Cardiomyopathies

Question 1

3 Main categories

Answer 1

1. Dilated (MC)
2. Hypertrophic (HT CM #1 cause sudden death athletes)
3. Restrictive

Question 2

Dilated pathophysiology

Answer 2

Dilated ventricles, no hypertrophy.
Systolic failure, EDV & ESV increase, LVEF decreases.
Dilation worsens due to increased volume blood.
May cause mitral/tricuspid regurgitation-worsens CO.
S/s due to back up of blood or decreased CO.

Question 3

Dilated causes

Answer 3

Alcohol
Beriberi
Coxsackie B/Chagas
Drugs
Pregnancy
Idiopathic (50%)/Infection
Genetic

Interstitial/Endocardial fibrosis

Question 4

Ischemic dilated CM

Answer 4

MCC of dilated CM.
MCC of systolic HF.
LVEF<35-40% from CAD.
Tx: same + CAD tx

Question 5

HTN dilated CM

Answer 5

Concentric LVH–Dilation–Eccentric remodeling

With Systolic dysfunction

Question 6

Alcoholic dilated CM

Answer 6

Mechanism: unclear, Acetaldehyde–myocardial depression
>90g (7-8 drinks) for >5 years
Increased QTc due to decreased Mg/K
Tx: Abstinence, will improve outcome.

Question 7

Peripartum dilated CM

Answer 7

When? Late preg (after 36 wks) & w/in 5 mo of birth
Risk factors: AA, >30yo, Cocaine, Multiple fetus, Ecclampsia/Pre.
Tx: transplant 1/3. If LVEF >50% 6mo., can wean off tx.
Mortality: 10% in 2 years.

Question 8

Takotsubo dilated CM

Answer 8

Transient decreased LVEF
Systolic apical ballooning on echo (hyperkinesis in upper walls, apex balloons)
Sx: CP, Trop increased, ST elevation. NO CAD.
Cause: stress, catelcholamines, post-menopausal
Recovery: 1-4 weeks, increased risk for arrhythmia/shock.
Recurrence: beta-blockers

Question 9

Dilated CM clinical presentation

Answer 9

Age 20-60, but any age
HF sx: dyspnea, JVD, peripheral edema, congestive cough with white sputum.
Misdx: Viral URI in younger population.
Hx: Breast cancer chemo, thyroid, drug use, family hx of sudden cardiac death.

Question 10

Dilated CM PE signs

Answer 10

1. HF signs (Hepatojug reflux/JVD/crackles/clubbing)
2. PMI shifted left
3. S3 gallop

Question 11

Dilated CM Dx test

Answer 11

1. Labs: CBC (anemia), CMP (hyponatremia=poor prognosis), Thyroid, Biomarkers/BNP, Urine drug if suspicious
2. CXR: cardiomegaly, Kerley B, R pleural effusion, congestion
3. EKG: LVH, arrhythmias, conduction delay
4. Cath: R/O ischemic cause
5. Biopsy: limited use, for amyloid/sarcoid

Question 12

Dilated CM medical tx

Answer 12

1. ACE/ARB + Diuretic/Aldosterone ant. + Beta-blocker
2. ARNI
3. Inotropes for end-stage
4. Anticoag for AFib
5. Nitrates for sx relief
6. Revascularize if ischemic

Question 13

Dilated CM surgical tx

Answer 13

1. LVAD
2. CRT (resynchronization): LVEF<35, BBB, pers HF sx
3. AICD (cardiovert/defib): At risk for ventricular arrhythmias
4. Transplant: Dilated CM=45% of transplants in US

Question 14

Hypertrophic CM etiology

Answer 14

1. Familial HCM (50-60%)
Genetic, Autosomal dominant mutation of sarcomere
2. Septum abnormalities
3. Subendocardial ischemia
4. Abnormal SNS 
5. Ab thick intramural coronary aa.

Question 15

Hypertrophic CM epidemiology

Answer 15

0.5% population (1/200 adults)
M>F, F present younger
MC in 3rd decade

Question 16

Obstructive HCM physiology

Answer 16

LVOTO: left ventricular outflow tract obstruction
SAM: systolic anterior motion of mitral valve toward septum. Can lead to mitral regurg.
Worse: with decreased preload/afterload & increase inotropy due to ventricle not being as large
Better: with increased preload/afterload. Ventricle more open.

Question 17

LVOTO murmur & maneuvers/factors

Answer 17

Systolic crescendo-decrescendo
Between apex & LSB
1. INCREASE with DECREASE preload/afterload or INCREASE contractility
Valsalva, Standing, Dehydration, Dihydropyridines, Fever, Amyl Nitrate, Dobutamine
2. DECREASE with INCREASE preload/afterload
Squatting, Hand grip, Supine w/legs up, Nondihydro, Beta blocker.

