Clinical- Cardiomyopathies- Lunderg Flashcards

1
Q

What are pathologies that bring on changes in heart structure leading to changes in heart function?

A

cardiomyopathies

(diseases of the myocardium associated with cardiac dysfunction)

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2
Q

What is the name of the cardiomyopathy also known as stress-induced cardiomyopathy or broken-heart syndrome?

A

Takotsubo cardiomyopathy

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3
Q

Cardiomyopathies caused by acquired, genetic, and/or a mix between the two are:

A

primary cardiomyopathies

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4
Q

Cardiomyopathies involved with autoimmune/inflammatory, endocrine, infectious, infiltrative disorders, neuromuscular and storage diseases, nutritional deficiencies, and toxic substances are primary/secondary cardiomyopathies?

A

secondary

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5
Q

Clinical presentation of cardiomyopathies are similar to what other pathology?

A

CHF

Peripheral edema • Fatigue • Orthopnea • Dyspnea on exertion • Paroxysmal nocturnal dyspnea • Presyncope • Syncope • Cardiac ischemia

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6
Q

What is the first step to a clinical evaluation?

A

history and physical

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7
Q

What labs would be important to order for suspected cardiomyopathy?

A

CBC (anemia or infection can worsen HF)

Electrolytes (including calcium and magnesium)

BUN/creatinine

Liver Function Ts (affected by hepatic congestion)

Fasting blood glucose (underlying diabetes mellitus)

Fasting lipid profile

TSH (hyperthyroidism or hypothyroidism can affect the heart)

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8
Q

Concerning clinical eval for suspected cardiomyopathy, which test could tell us about electrical activity of heart?

A

ECG

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9
Q

Concerning clinical eval for suspected cardiomyopathy, which test could tell us about heart chamber size (dilation, restriction, hypertrophy)?

A

echocardiogram

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10
Q

Concerning clinical eval for suspected cardiomyopathy, what are some other tests that would be clinically relevant other than • History and physical • Electrocardiography • Echocardiography?

A

Cardiac magnetic resonance imaging (CMRI or CMR) • Coronary angiography • Genetic testing (with genetic counseling)

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11
Q

Which cardiomyopathy exhibits left ventricular hypertrophy without chamber dilation?

A

HYPERTROPHIC CARDIOMYOPATHY (HCM)

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12
Q

What is the inheritance pattern for HYPERTROPHIC CARDIOMYOPATHY (HCM), and the gene mutations affect what?

A

Autosomal dominant mutations of genes coding for sarcomere protein

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13
Q

Which cardiomyopathy characteristically presents with

  • atypical chest pain (may be associated with meals, dehydration, or exertion)
  • sudden cardiac death (or family members with sudden unexplained death)
  • Systolic murmur that increases in intensity with Valsalva maneuvers
  • May feel systolic thrill
  • Normal S1 and S2, but may hear S4
A

HCM

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14
Q

HCM can present with atypical chest pain that may be associated with which 3 conditons?

A

after meals (GERD like pain), dehydration, or exertion

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15
Q

The systolic murmur in HCM is a harsh heard all over the precordium but usually is not transmitted to the carotids.

A

crescendo-decrescendo

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16
Q

What cardiomyopathy is suspected with the following common ECG findings?

  • Prominent abnormal Q waves
  • P wave abnormalities
  • Left atrial enlargement
  • Left axis deviation

LVH

• Deeply inverted T waves in V2 - V4

A

HCM

• Deeply inverted T waves in V2 - V4 in apical variant HCM

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17
Q

What are the two ways of diagnosing HCM with Sokolow-Lyon criteria on ECG:

A

Sum of S wave in V1 and R wave in V5 or V6 ≥3.5 mV (35 mm)

R wave in aVL ≥1.1 mV (11 mm; or sometimes ≥1.3 mV [13 mm]

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18
Q

What would be echocardiography findings in the case of hypertrophic cadiomyopathy?

A

Echocardiography shows hypertrophy of left ventricle with reduction in ventricular chamber volume

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19
Q

What criteria is met in this ECG (in absence of HTN) concerning apical variant HCM?

A

Deeply inverted T waves in precordial leads and also T-wave abnormalities in the limb leads.

(APICAL HYPERTROPHIC CARDIOMYOPATHY suspected in the absence of HTN along with ECG findings)

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20
Q

What is being demonstrated in this echo as concerning HCM?

A

asymmetric septal hypertrophy (white arrow) compared to the inferolateral wall of the LV

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21
Q

What is being demonstrated in this MRI as concerning HCM?

A

LV septal and wall hypertrophy

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22
Q

What is being demonstrated in this cardiac magnetic resonance as concerning HCM? Also what are the small round circles?

