Hypertrophic Cardiomyopathy Quiz
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Questions and Answers

What is a consequence of diastolic dysfunction in left ventricular hypertrophy?

  • Improved early-diastolic LV filling
  • Enhanced ventricle compliance
  • Decreased left atrial pressure
  • Increased LV filling pressures (correct)

Which pathology is NOT commonly associated with an increased prevalence of HCM?

  • Chronic kidney disease (correct)
  • Aging
  • Hypertension
  • Aortic stenosis

What characterizes the left ventricle outflow tract obstruction (LVOT) in obstructive HCM?

  • Dynamic obstruction due to thickened septum (correct)
  • A fixed obstruction caused by congenital defects
  • Obstruction at the level of the mitral valve
  • Decreased ejection fraction

What is the effect of the Venturi effect in HCM?

<p>It translates to a pressure gradient between the left ventricle and aorta (B)</p> Signup and view all the answers

In patients with HCM, what does preserved LV systolic function imply?

<p>The ejection fraction may be normal or elevated (A)</p> Signup and view all the answers

What leads to abnormal myocyte architecture in HCM?

<p>Loss of structural integrity in myocytes (D)</p> Signup and view all the answers

What effect does LV hypertrophy have on late phases of diastole?

<p>Impairment of end-diastolic LV filling (A)</p> Signup and view all the answers

What condition may occur due to the presence of mild to moderate pulmonary hypertension in HCM?

<p>Increased left atrial pressures (D)</p> Signup and view all the answers

What is the minimum QT interval associated with an increased risk of cardiac arrhythmias in short QT syndrome?

<p>300 milliseconds (A)</p> Signup and view all the answers

Which class of cardiomyopathy primarily affects only the heart muscle?

<p>Primary cardiomyopathies (A)</p> Signup and view all the answers

Which of the following is NOT a subtype of primary cardiomyopathy?

<p>Coronary artery disease (B)</p> Signup and view all the answers

In hypertrophic cardiomyopathy, what is the defining feature regarding the left ventricular wall thickness in adults?

<p>15mm or more (D)</p> Signup and view all the answers

What type of pattern of inheritance is most common for hypertrophic cardiomyopathy?

<p>Autosomal dominant inheritance (B)</p> Signup and view all the answers

Which of the following is a characteristic of the MOGES classification?

<p>Considers five distinct parameters (B)</p> Signup and view all the answers

Which of the following statements about hypertrophic cardiomyopathy is incorrect?

<p>It shows a geographic pattern of distribution. (A)</p> Signup and view all the answers

What does the 'O' in the MOGES classification stand for?

<p>Organ/system involvement (D)</p> Signup and view all the answers

What percentage of patients with hypertrophic cardiomyopathy have a familial disease associated with gene mutations?

<p>Approximately 60% (D)</p> Signup and view all the answers

Which of the following conditions is most likely to evolve into another cardiomyopathy phenotype in an individual patient?

<p>Hypertrophic cardiomyopathy (D)</p> Signup and view all the answers

What is a major consequence of increased left ventricle filling pressure in patients with hypertrophic cardiomyopathy?

<p>Reduced coronary blood flow (C)</p> Signup and view all the answers

Which symptom is experienced by more than 90% of symptomatic patients with hypertrophic cardiomyopathy?

<p>Exertional dyspnea (D)</p> Signup and view all the answers

What is a characteristic auscultatory finding in obstructive hypertrophic cardiomyopathy?

<p>Paradoxical split S2 (C)</p> Signup and view all the answers

Which ECG finding is commonly associated with hypertrophic cardiomyopathy?

<p>Increased QRS voltage (A)</p> Signup and view all the answers

Which of the following arrhythmias is least likely to occur in patients with hypertrophic cardiomyopathy?

<p>Ventricular flutter (B)</p> Signup and view all the answers

What is a common result of myocardial hypertrophy in relation to oxygen demand?

<p>Increased oxygen demand (A)</p> Signup and view all the answers

Which physical examination finding is typically observed in patients with hypertrophic cardiomyopathy?

<p>Forceful and leftward displaced precordial impulse (C)</p> Signup and view all the answers

What is a potential first clinical manifestation in hypertrophic cardiomyopathy patients?

