Cardiomyopathy Flashcards

1
Q
  • What is the simple definition of cardiomyopathy?
  • Which ventricle is predominantly involved?
  • What types of presentations does cardiomyopathy have?
A
  • Disease of the myocardium
  • Left ventricle
  • Can vary from asymptomatic to decompensated congestive heart failure to cardiac arrest
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q
  • How are cardiomyopathies categorized?
  • What are the diagnostic imaging modalities that can be used to detect and diagnose cardiomyopathy?
  • Which modality is the study of choice?
  • What types of dysfunction can occur as a result of cardiomyopathy? (very generally speaking)
A
  • By cause and pathophysiology
  • Echocardiography, nuclear imaging, coronary angiography with left ventriculography, cardiac MRI
  • Echocardiography
  • Transient and permanent
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

-What are the different ways to classify cardiomyopathies?

A
  • Intrinsic vs extrinsic
  • Primary vs secondary
  • Ischemic vs non-ischemic
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

-What are the WHO/ISFC classes of cardiomyopathy?

A
  • Dilated cardiomyopathy (DCM)
  • Hypertrophic cardiomyopathy (HCM)
  • Restrictive cardiomyopathy (RCM)
  • Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D)
  • Unclassified
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q
  • What is systolic dysfunction?
  • What are the compensatory mechanisms for systolic dysfunction?
  • What eventually happens to these mechanisms?
A
  • A decrease in myocardial contractility and a reduction in left ventricular ejection fraction
  • Left ventricular enlargement (resulting in higher stoke volume), Frank-Starling relationship
  • They eventually fail and heart failure ensues
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q
  • What is diastolic dysfunction?
  • What is the relationship between this and ventricular dysfunction?
  • Why is diastolic dysfunction often missed or underestimated?
A
  • Left ventricular relaxation and filling are abnormal resulting in elevating filling pressures
  • There is non, diastolic may occur with or without systolic dysfunction
  • It is very difficult to quantitate on echocardiography
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q
  • What is the most common type of heart failure in the US?
  • What does the lack of O2 in the myocardium cause?
  • What is the main cause of ischemic cardiomyopathy?
  • What other causes are there?
  • Which type of dysfunction characterizes ischemic cardiomyopathy?
A
  • Ischemic
  • Myocardial damage, hibernation and death
  • CAD
  • Cocaine, vasospasm, thrombus
  • Systolic dysfunction that can be transient or permanent, and predominantly involves the LV, but can include the RV too
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q
  • What is the clinical presentation of ischemic cardiomyopathy?
  • What will an echocardiogram show?
  • What imaging study is recommended if the cause of LV dysfunction is unknown?
A
  • CHF symptoms (edema, dyspnea); EKG may indicate old MI; chest xray may show pulmonary edema
  • Reduced LVEF and regional wall motion abnormality
  • Coronary angiography
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q
  • How do you manage ischemic cardiomyopathy?

- Why should a nuclear viability study be considered?

A
  • Revascularization of the myocardium with PCI or CABG, especially during acute ischemia/infarction
  • To determine if myocardial dysfunction is due to scar or hibernating myocardium
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q
  • How do you prevent sudden cardiac arrest in patients with ischemic cardiomyopathy?
  • Which patients should get cardiac rehab?
A
  • External wearable defibrillator, implanted cardioverter-defibrillator
  • Post MI patients; extremely important, physical therapy with a telemetry monitor (raise heart rate to 85% of max)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q
  • What is the most common form cardiomyopathy?
  • What are the characteristics of dilated cardiomyopathy?
  • What are the clinical presentations of dilated cardiomyopathy?
  • What type of surgery is this cardiomyopathy the primary indication for?
A
  • Dilated cardiomyopathy

- Dilation and impaired contraction of one or both ventricles, predominantly in the left ventricle, with LVEF

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q
  • What is the etiology of dilated cardiomyopathy?
  • What is the most common type of infectious DCM? Caused by?
  • How is the cause of DCM confirmed?
  • What is the mechanism of pathology of infectious DCM?
  • What are the bacterial causes of DCM?
A
  • Commonly unknown, idiopathic but can be infectious, genetic, caused by toxins or systemic disorder, or occur peripartum, as a result of endocrine dysfunction or be tachycardia induced
  • Viral: Parvovirus B19, herpes, coxsackievirus, influenza, adenovirus, CMV, HIV
  • Biopsy
  • Unknown, but direct cytotoxicity to cardiac myocytes and adverse autoimmune response are both possibilities
  • TB, Meningococcal, Pneumococcal
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q
  • What are two diseases that may cause dilated cardiomyopathy?
  • Where is Chagas disease the leading cause of DCM?
  • How does DCM resulting from lyme disease manifest?
A
  • Chagas disease caused by protozoan infection; and Lyme disease
  • Central and South America
  • Usually as a conduction abnormality, but may cause myocardial dysfunction due to myocarditis; also arrhythmias, AV blocks and effusions
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q
  • Is genetic DCM predominantly autosomal dominant or recessive?
  • What is the mechanism of pathology of genetic DCM?
  • Which inherited syndromes can lead to DCM?
  • Which systemic disorders can include DCM?
A
  • Autosomal
  • Involves antibodies to a variety of cardiac proteins
  • Muscular dystrophies, hemochromatosis, thalassemias
  • Sarcoidosis, SLE, celiac disease, scleroderma, RA
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q
  • What are the typical “toxins” that can lead to DCM?
  • Which meds in particular?
  • How does alcohol cause DCM?
A
  • Alcohol, cocaine, Meds
  • Adriamycin, Trastuxamab, Lithium
  • Poorly understood, occurs with excessive consumption, and abstinence can result in improvement in LVEF
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q
  • How common is peripartum DCM?

