Cardiomyopathies Flashcards

(45 cards)

1
Q

What is a cardiomyopathy (CM)?

A

Disease of heart muscle affecting myocardial systolic and/or diastolic function

Cardiomyopathies can be categorized based on various criteria, including etiology and physiology.

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2
Q

How are cardiomyopathies classified?

A

Classified by etiology as:
* Ischemic CM
* Non-ischemic CM

Ischemic CM is often associated with dilated CM due to ischemic heart disease.

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3
Q

What is the most common classification of cardiomyopathies based on physiology?

A

Dilated vs. hypertrophic vs. restrictive

This classification is essential for understanding the functional impairments associated with each type.

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4
Q

What is the definition of dilated cardiomyopathy (DCM)?

A

Dilated – dilated ventricle with impaired inotropy

DCM primarily affects the heart’s ability to contract effectively.

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5
Q

What is the definition of hypertrophic cardiomyopathy (HCM)?

A

Hypertrophic – abnormally thickened ventricular wall with impaired lusitropy

HCM usually maintains intact systolic function but is characterized by diastolic dysfunction.

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6
Q

What is the definition of restrictive cardiomyopathy (RCM)?

A

Restrictive – abnormally stiffened myocardium with impaired lusitropy

Systolic function is usually normal or near-normal in RCM.

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7
Q

What are the clinical manifestations of dilated cardiomyopathy (DCM)?

A

CHF due to reduced contractility

DCM leads to low cardiac output, resulting in congestive heart failure symptoms.

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8
Q

What are the clinical manifestations of hypertrophic cardiomyopathy (HCM)?

A

CHF and exertional syncope

HCM can lead to sudden death due to arrhythmias.

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9
Q

What are the clinical manifestations of restrictive cardiomyopathy (RCM)?

A

CHF due to reduced preload and elevated LVEDP

Symptoms arise from the heart’s inability to fill properly.

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10
Q

What is the differential diagnosis of non-ischemic dilated cardiomyopathy (DCM)?

A

Includes:
* Genetic factors
* Valvular heart disease
* Inflammatory myocarditis
* Toxins (e.g., alcohol, anthracycline chemotherapy)
* Peripartum cardiomyopathy

Non-ischemic DCM can have various underlying causes, including familial and acquired conditions.

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11
Q

What is stress cardiomyopathy (Takotsubo CM)?

A

Catecholamine-related CM with transient regional systolic dysfunction mimicking IHD

It is characterized by apical ballooning of the left ventricle and occurs often in response to stress.

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12
Q

What triggers stress cardiomyopathy?

A

Excessive sympathetic response often triggered by emotional or physical stress

It primarily occurs in older women and can be associated with conditions like pheochromocytoma.

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13
Q

How is the diagnosis of Takotsubo CM made?

A

Diagnosis of exclusion with normal coronary arteries and clinical findings consistent with Takotsubo CM

Clinical findings include wall motion abnormalities and apical ballooning.

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14
Q

What is the prevalence of LVOT obstruction in Takotsubo CM?

A

25%

LVOT obstruction can complicate the management of Takotsubo CM.

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15
Q

What is the initial management for stress cardiomyopathy?

A

R/o ACS, supportive therapy for CHF, and consider heparin to prevent thrombus formation

Management may involve inotropic support or other medications depending on the patient’s hemodynamic status.

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16
Q

What is the usual treatment for patients with dilated cardiomyopathy?

A

Treatment of CHF with:
* Diuretics
* ACEI or ARB
* Beta blockers
* Aldosterone antagonists
* Cardiac resynchronization therapy

These therapies aim to improve heart function and manage symptoms.

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17
Q

What are major risk factors for sudden death in hypertrophic cardiomyopathy (HOCM)?

A

Includes:
* Prior cardiac arrest
* Non-sustained VT
* Unexplained syncope
* LV thickness > 30 mm
* Abnormal exercise blood pressure
* Family history of sudden death

These factors indicate a higher risk of arrhythmias and sudden cardiac events.

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18
Q

What is the pathophysiology of hypertrophic cardiomyopathy?

A

Impaired filling due to reduced diastolic relaxation and myocardial ischemia

The condition causes increased LVEDP and oxygen demand, leading to complications.

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19
Q

What is the significance of the Venturi effect in HOCM?

A

It draws the anterior mitral valve leaflet toward the septum, leading to systolic anterior motion (SAM)

SAM can cause mitral regurgitation and further obstruct left ventricular outflow.

20
Q

What is the Venturi effect in HCM with ASH?

A

It is a lower pressure that draws the anterior mitral valve leaflet toward the septum, leading to failure of mitral valve leaflets to coapt.

This phenomenon contributes to left ventricular outflow tract obstruction.

21
Q

How does HOCM present clinically?

A
  • Dyspnea on exertion (DOE)
  • Angina
  • Syncope

These symptoms are due to elevated left ventricular end-diastolic pressure (LVEDP), mitral regurgitation (MR), and left ventricular outflow tract (LVOT) obstruction.

22
Q

What is a distinguishing feature of the HOCM murmur compared to aortic stenosis (AS) murmur?

A

The HOCM murmur does not radiate to the carotids.

It is also affected by changes in preload.