<p>Sudden death (A)</p> Signup and view all the answers

What is a key pathological feature of Arrhythmogenic Cardiomyopathy?

<p>Replacement of myocytes by fibrous or fibro-adipose tissue (A)</p> Signup and view all the answers

Which of the following describes the histological findings associated with the right ventricular free wall in Arrhythmogenic Cardiomyopathy?

<p>Transmural myocardial atrophy with fibrofatty replacement (B)</p> Signup and view all the answers

What can result from the enlargement and dysfunction of the ventricles in Arrhythmogenic Cardiomyopathy?

<p>Heart failure and sudden cardiac death (C)</p> Signup and view all the answers

Which statement about the biventricular variant of Arrhythmogenic Cardiomyopathy is true?

<p>It demonstrates both right and left ventricular involvement. (C)</p> Signup and view all the answers

What distinguishes the left ventricular free wall histology in Arrhythmogenic Cardiomyopathy?

<p>Subepicardial midmural involvement without transmural changes. (B)</p> Signup and view all the answers

What is a primary electrical disturbance associated with Arrhythmogenic Cardiomyopathy?

<p>Abnormal electrical circuits resulting in ventricular arrhythmias (C)</p> Signup and view all the answers

In terms of gross examination, what is observed in the right ventricular walls in Arrhythmogenic Cardiomyopathy?

<p>Regional wall thinning due to myocardial atrophy (A)</p> Signup and view all the answers

Which of the following symptoms is NOT commonly associated with Arrhythmogenic Cardiomyopathy?

<p>Improved exercise tolerance (A)</p> Signup and view all the answers

What is the primary reason biopsy should be reserved for selected patients in the diagnosis of AC?

<p>Biopsy is invasive and carries risks. (A), A definitive diagnosis can often be made without it. (C)</p> Signup and view all the answers

Which treatment is prioritized for the prevention of sudden cardiac death (SCD) in ARVC patients?

<p>Intracardiac cardioverter defibrillator (ICD) (B)</p> Signup and view all the answers

In which situation is the use of antiarrhythmic drugs particularly indicated for ARVC patients?

<p>Patients with a history of cardiac arrest from ventricular fibrillation. (D)</p> Signup and view all the answers

Which patient characteristics are considered when determining eligibility for heart transplantation in ARVC?

<p>Young age and male sex (B)</p> Signup and view all the answers

What diagnostic technique is recommended for obtaining samples in the assessment of arrhythmogenic right ventricular cardiomyopathy (ARVC)?

<p>Samples from the RV free wall (D)</p> Signup and view all the answers

What is assessed through echocardiography in patients with potential left ventricular obstruction?

<p>Left ventricular wall thickness (A)</p> Signup and view all the answers

Which statement regarding exercise stress echocardiography is accurate?

<p>It can reveal abnormalities in hypertrophic cardiomyopathy (HCM) patients. (A)</p> Signup and view all the answers

What limitation is associated with stress tests in the context of hypertrophic cardiomyopathy?

<p>High false-positive rates due to EKG abnormalities (D)</p> Signup and view all the answers

Which echocardiographic parameter is specifically used for understanding hemodynamics related to left ventricular outflow tract?

<p>Left ventricular outflow tract (LVOT) gradient measurement (A)</p> Signup and view all the answers

How does cardiovascular magnetic resonance (CMR) imaging assist in cardiac evaluation?

<p>It accommodates for poor acoustic windows. (D)</p> Signup and view all the answers

What critical assessment does the Valsalva maneuver provide in echocardiography?

<p>Detection of LVOT gradient in hypertrophic cardiomyopathy (D)</p> Signup and view all the answers

What is a recognized abnormal blood pressure response during exercise for assessing severe obstruction?

<p>Inappropriate increase greater than 20 mmHg (C)</p> Signup and view all the answers

What does late gadolinium enhancement (LGE) indicate in cardiovascular magnetic resonance imaging?

<p>Presence of infiltrative disorders or myocardial fibrosis (C)</p> Signup and view all the answers

Flashcards

Short QT Syndrome

A rare heart condition characterized by an unusually short QT interval on an electrocardiogram (ECG).

