Cardiomyopathies Flashcards

1
Q

How common is cardiomyopathy in US?

A
  • 3rd most common form of heart disease
  • 2nd most common cause of adolescent heart disease
  • pt will have CHF (usually presents at late stage)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

CXR finding of cardiomyopathy?

A
  • cardiomegaly
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

How are cardiomyopathies classified?

A
  • primary: due to idiopathic process effecting the myocardium
  • secondary: related to specific heart muscle disease
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Functional classification of cardiomyopathies?

A
  • dilated:( congestive, DCM, IDC) - ventricular enlargement and systolic dysfxn
    (alcohol, postpartum/peripartum, viral, genetic)
  • hypertrophic: (IHSS, HCM, HOCM) - inapprop. myocardial hypertrophy in absence of HTN or aortic stenosis
  • restrictive: infiltrative (amyloidosis), abnormal filling and diastolic function, won’t have cardiomegaly
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Definition of IDC?

A
  • disease of unknown etiology that principally affects the myocardium
  • LV dilation and systolic dysfunction
  • pathology: increased heart size and wt, ventricular dilation, normal wall thickness, heart dysfunction out of portion to fibrosis
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Dilated cardiomyopathy etiologies? pathophys?

A
  • ventricles stretched and become flabby and myocardium deteriorates
  • cause often unknown (autoimmune)
  • drug toxicty (alcohol, cocaine, catecholamines, chemotherapeutic agents), hypothyroidism, inflammation of the heart in some cases - CHF
  • hearts attempt to work harder results in increasing levels of Ca in cardiac cells, this activates Ca sensitive enzyme initiating cascade which switches on genest that cause heart enlargement
  • because ventricular contractility is impaired, CO is poor and condition progressive worsens
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Most common etiology of DCM, what groups affected the most?

A
  • systolic dysfxn and pump failure with low cardiac output
  • 50% idiopathic
  • african americans and males have 2.5x increased risk
  • most common age of dx: 20-50 years
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

characteristics of DCMs, what is seen on EKG and echo

A
  • impaired systolic function leads to cHF
  • dilation of one or both ventricles
  • often idiopathic
  • ethanol, chemotherapy
  • peripartum
  • ekg: nonspecific ST-T wave changes
  • echo: show ventricular dilation
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

How tod tell difference b/t dilated cardiomyopathy and dilated left ventricle due to severe CAD?

A
  • usually post MI: local dysfunction
  • dilated myopathy: global dysfxn
    seen on echo!!
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Clinical presentations of idiopathic DCMs?

A
  • HF sxs: 75%
  • anginal CP: 8-20%
  • emboli: 1-4%
  • syncope: less than 1%
  • sudden cardiac death and arrhythmias
  • hear systolic regurg murmur, rales and edema
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Dx tests presentation of DCMs?

A
  • CXR: enlarged heart, biventricular enlargement and pulm vascular congestion (kerley B lines)
  • EKG: LVH, LAE, Q waves, poor R wave progression, and afib
  • echo: confrms dx, ventricular enlargement, increased systolic and diastolic volumes, decreased EF
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Management of DCM?

A
  • limit activity based on fxnl status
  • salt restriction
  • fluid restricition
  • initiate medical therapy:
    ACEI, diuretics
    digoxin
    hydralazine/nitrate combo
    anticoag
    anti-arrhythmicas
  • consider transplant: tx with meds for 6 months to see if progress: echo
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Goals of DCM therapy? diseases that can be tx?

A
  • tx congestive sxs: diuresis
  • ID diseases that can be tx:
    CA
    thyroid disease
    hemochromatosis
    sarcoidosis
    infections
    ETOH
    drugs (chemo: doxirubicin)
    peripartum
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Alchohol induced cardiomyopathy is how common? why does it occur, can it be tx?

A
  • 3-4% of DCM
  • more common in men
  • etiology: direct toxic effects of ETOH, malnutrition
  • maybe a genetic predisposition
  • IT is reversible!!!! - stop drinking
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Workup of pt with dilated cardiomyopathy?