A

severe asymmetric left ventricular (LV) hypertrophy involving the septum (S). Multiple papillary muscle heads are also noted in the LV cavity. RV, right ventricle.

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23
Q

What are the main goals of therapy for hypertrophic cardiomyopathy?

A

Decrease exertional dyspnea and chest pain

Prevent sudden cardiac death

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24
Q

What is the first line therapy for symptomatic HCM?

A

Beta Blockers

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25
Q

What therapy can be used for symptomatic HCM if B Blockers are not tolerated?

A

Nondihydropyridine calcium channel blockers (like verapamil)

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26
Q

What are the 4 surgical options for treatment of symptomatic HCM?

A

surgical septal myectomy,

alcohol septal ablation,

and dual-chamber pacing,

heart transplantation (severe systolic symptoms)

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27
Q

All patients with HCM need risk stratification for and evaluation for placement of an implantable cardioverter-defibrillator

A

SCD (sudden cardiac death)

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28
Q

What is the most common cause of SCD in athletes (1/3 of cases)?

A

HCM

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29
Q

What populations groups is SCD more prevalent?

A

male athletes, nonwhites

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30
Q

What are some of the risk factors for sudden cardiac death?

A

Family history of sudden cardiac death in first degree relative

LV wall thickness at least 30mm

Recent unexplained syncope

Prior cardiac arrest

Prior sustained VT

Non-sustained VT

or abnormal BP response

31
Q

Therapy for HCM involves 3 main components for the best outcomes:

A
  1. Symptom control (Bblockers, CCB, diuresis)
  2. SCD risk stratification (possible ICD implantation)
  3. Genetic counseling and screening
32
Q

What is the cardiomyopathy disease of desmosomal proteins characterized by fibrofatty infiltration of healthy myocardium - leads to thinning and ballooning of ventricular wall, typically RV?

A

ARRHYTHMOGENIC RIGHT VENTRICULAR DYSPLASIA/CARDIOMYOPATHY (ARVD/C)

33
Q

ARRHYTHMOGENIC RIGHT VENTRICULAR DYSPLASIA/CARDIOMYOPATHY (ARVD/C) is less common of the cardiomyopathies, about the cases are familial, and patients commonly present with symptoms in what age group?

A

1/2; 30s

34
Q

What are 2 common symptoms of ARRHYTHMOGENIC RIGHT VENTRICULAR DYSPLASIA/CARDIOMYOPATHY (ARVD/C)?

A

paplitations

syncope

35
Q

What are some ECG abnormalities with ARRHYTHMOGENIC RIGHT VENTRICULAR DYSPLASIA/CARDIOMYOPATHY (ARVD/C)?

A

inverted T waves and epsilon waves in right precordial leads

36
Q

As concerning arrhthmogenic right ventricular dysplasia/cardiomyopathy, what are some possible cardiac imaging RV abnormatlities?

A

aneurysms,

segmental dilation,

reduced ejection fraction

37
Q

Similar to pts with HCM, those with ARVD/C, what type of sports should be avoided due to increased risk of sudden cardiac death?

A

Should avoid competitive and endurance sports

38
Q

What a 4 treatment options aimed at reducing arrhythmia and preventing sudden death in ARVD/C?

A

Beta blockers, antiarrhythmic drugs

  • Catheter ablation
  • Implantable cardioverter-defibrillator
  • Heart transplantation
39
Q

What is the most common cardiomyopathy in age 40 - 59 (but can occur at any age)?

A

DCM

40
Q

A diagnosis of DCM is the leading indication for which treatment option?

A

heart transplantation

41
Q

Which cardiomyopathy is associated with Enlarged ventricles, normal LV wall thickness, systolic dysfunction, and decreased ejection fraction?

A

DCM

42
Q

What are the 2 characteristic symptoms of DCM?

A

arrhythmias and thromboembolic events

43
Q

With which testing modality is DCM diagnosed?

A

echocardiography

44
Q

Treatment guidlines is similar for what condition for the treatment of DCM?

A

CHF

45
Q

Which cardiomyopathy is characterized by nondilated ventricles with impaired ventricular filling, often with moderate to marked biatrial enlargement?

A

Restrictive cardiomyopathy

46
Q

In what place is restrictive cardiomyopathy most common?

A

tropics (Africa, India, South and Central America

47
Q

What are 3 common presenting symptoms of restrictive cardiomyopathy?

A

dyspnea,

peripheral edema (often hepatomegaly and ascites),

fatigue (from reduced cardiac output from reduced preload)

48
Q

Chest radiography often shows vascular congestion and a normal cardiac silhouette

A

pulmonary

49
Q

What are 2 possible ECG findings with restrictive cardiomyopathy?

A

diffuse reduced voltage or prolonged PR interval

50
Q

In which cardiomyopathy may echocardiography reveal biatrial enlargement and diastolic dysfunction, although left ventricular diastolic volume, wall thickness, and systolic function typically appear normal?