Cardiomyopathies

A group of heart muscle diseases where the heart's structure and function are abnormal, not caused by issues like coronary artery disease, hypertension, or valve problems.

Hypertrophic Cardiomyopathy (HCM)

A type of cardiomyopathy characterized by thickening of the heart muscle, particularly in the left ventricle, often affecting the septum (wall) between the heart's chambers.

Dilated Cardiomyopathy (DCM)

A type of cardiomyopathy where the heart's chambers enlarge and weaken, leading to poor pumping function.

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Restrictive Cardiomyopathy

An abnormal condition affecting the heart muscle, but with no known cause (idiopathic) and without enlargement of the left ventricle.

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Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC)

A rare type of cardiomyopathy where the right ventricle of the heart is affected, leading to abnormal heart rhythms.

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Non-Dilated Left Ventricular Cardiomyopathy

A type of cardiomyopathy where the left ventricle of the heart is not enlarged but shows scarring or fat deposits.

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MOGES Classification

A classification system for cardiomyopathies, focusing on five key parameters: morpho-functional phenotype, organ/system involvement, genetics, etiology, and stage.

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American Heart Association Classification

A classification system for cardiomyopathies based on the heart's primary structure or function.

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Primary Cardiomyopathies

A group of cardiomyopathies where the cause is unknown and not related to any other underlying illness.

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What is hypertrophic cardiomyopathy (HCM)?

A heart condition where the heart muscle, particularly the left ventricle, thickens, often affecting the septum between the heart's chambers.

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How does HCM impact coronary blood flow?

The reduced flow of blood to the heart muscle due to increased pressure inside the left ventricle, especially during the heart's relaxation phase.

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How does HCM affect cardiac output?

HCM can cause reduced cardiac output, which is the amount of blood pumped by the heart per minute.

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How does HCM impact oxygen demand?

HCM can increase the demand for oxygen by the thickened heart muscle, leading to potential problems.

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What is the most common symptom of HCM?

The most common symptom of HCM is shortness of breath during exertion.

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What is a common physical sign of HCM?

Due to the thickened heart muscle, there is often a forceful and displaced pulse that can be felt in the chest.

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How can HCM affect heart rhythms?

HCM can disrupt electrical signal conduction through the heart, leading to various rhythm irregularities.

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What is a 'tachycardia' as related to HCM?

An abnormal heart rhythm, specifically a heart rate that is too fast, can occur in HCM.

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What is hypertrophy in HCM?

The thickened heart muscle, particularly in the left ventricle, often affecting the septum (wall) between the heart's chambers, leading to a decrease in the size of the left ventricle. This is a common finding in people with hypertrophic cardiomyopathy (HCM).

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What is the relationship between coronary wall thickness and ischemia?

The process by which the coronary wall becomes thicker, often related to ischemic events. This can lead to damage to the heart muscle cells and affect the heart's function.

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What is diastolic dysfunction in HCM?

A condition where the heart's ability to relax and fill with blood properly is impaired. This can be caused by the thickened ventricle wall and the loss of elasticity. It can lead to problems with blood flow throughout the body.

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What is reduced LV distensibility and compliance in HCM?

This occurs when the ventricle cannot fill with enough blood during the late stages of diastole, leading to a reduced capacity to pump blood. This is a consequence of the thickened ventricle wall and reduced elasticity.

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What is LVOT obstruction?

The flow of blood out of the left ventricle is restricted due to the thickened septum which narrows the opening below the aortic valve. This narrowing can impede the flow of blood into the aorta.

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What is systolic anterior motion (SAM) of the mitral valve?

The mitral valve leaflet moves forward and narrows the opening between the left ventricle and the aorta due to increased blood flow velocity and abnormal papillary muscle insertion. This further constricts the outflow of blood from the left ventricle, contributing to LVOT obstruction.

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What is preserved LV systolic function in HCM?

A condition where the left ventricle can still pump blood effectively, despite the increased wall thickness. This means that the heart's ability to contract and eject blood is not significantly impaired.

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What is a typical ejection fraction in HCM?

The amount of blood ejected from the heart with each beat is typically within the normal range or even slightly elevated in individuals with HCM. This is because the heart is still able to effectively pump blood despite the thickened muscle walls.