A
  • hx focused on HF
  • lab eval: EKG, CXR, echo and coronary angiography (CAD?)
  • if echo shows dilated ventricles - and LVEF is less than 40% and no severe disease -
  • if rapidly progressive sxs overe one month or new ventricular tachycardia or conduction abnorm and or suspected cause of myocarditis - get bx
  • if not: tx with conventional HF meds - if no improvement in 1 week - consider bx
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

What is peripartum cardiomyopathy, RFs?

A
  • ventricular dysfxn developing in last month before delivery to 5 months postpartum
  • rare
  • RFs:
    advanced maternal age, African American race, multifetal pregnancies, preeclampsia, gestational HTN
  • cause is uncertain
17
Q

Signs and sxs of peripartum cardiomyopathy?

A
  • resemble those of dilated cardiomyopathy
  • often sxs are ignored b/c of dyspnea, swelling and fatigue are normal sxs at end of pregnancy
    (have to watch wt closely)
18
Q

Dx, and Tx of peripartum cardiomyopathy?

A
  • dx: echo, EKG, and other tests of cardiac fxn

- tx: same as for dilated cardiomyopathy: diuretics, ACEI, anticoag

19
Q

3 possible outcomes of peripartum cardiomyopathy?

A
  • 1/3 heart returns to normal in 6 months
  • 1/3 cardiomegaly persists and outcome is poor
  • 1/3 heart returns to normal on max lifelong medical therapy (diuretics, ACEI)
20
Q

Describe hypertrophic cardiomyopathy?

A
  • inappropriate, asymmetric myocardial hypertrophy
  • septum is usual site for hypertrophy
  • cells will have bizarre appearance (sarcomeres are crossing the wrong way)
  • familial (auto dominant) and sporadic forms
  • hypertrophic cardiomyopathy with obstruction
21
Q

characteristics of HCM?

A
  • pathological changes are of muscular hypertrophy assoc with fibrosis
  • hypertrophied and non-dilated LV in absence of another disease, small LV cavity, uncommon disease
  • abnormal compliance - impaired diastolic relaxation and filling - diastolic disorder
22
Q

Genetic abnormality hypothesis of HCM?

A
  • hypertrophy develops as compensatory response to sarcomeric dysfxn
  • normal myocytes react to excessice mechanical loads with altered patterns of gene expression and release of autocrine growth factors
  • in HCM: normal systolic pressures sensed by abnorm myocyte as excessive load, intiating further hypertrophy
  • in short term: increased LV thickness, and small cavity size decreases wall tension for systolic contraction
23
Q

Pathophys of HCM?

A
  • systole: dynamic outflow tract gradient
  • diastole: impaired diastolic filling, increased filling pressure
  • Myocardial ischemia: increase muscle mass, and O2 demand, systolic compression of arteries
24
Q

Clinical features of HCM?

A
  • sxs severity progresses with age
  • HTN hx
  • DOE most common initial sx
  • angina - like CP, palpitations and syncope may also be present
  • syncope
  • sudden death - no sxs
25
Q

What will you find on PE of HCM?

A
  • apex will be localized
  • palpable S4 (stiff noncompliant ventricle)
  • harsh systolic ejection murmur across precordium - apex and heart base
  • often mitral regurg murmur
26
Q

HCM findings on Dx tests?

A
  • doppler echo: LVH, systolic anterior motion of anterior mitral leaflet, asymmetric septal thickening (Most impt dx test)
  • EKG: abnorm Q waves (Deep and narrow), LVH (30%)

CXR: usually normal (small cavity)

27
Q

Management and screening of HCM?

A
  • all first degree relatives: screening, echo/genetic counseling
  • avoid competitive athletic/discourage rigorous exercise
  • holterx48 hrs (sudden death comes from arrhythmias, afib to vtach)
28
Q

Tx of HCM?

A
  • maneuvers that decrease end diastolic volume (decrease venous return and afterload, and increase contractility) - valsalva and standing,
    vasodilators (nitrates)
    inotropes
    diuretics (only if volume overload)
  • BBs for longer diastolic filling time (slows HR)
29
Q

Options for pts that don’t respond to medical therapy?