A

restrictive cardiomyopathy

51
Q

The treatment for restictive cardiomyopathy should be aimed at:

A

addressing underlying cause

52
Q

What are symptomatic interventions addressing restrictive cardiomyopathy?

A

managing volume overload with diuretics

or aldosterone antagonists

53
Q

Its important to evaluate pts. with restrictive cardiomyopathy for insertion?

A

AV block/pacemaker

54
Q

Concerning peripartum cardiomyopathy, pts. present with LV systolic dysfunction in last trimester to months postpartum (most patients present in the first postpartum month).

A

6

55
Q

Peripartum cardiomyopathy is associated with increasing , black race, preeclampsia, HTN, gestations and history of peripartum cardiomyopathy in previous pregnancy

A

age; multiple

56
Q

Symptoms of peripartum cardiomyopathy are consistent with heart failure (fatigue, edema and dyspnea on exertion), but can be confused with more common pregnancy complications like (can delay diagnosis of cardiomyopathy)

A

preeclampsia

57
Q

Concerning peripartum cardiomyopathy, Echocardiography commonly shows left ventricular , left ventricular dysfunction, and hypertension

A

dilation; systolic; pulmonary

58
Q

As concerning peripartum cardiomyopthy• Treatment follows standard heart failure therapy, but must consider pregnancy, which pharmocological treatments should be avoided?

A

ACE inhibitors/ARBs should be avoided in pregnancy

59
Q

In postpartum cardiomyopathy, what should you consider if treating with diuresis?

A

Avoid hypotension and reduced uterine perfusion

60
Q
A

Takotsubo Cardiomyopathy (Broken-Heart Syndrome)

61
Q

Which cardiomyopathy is also called stress-induced cardiomyopathy or broken-heart syndrome?

A

TAKOTSUBO CARDIOMYOPATHY

62
Q

Which cardiomyopathy presents with Abrupt onset of LV dysfunction in response to severe emotional or physiologic stress?

A

TAKOTSUBO CARDIOMYOPATHY

63
Q

Which population is most commonly affected by TAKOTSUBO CARDIOMYOPATHY?

A

postmenopausal women

64
Q

The presentation of Takotsubo cardiomyopathy is most similar to what other condition?

A

acute coronary syndrome

65
Q

Treatment for Takotsubo cardiomyopathy should be consistent with what other condition?

A

acute coronary syndrome

66
Q

As concerning Takotsubo cardiomyopathy, what is indicated when a loss of wall motion is noted in the left ventricular apex?

A

anticoagulation

67
Q

As concerning Takotsubo cardiomyopathy, treatments usually can be withdrawn concurrently with reversal of symptoms within month/months

A

one

68
Q

What is the diagnosis:

A 21-year-old man is evaluated during a routine medical examination prior to employment. He voices no active complaints and has no medical problems and takes no medications or illicit drugs. On review of symptoms (ROS) he tells you that 9 months ago he experienced an episode of syncope after playing volleyball with some friends. He attributed the episode to dehydration and did not seek medical attention for it. He has no family history of cardiovascular disease but reports that his father died at the age of 40 years for unclear reasons.

A

Hypertrophic Cardiomyopathy

69
Q

What is the diagnosis:

A

Hypertrophic cardiomyopathy

70
Q

What is the diagnosis:

A 45-year-old male comes to the emergency department complaining of chest pain and dyspnea. He has also noticed swelling in his legs and feet. His chest radiograph shows pulmonary vascular congestion with a normal cardiac silhouette, his electrocardiogram shows diffuse reduced voltage without signs of cardiac ischemia, and his echocardiogram reveals bi-atrial enlargement and diastolic dysfunction.

A

restrictive cardiomyopathy (RCM

71
Q

What is the diagnosis:

A 64-year-old female comes to the emergency department complaining of sharp sub-sternal chest pain that comes with exertion and is associated with diaphoresis and dyspnea. Her symptoms started soon after hearing that her children had all been killed in a car accident.

A

Takotsubo cardiomyopathy

72
Q

What is the diagnosis:

A 24-year-old male comes to your office for his pre-participation sports physical. He gets short of breath while playing sports occasionally, with associated transient mild chest pain that he attributes to the intensity of his exertion. You hear a harsh crescendo-decrescendo systolic murmur at the lower left sternal border on exam.

A

hypertrophic cardiomyopathy (HCM)

73
Q

What is the diagnosis:

A 38-year-old female who is 39-weeks pregnant comes to your office complaining of fatigue and dyspnea that is worse with any exertion. She has also noticed swelling in her feet and ankles that has been worsening over the last week.

A

peripartum cardiomyopathy

74
Q
A