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Flashcards in Cardiomyopathies Deck (37)
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1
Q

3 Main categories

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

Dilated pathophysiology

A

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.

3
Q

Dilated causes

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

Interstitial/Endocardial fibrosis

4
Q

Ischemic dilated CM

A

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

5
Q

HTN dilated CM

A

Concentric LVH–Dilation–Eccentric remodeling

With Systolic dysfunction

6
Q

Alcoholic dilated CM

A

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

7
Q

Peripartum dilated CM

A

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.

8
Q

Takotsubo dilated CM

A

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

9
Q

Dilated CM clinical presentation

A

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.

10
Q

Dilated CM PE signs

A
  1. HF signs (Hepatojug reflux/JVD/crackles/clubbing)
  2. PMI shifted left
  3. S3 gallop
11
Q

Dilated CM Dx test

A
  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
12
Q

Dilated CM medical tx

A
  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
13
Q

Dilated CM surgical tx

A
  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
14
Q

Hypertrophic CM etiology

A
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.
15
Q

Hypertrophic CM epidemiology

A

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

16
Q

Obstructive HCM physiology

A

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.

17
Q

LVOTO murmur & maneuvers/factors

A

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.

18
Q

Nonobstructive HCM pathophysiology

A

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

19
Q

HCM risks

A

SVT/VT

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

20
Q

Risk factors for SCD

A
  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
21
Q

HCM progression

A

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

10-15% progress to DCM-Slow clinically.

22
Q

HCM sx

A
Syncope
Palpitations
Orthopnea/PND/Dyspnea
Dizzy
Angina
23
Q

HCM PE findings

A
  1. S4
  2. Double apical pulse (due to LA forceful contraction)
  3. Murmur
  4. S2 splitting in severe LVOTO
24
Q

HCM Dx

A
  1. Dx= Transthoracic Echo
  2. Exercise test for BP response
  3. Ambulatory EKG for arrhythmias
25
Q

HCM management (nonpharm)

A
  1. Risk stratify for ICD
  2. Limit physical activity
  3. Avoid volume depletion (decreases preload)
26
Q

HCM medical tx

A
  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.
27
Q

HCM surgical options

A
  1. Septal myectomy. Can repair MV at same time.

2. Alcohol septal ablation. Local MI at SAM area.

28
Q

Restrictive CM Etiology

A
  1. Primary=idiopathic (Endomyocardial fibrosis/Loeffler)
  2. Secondary=other ds causes
    Amyloidosis (#1 US cause)
    Sarcoidosis, Scleroderma, Metastasis, Radiation, Drug (chloroquine), Hematochromatosis
29
Q

Restrictive CM Epidemiology

A

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

30
Q

Restrictive CM pathophysiology

A
  1. Infiltration decreases ventricular compliance
  2. Increase ventricular pressure
  3. Reduced V filling, decreases CO
  4. Pressure backs up into atria=Bi-atrial enlargement
31
Q

Restrictive CM clinical presentation

A

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

32
Q

Restrictive CM PE findings

A
  1. Kussmauls sign
  2. R HF: JVP, pleural effusion,etc
  3. Cardiac cachexia
  4. Amyloidosis: bruising, periorbital purpura, macroglossia
33
Q

Restrictive CM Dx imaging

A
  1. Echo:
    Infiltrative=LVH, septal/valve thickening
  2. Cardiac MRI:
    LGE pattern (global subendocardial late gad. enhancement)
    (May do Cath/Biopsy)
34
Q

Restrictive CM lab tests

A
  1. CBC w/smear-Eosinophilia
  2. CMP-kidney/liver fxn
  3. Iron/Ferritin/TIBC
  4. BNP (NOT increased in pericarditis)
35
Q

Restrictive CM EKG changes

A
  1. Low voltage QRS
  2. AFib common
  3. May see ST depression
36
Q

Restrictive CM medical tx

A
  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

37
Q

Restrictive CM management non pharm

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