- What types of endocrine dysfunction can lead to DCM?

A
  • It is rare, the etiology is unclear, occurs in the last month of pregnancy to 5 months after delivery and presents as CHF or SCA
  • Thyroid dysfunction, pheochromocytomas, Cushing’s, or GH excess or deficiency
17
Q
  • What types of tachycardias can lead to DCM?

- How can tachycardia cause DCM?

A
  • Afib, SVT, AVNRT

- Heart rate correlates with LV dysfunction, causes reduced myocyte contractility

18
Q

-How do you treat DCM?

A

-Treat underlying cause if known, CHF management, SCA prevention

19
Q
  • What causes hypertrophic cadiomyopathy?
  • What are the characteristics of HCM?
  • What does the LV hypertrophy eventually lead to?
  • What else may HCM lead to?
  • How does HCM present?
  • What types of arrhythmias are patients with HCM at higher risk for?
  • What type of murmur should you listen for on sports physicals?
A
  • Genetics: mutation of sarcomere genes, but just because you have the genes, doesn’t mean that you will have the disease
  • Left Ventricular hypertrophy, occasionally RV involved, NOT caused by pathologic loading conditions such as HTN and valvular disease, interventricular septum is commonly more prominently involved
  • Diastolic dysfunction
  • LV outflow obstruction, myocardial ischemia and/or mitral regurgitation
  • Chest pain, syncope and palpitation are the triad, also fatigue, CHF, and SCA
  • Afib, SVT and VT/VF and also SCA
  • Mis-systolic, harsh, 3rd and 4th intercostals, louder with valsalva, quieter with squatting
20
Q
  • What might you see on EKG of a person with HCM?
  • Which imaging modality is the diagnosing method of choice?
  • How do you manage HCM?
A
  • May demonstrate LVH pattern
  • Echocardiogram
  • Avoid volume depletion, activity restriction, beta blockers or verapamil, septal myectomy or alcohol septal ablation, screening of 1st degree relatives (annual echo until age 20 and then every 5 years)
21
Q
  • I say restrictive cardiomyopathy and you think?
  • What characterizes RCM?
  • What does it cause?
  • Which chambers are usually effected by RCM?
  • How will they appear on echo?
  • Which imaging modality is the best for diagnosing RCM?
A
  • Stiff heart, not thick heart
  • Nondilated ventricles with impaired filling
  • Diastolic dysfunction
  • Ventricles predominantly
  • Nonhypertrophied, with moderate to sever biatrial enlargement due to impaired diastolic function
  • Cardiac MRI, but echo helps as well
22
Q
  • What is the etiology of RCM?

- What are the potential causes?

A

-Fibrosis or infiltration of the ventricular wall
-Infiltrative disorders: amyloidosis, sarcoidosis, fatty infiltration
Storage diseases: Hemochromatosis, Fabry disease
Radiation, chemo (usually causes DCM), carcinoid heart disease, hypereosinophilic syndrome (zebra)

23
Q
  • Why should a biopsy be considered in patients with RCM?

- How do you treat RCM?

A
  • It differentiates between constrictive pericarditis and restrictive cardiomyopathy
  • Treat underlying cause if known, reduce pulmonary and systemic congestion with diuretics
24
Q
  • Which cardiomyopathy are we learning about that is very rare and more common in Europe?
  • What characterizes it?
  • How does ARVC present?
  • How do you diagnose it?
  • How do yo manage it?
A
  • Arrhythmogenic Right Ventricular Cardiomyopathy
  • Ventricular arrhythmias and right ventricular free wall myocardium replaced by fibrous/fatty tissue, producing RV dilation
  • Chest pain, palpitations, syncope, SCA
  • Echo and cardiac MRI
  • Pulmonary and systemic congestion with diuretics, ventricular arrhythmias with antiarrhythmics, ablation or ICD
25
Q

Tell me about unclassified cardiomyopathies

A

-Left Ventricular Noncompaction
-Rare, 0.05% of all echoes done in the US
-Altered myocardial wall due to intrauterine
arrest of compaction of the loose interwoven . meshwork
-CHF, thromboembolism, and ventricular
arrhythmias
-Cardiac MRI to confirm
-Stress-Induced Cardiomyopathy
-Causes an ACS (even STEMI) in the absence of
critical CAD
-A result of intense psychological or physical stress
-Primarily occurs in postmenopausal women
-AKA Broken heart syndrome and Takotsubo
cardiomyopathy
-Characterized by LV apical ballooning on echo of LV
angiography (systolic dysfunction of the apical and/or mid segments)
-Almost all patients recover in a few weeks