Question 18

Nonobstructive HCM pathophysiology

Answer 18

Decreased ventricle size, Decreased diastolic filling=Diastolic failure
Which decreases the outflow/CO

Question 19

HCM risks

Answer 19

SVT/VT

SCD (sudden cardiac death)-risk stratify to see if they need an ICD.

Question 20

Risk factors for SCD

Answer 20

1. Hx of syncope
2. Family hx of sudden death
3. Abnormal BP response to exercise
4. LV wall >30mm
5. Vtach
6. <30 y.o.
7. Hx of survived cardiac arrest

Question 21

HCM progression

Answer 21

Most asx. Children more sx early on (higher risk SCD)

10-15% progress to DCM-Slow clinically.

Question 22

HCM sx

Answer 22

Syncope
Palpitations
Orthopnea/PND/Dyspnea
Dizzy
Angina

Question 23

HCM PE findings

Answer 23

1. S4
2. Double apical pulse (due to LA forceful contraction)
3. Murmur
4. S2 splitting in severe LVOTO

Question 24

HCM Dx

Answer 24

1. Dx= Transthoracic Echo
2. Exercise test for BP response
3. Ambulatory EKG for arrhythmias

Question 25

HCM management (nonpharm)

Answer 25

1. Risk stratify for ICD 2. Limit physical activity 3. Avoid volume depletion (decreases preload)

Question 26

HCM medical tx

Answer 26

1. Beta-blocker (negative inotropy, increase fill time) 2. Nondihydro CCB (MUST D/C BB) Use if BB not effective. Careful for: vasodilation, sinus arrest, pulmonary edema, AV node block 3. Disopyramide (1a anti-arrhythmic) Use in conjunction with BB or CCB if sx on monotherapy. Negative inotrope (Increase QTc, AV node, anticholinegric) Dont use for BPH.

Question 27

HCM surgical options

Answer 27

1. Septal myectomy. Can repair MV at same time. | 2. Alcohol septal ablation. Local MI at SAM area.

Question 28

Restrictive CM Etiology

Answer 28

1. Primary=idiopathic (Endomyocardial fibrosis/Loeffler) 2. Secondary=other ds causes Amyloidosis (#1 US cause) Sarcoidosis, Scleroderma, Metastasis, Radiation, Drug (chloroquine), Hematochromatosis

Question 29

Restrictive CM Epidemiology

Answer 29

F=M, Children worse prognosis Genetic link. Loeffler=worldwide Amyloidosis=US

Question 30

Restrictive CM pathophysiology

Answer 30

1. Infiltration decreases ventricular compliance 2. Increase ventricular pressure 3. Reduced V filling, decreases CO 4. Pressure backs up into atria=Bi-atrial enlargement

Question 31

Restrictive CM clinical presentation

Answer 31

Late, with dyspnea, orthopnea, fatigue Palpitations-often causes AFib Thromboembolic events Orthostatic hypotension/syncope (Amyloidosis with Neuropathy)

Question 32

Restrictive CM PE findings

Answer 32

1. Kussmauls sign 2. R HF: JVP, pleural effusion,etc 3. Cardiac cachexia 4. Amyloidosis: bruising, periorbital purpura, macroglossia

Question 33

Restrictive CM Dx imaging

Answer 33

1. Echo: Infiltrative=LVH, septal/valve thickening 2. Cardiac MRI: LGE pattern (global subendocardial late gad. enhancement) (May do Cath/Biopsy)

Question 34

Restrictive CM lab tests

Answer 34

1. CBC w/smear-Eosinophilia 2. CMP-kidney/liver fxn 3. Iron/Ferritin/TIBC 4. BNP (NOT increased in pericarditis)

Question 35

Restrictive CM EKG changes

Answer 35

1. Low voltage QRS 2. AFib common 3. May see ST depression

Question 36

Restrictive CM medical tx

Answer 36

1. Beta blocker 2. Nondihydro CCB (both increase fill time) 3. Diuretic (decrease preload but careful!) 4. Anticoag AFib Amyloidosis: NO ACE/ARB, hypotension due to neuropathy

Question 37

Restrictive CM management non pharm

Answer 37

1. Amyloidosis=chemo, Sarcoidosis=corticosteroids, Hematochromatosis=phleb/chelation 2. Pacemaker if AV/SA node fibrosis causing heart block 3. LVAD/transplant