23
Q

What do you see on ECG with classic HOCM?

A

Dagger-like septal Q waves in lateral (I, aVL, V5-6) and sometimes inferior leads, along with left ventricular hypertrophy (LVH) criteria.

These findings are indicative of hypertrophic cardiomyopathy.

24
Q

How does the degree of LVOT obstruction affect intraoperative management of patients with HOCM?

A

Systolic LVOT obstruction in HCM is dynamic and increases with decreasing preload and increased contractility.

Management involves volume optimization and avoiding vasodilators.

25
What echocardiographic findings can guide the diagnosis of HOCM?
* Degree of left ventricular hypertrophy (LVH) * Location of asymmetrical septal hypertrophy (ASH) * Degree of systolic anterior motion (SAM) * Doppler measurement of LVOT obstruction * Quantitation of MR ## Footnote These parameters help in assessing the severity and management of the condition.
26
Is endomyocardial biopsy useful in HOCM?
No, clinical and echocardiographic findings guide diagnosis and treatment. ## Footnote This differs from restrictive cardiomyopathy.
27
What is the treatment for HOCM?
* Beta blockers * Non-dihydropyridine calcium channel blockers * Antiarrhythmics * Implantable cardioverter-defibrillators (ICDs) * Percutaneous septal ablation * Open septal myomectomy * Mavacampten ## Footnote Mavacampten is a cardiac myosin inhibitor approved for NYHA class III patients.
28
What is mavacampten?
It is a cardiac myosin inhibitor used for treating HOCM, with potential side effects including CHF due to systolic dysfunction. ## Footnote Mavacampten was released in April 2023 and costs $9,200 for a 30-day supply.
29
Are elevated troponin I subunits specific for cardiac muscle?
Yes, they are specific for cardiac muscle but can occur with all types of cardiomyopathy. ## Footnote Elevated troponins alone do not confirm myocardial infarction (MI); other findings are necessary.
30
What findings are associated with elevated troponin I in HCM?
* Higher left ventricular mass * Higher LVOT gradient * More fibrosis * Higher mortality ## Footnote Sudden cardiac death may occur in HCM with normal troponin I levels.
31
What are the characteristics of restrictive cardiomyopathy (RCM)?
Abnormally rigid ventricles that impair diastolic filling; systolic function is normal or near normal. ## Footnote RCM often leads to elevated LVEDP and reduced cardiac output.
32
What is the differential diagnosis of restrictive cardiomyopathy based on physiology?
* Infiltration (e.g., amyloidosis) * Endomyocardial disorders (e.g., Loeffler endocarditis) * Storage diseases (e.g., hemochromatosis) * Fibrosis (e.g., post-radiation therapy) ## Footnote Some conditions may present with features of both dilated and restrictive cardiomyopathy.
33
What is the most common form of restrictive cardiomyopathy?
Amyloidosis. ## Footnote It involves the buildup of amyloid fibrils in tissues.
34
How is the diagnosis of amyloidosis made in the correct clinical setting?
Often through an abdominal fat pad biopsy, although sensitivity may vary. ## Footnote This biopsy helps confirm the presence of amyloid deposits.
35
What is the common treatment for restrictive cardiomyopathy?
* Treatment of congestive heart failure (CHF) * Diuretics * Anticoagulation for atrial fibrillation (AF) * Treatment of the underlying systemic disorder ## Footnote Vasodilators are usually not beneficial due to normal systolic function.
36
What is Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC)?
A condition characterized by the replacement of right ventricle with adipose and fibrous tissue, associated with arrhythmias and right heart failure. ## Footnote It can lead to sudden death in young athletes.
37
What causes Arrhythmogenic Right Ventricular Cardiomyopathy?
It is inherited with autosomal dominance and variable penetrance, often exacerbated by exercise-induced RV wall stress. ## Footnote Genetic testing can help confirm the diagnosis.
38
What is the treatment for ARVC?
* ICD implantation * Treatment of right heart failure (RHF) ## Footnote Management focuses on preventing sudden cardiac events.
39
What is the key physiologic problem in restrictive cardiomyopathy?
Impaired filling (lusitropy) with usually normal left ventricular ejection fraction (LVEF). ## Footnote Elevated LVEDP can lead to CHF.
40
What distinguishes restrictive cardiomyopathy from constrictive pericarditis?
Kussmaul’s sign, which indicates right heart dysfunction, is typically present in constrictive pericarditis but not in RCM. ## Footnote Imaging studies help differentiate these conditions.
41
What are the treatment options for amyloidosis-related restrictive cardiomyopathy?
* Diuretics * Treatment of AF * Anticoagulation * Antineoplastic therapy for amyloidosis ## Footnote Afterload reduction is not useful due to normal systolic function.
42
All cardiomyopathies have what?
Low CO Due to low SV
43
Kussmaul’s sign
Rise in JVD with inspiration
44
In which cardiomyopathy do you see low voltage?
Restrictive
45
Amyloidosis
● Multisystem disease including heart, kidneys (nephrotic syndrome GN), sensory and autonomic neuropathies, GI, endocrine, CT syndrome -buildup of amyloid fibrils (derived from protein) in tissue Primary=immune Secondary=chronic inflammatory conditions