Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

BCC > Cardiomyopathy > Flashcards

Cardiomyopathy Flashcards

1
Q

What are the types of primary cardiomyopathies issued by the AHA?

A

Genetic, mixed, and acquired

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

What are the genetic causes of primary cardiomyopathies?

A

HCM, ARVC/D, LVNC, glycogen storage disorders (PRKAG2, Danon), Conduction defects, mitochondrial myopathies, Ion channel disorders (LQTS, Brugada, SQTS, CVPT, Asian SUNDS)

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

What are the acquired causes of primary cardiomyopathies?

A

Inflammatory (myocarditis), stress-induced (Takotsubos), peripartum, tachycardia-induced, infants of insulin-dependent diabetic mothers

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

What are the mixed causes of primary cardiomyopathies?

A

Dilated CM, restrictive (non-hypertrophied and non-dilated)

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

What are the causes of secondary cardiomyopathies?

A

Infiltrative (amyloidosis, Gaucher disease, Hurler’s disease, Hunter’s disease), Storage (hemochromatosis, Fabry’s disease, Glycogen storage disease - type II, Pompe, Niemann-Pick disease), Toxicity, Endomyocardial (fibrosis or hypereosinophilic syndrom), Inflammatory/granulomatous (Sarcoidosis), Endocrine (DM, hyper/hypothyroidism, pheo, acromegaly), Cardiofacial (Noonan syndrome, Lentiginosis), Neuromuscular (Friedrich’s ataxia, Duchenne-Becker, Emery-Dreifuss, NF1/2, tuberous sclerosis), Nutritional deficiencies (Beriberi -thiamine, pellagra, scurvy, selenium, carnitine, kwashiorkor), Autoimmune/collagen (SLE, dermatomyositis, RA, scleroderma, polyarteritis nodosa), electrolyte imbalances, cancer therapy (anthracyclines like doxorubicin, adriamycin, danorubicin, cyclophosphamide, radiation)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

How does the European Society of Cardiology (ESC) classify cardiomyopathies?

A

Based on ventricular morphological and functional phenotypes; HCM, dilated CM, ARVC, restrictive, or unclassified

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

What is the most common type of CM as classified by the ESC?

A

Dilated cardiomyopathy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

What is familial dilated cardiomyopathy?

A

20-48% of all cases + genetics involved (autosomal dominant with incomplete penetrance and variable extression) + 4th to 5th decade of life

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

What are secondary causes of dilated CM?

A

Infection, toxins, autoimmune disease, pregnancy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

What is the 5-year mortality rate of dilated CM once you develop symptoms?

A

50%; 25% of patients who develop symptoms will recover sponaneously though

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

What do you see in the heart with patients with dilated CM?

A

4 chamber dilation (ventricles > atria) + thinning of ventricular walls + hypertrophy of heart + regurgitant valves

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

What are the general characteristics of a restrictive cardiomyopathy?

A

Normal or decreased volume of both ventricles associated with biatrial enlargement + normal LV wall thickness + normal AV valves + impaired ventricular filling with restrictive physiology + normal/near normal systolic function

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

What are myocardial causes of restrictive CM?

A

Noninfiltrative (idiopathic, familial, hypertrophic, scleroderma, pseudoxanthoma elasticum, diabetic CM) + infiltrative (amyloidosis, sarcoidosis, Gaucher disease, Hurler disease, fatty infiltration) + storage diseases (hemochromatosis, Fabry disease, glycogen storage disease)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

What are endomyocardial causes of restrictive CM?

A

Endomyocardial fibrosis + hypereosinophilic syndrome + carcinoid heart disease + metastatic cancers + radiation + toxic effects of anthracycline + drugs causing fibrois endocarditis (serotonin, methysergide, erotamine, mercurial agents, busulfan)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Difference between restrictive CM and constrictive pericarditis

A

RCM: LVEDP > RVEDP (by at least 5 mmHg) + inspiration in spontaneous ventilation leads to drops in both right and left ventricular pressures due to decreased intrathoracic pressure; CP: ventricular diastolic pressures are equal and elevated + inspiration causes increased right-sided filling and subsequent decreased left-sided filling from ventricular interdependence

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

What signs are associated with a worse prognosis in restrictive CM?

A

Age > 70yo + increasing NYHA functional class + LA diameter > 60mm

17
Q

What is arrhythmogenic RV cardiomyopathy (ARVC)?

A

Progressive fibrofatty replacement of myocardiaum predominantly along the RV free wall –> leads to ventricular wall thinning and aneurysmal dilation (increased risk of re-entry arrhthmias)

18
Q

What is the pathophysiology of ARVC?

A

Mutations of desmosomes (cellular adhesion proteins) which can disrupt intercellular junctions and cause myocyte detachment and cell death

19
Q

What is considered a disease modifier in ARVC?

A

Sports activity; promotes the progression of disease by aggravating the mechanical uncoupling of myocytes and triggers malignant ventricular arrhythmias through catecholamine release

20
Q

What age and gender do you normally see ARVC in?

A

Between adolescence and 4th decade of life, men > women + 50% have a family history of sudden cardiac death

21
Q

What is the concealed phase of ARVC?

A

The phase before clinically significant disease; no overt structural damage

22
Q

What is a common first presenting symptom of ARVC?

A

Sudden cardiac death (accounts for up to 20% of sudden cardiac death in young people)

23
Q

What finding is pathognomonic for ARVC?

A

RV aneurysms

24
Q

Can the left side be affected by ARVC?

A

Yes, sometimes this is confused with dilated CM because of biventricular involvement

25
Q

What is the classic EKG finding for ARVC?

A

T-wave inversions in the right-sided precordial leads (V1-V3) + Epsilon wave (V1-V2) + QRS > 120ms + extrasystoles with LBBB

26
Q

What is an epsilon wave on EKG?

A

Common finding in ARVC; small-amplitude distinct potentials between the end of the QRS complex and the beginning of the T-waves

27
Q

What is the most common cause of myocarditis?

A

Viral infections (Parvovirus B19 and human herpes virus 6)

28
Q

What are the 3 phases of pathogenesis for viral cardiomyopathies?

A
  1. Acute viral: virus proliferates in host tissue, often missed
  2. Subacute immune: activation of acquired immunity leads to chronic inflammation that promotes myocyte necrosis, fibrosis and remodeling
  3. Chronic myopathic: persistent inflammatory response leads to structural changes, heart failure, and dilated CM
29
Q

What is the gold standard for diagnosing viral CM?

A

Endomyocardial biopsy

30
Q

What are risk factors for peripartum cardiomyopathy?

A

African-American ethnicity + hypertensive disorders of pregnancy + multiparity + advanced maternal age + multifetal pregnancy + prolonged tocolysis + family history

31
Q

Why do we think peripartum cardiomyopathy occurs?

A

It is a vascular disease triggered by hormonal changes of pregnancy

32
Q

What is the clinical course of peripartum CM?

A

Highly variable; sometimes it can lead to rapid progression of heart failure or completely recovery; usually seen 3-6 months after diagnosis but can take up to 48 months

33
Q

What are some risk factors that lead to lower likelihood of recovery from peripartum CM?

A

African-American ethnicity + LVEF < 30% + LV dilation + LV thrombus + RV systolic dysfunction + obesity

BCC (29 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions