonary edema 2) LHF: Dyspnea, fatigue, PH, pulmonary edema

Question 42

Restrictive cardiomyopathy: 1) What will CXR show? 2) What abt ECG? 3) What abt echo?

Answer 42

1) Pulmonary vascular congestion with normal cardiac silhouette 2) Diffuse reduced voltage or prolonged PR interval (atrial enlargement) 3) Bi-atrial enlargement and right ventricular diastolic dysfunction (right HFpEF)

Question 43

How do you Tx restrictive cardiomyopathy?

Answer 43

1) Limited, focus on underlying process -Symptomatic – diuretics and aldosterone antagonist for overload, evaluate for AV block, pacer as indicated

Question 44

Acquired Cardiomyopathies: Peripartum CM 1) What is it and when does it present? 2) What is it assoc with? 3) What are the S/Sx? 4) What will an ECG show?

Answer 44

1) LV systolic dysfunction third trimester of pregnancy on into postpartum; usually presents first month PP 2) Associated with increased maternal age, black race, preeclampsia, HTN, peripartum CM prior pregnancy, and multiple gestations 3) S/S c/w HF – fatigue, edema, and dyspnea – DDX preeclampsia, PE 4) ECG – nonspecific

Question 45

Peripartum CM: What is the Tx? Do pts recover?

Answer 45

1) GDMT based on HF stage…avoid ACE/ARB, monitor for dehydration and uterine ischemia with diuretics 2) Most recover LV function

Question 46

Stress CM or Takotsubo CM (broken heart syndrome): 1) What is it? 2) Describe the epidemiology 3) What does EKG show?

Answer 46

1) Abrupt onset of LV dysfunction in response to severe emotional or physiologic stress 2) Post menopausal women - most commonly affected 1-2% of admission for ACS secondary angina & dyspnea 3) Typical ischemic changes Positive cardiac biomarkers…quick rise and fall

Question 47

Stress CM or Takotsubo CM (broken heart syndrome): 1) What will labs show? 2) What is the initial Tx? 3) How do you Tx stable patients? 4) Describe the course

Answer 47

1) may see elevated cardiac enzymes 2) same as ACS 3) Stable patients with ACE/ARB, BB, aldosterone antagonist, diuretics, AC if wall motion defects in apex 4) Symptoms and pathology usually resolves within a month and treatments can be withdrawn