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
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
3
Q
-What are the different ways to classify cardiomyopathies?
A
- Intrinsic vs extrinsic
- Primary vs secondary
- Ischemic vs non-ischemic
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
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
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
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
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
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
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)
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
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
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
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
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
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