A
  • surgery (myotomy/myectomy) +/- MVR
  • ICD (prevents sudden death)
  • DDD pacemaker (decreases gradient)
  • NSRT (non surgical septal reduction therapy - alcohol septal ablation - decrease gradient, first septal artery occluded with balloon cath and ETOH is injected distally)
30
Q

Recommendations for athletic activity in HCM?

A
  • avoid most competitive sports (whether or not sxs and or outflow gradient are present)
  • low risk older pts (older than 30) may participate in athletic activity if all of following are absent:
  • vtach on holter monitor
  • family hx of sudden death due to HCM
  • hx of syncope or episode impaired conscicousness
  • severe hemodynamic abnorm, gradient of equal or greater than 50 mmHg
  • exercise induced Hypotension
  • mod or severe MR
  • enlarged LA (greater than 50 mm)
  • paroxysmal afib
  • abnorm myocardial perfusion
31
Q

Describe restrictive cardiomyopathy?

A
  • least common of 3 major categories
  • diastolic dysfxn: ventricular walls are excessively rigig and impede ventricular filling
  • frequently accompanied by small degree of depressed contractility and EF
  • myocardial fibrosis, hypertrophy, or infiltration due to variety causes, mostly idiopathic
  • atria frequently become enlarged after chronic exposure to high filling pressure
32
Q

Common causes of restricitive cardiomyopathy?

A
  • deposition of abnormal substances in myocardium
  • infiltrative disease: abnormal substance is largely b/t myocytes: amyloidosis, sarcoidosis
  • endomyocardial: fibrosis or radiation
33
Q

Clinical features of restrictive cardiomyopathy?

A
  • exercise intolerance and dyspnea, dependent edema, ascites, anasarca, enlarged, tender, and often pulsatile liver (R sided sxs)
  • thromboembolic complications: 1/3 of pts
  • JVD, S3 and S4
34
Q

Dx of Restrictive cardiomyopathy?

A
  • CXR: signs of CHF w/o cardiomegaly
  • EKG: nonspecific changes most likely
  • Echo: thickened LV walls with normal or slightly decreased volume (rapid early ventricular filling, but late filling will be absent because no compliance)
  • cath: R heart cath - filling pressure
35
Q

Describe findings of amyloid heart disease?

A
  • thickened, firm, rubbery ventricular muscle
  • major diastolic dysfunction
  • possible systolic dysfxn
  • low voltage on EKG
  • abnorm Q waves (look like MI)
  • conduction disturbances, arrhythmias, not uncommon
  • echo: thickened walls everywhere
  • may find on rectal or gingival bx, may require myocardial bx
36
Q

difference b/t restrictive and constrictive cardiomyopathy?

A
  • hx provides impt clues to distinguish b/t the 2
  • constrictive pericarditis: hx of TB, trauma, pericarditis, collagen vascular disorders
  • restrictive cardiomyopathy: amyloidosis, sarcoidosis, hemochromatosis (iron deposits in skin)
37
Q

Tx of restrictive cardiomyopathy?

A
  • no satisfactory medical therapy
  • drug therapy must be used with caution:
    diuretics for extremely high filling pressures
    vasodilators may decrease filling pressures (ACEIs)
  • CCBs to improve diastolic compliance
38
Q

When should we bx in cardiomyopathy?

A
  • acute heart failure with sxs refractory to current medical management
  • rapidly decreaseing LVEF with no clear etiology despite conventional therapy of HF
  • HF with acutely worsening rhythm disturbances, particularly ventricular tachycardia
  • new HF with conduction disturbances, particularly nodal block
  • HF in setting of periph eosinophilia, rash and fevere
  • HF in setting of hx of:
    collagen vascular diseases: SLE, scleroderma, marfan
    infiltrative and storage diseases: amyloid, sarcoid, hemochromatosis
  • giant cell myocarditis (new tx)
  • neoplasms
39
Q

What conditions of restrictive cardiomyopathy warrant a bx that has effective therapy?

A
  • cardiac allograft rejection
  • sarcoidosis
  • giant cell myocarditis
  • hypereosinophilic syndrome
  • endocardial fibroelastosis
  • cardiac hemochromatosis
  • lyme carditis
  • some malignancies