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Echocardiography for HCM

Echocardiography measures heart structure and function using sound waves. It's helpful for seeing left ventricle size, wall thickness, and pumping function.

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Valsalva Maneuver Echocardiography

This echocardiography technique involves observing the heart during a Valsalva maneuver, a breathing technique that changes pressure in the chest. It helps detect left ventricular outflow tract obstruction (LVOTO).

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Exercise Stress Echocardiography

An echocardiogram performed during or after exercise. It helps identify HCM patients at risk for sudden cardiac death, though its value is debated.

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CMR for HCM

Cardiovascular Magnetic Resonance (CMR) imaging uses magnetic fields and radio waves to provide highly detailed images of the heart, including its structure, function, and tissue makeup.

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Late Gadolinium Enhancement (LGE)

Late gadolinium enhancement (LGE) is a special technique used in CMR to highlight areas of scar tissue in the heart muscle, helping in detecting myocardial fibrosis, a common feature of HCM.

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Abnormal Blood Pressure Response to Exercise in HCM

HCM can be associated with atypical blood pressure responses to exercise. An excessive increase or a decrease in blood pressure can signal severe obstruction.

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Impaired Functional Capacity and Functional Class in HCM

HCM patients may experience a decline in their ability to exercise and their functional class may worsen during exercise, indicating heart limitations.

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Ventricular Arrhythmias in HCM during Exercise

Ventricular arrhythmias, irregular heart rhythms, can occur in HCM patients during exercise, possibly due to the thickened heart muscle and its abnormal electrical activity.

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What is ARVC?

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a rare heart condition where the right ventricle's muscle is weakened, leading to abnormal heart rhythms and potential life-threatening events.

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What is the primary goal of ARVC treatment?

The most important goal in managing ARVC is preventing sudden cardiac death.

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How do beta-blockers help in ARVC treatment?

Beta-blockers are a type of medication used to slow the heart rate and reduce the risk of irregular heartbeats in ARVC patients.

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What is an ICD and its role in ARVC management?

An implantable cardioverter-defibrillator (ICD) is a device that is surgically implanted in the chest to detect and treat potentially life-threatening heart rhythms.

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When is a heart transplant considered for ARVC patients?

In cases of severe right ventricular dysfunction, heart transplantation may be considered as a final treatment option for ARVC.

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Replacement of myocytes

The heart muscle, normally composed of cells called myocytes, is replaced by fibrous or fatty tissue due to ARVC.

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Right Ventricular Dilation

A type of cardiomyopathy where the heart's chambers, particularly the right ventricle, enlarge and weaken, leading to poor pumping function.

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Ventricular arrhythmias

Abnormal electrical signals in the heart can cause irregular heartbeats, known as ventricular arrhythmias.

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Sudden Cardiac Death

The heart's electrical signals can become disrupted in ARVC, leading to an increased risk of sudden cardiac death.

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Right Ventricular Dysfunction

ARVC can cause the right ventricle to enlarge and become dysfunctional, leading to heart failure.

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Biventricular Variant of ARVC

ARVC can affect both the right and left ventricles, but primarily the right ventricle.

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Biventricular Variant of ARVC

In the biventricular variant of ARVC, both the right and left ventricles are affected, leading to significant heart problems.

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Study Notes

Short QT Syndrome

  • Short QT interval (less than 300 milliseconds) increases risk of cardiac arrhythmias.
  • Clinical symptoms include syncope during stress, and a family history of sudden death in 30% of cases.
  • ECG at rest is often normal.
  • Beta-blockers and implantable cardioverter-defibrillators (ICDs) are recommended.

Cardiomyopathies

  • Myocardial disorders, characterized by structural and functional abnormalities of the heart muscle.
  • Often occur in the absence of coronary artery disease, hypertension, valvular disease or congenital heart defects.
  • Different cardiomyopathy phenotypes can coexist in the same family.
  • Disease progression can evolve from one phenotype to another.

Classification of Cardiomyopathies

  • Primary cardiomyopathies: primarily affect the heart, genetic or acquired. Examples of primary cardiomyopathies include genetic and acquired conditions.
  • Secondary cardiomyopathies: involve myocardial involvement secondary to other systemic diseases. Examples of secondary cardiomyopathies include hypertrophic cardiomyopathy, dilated cardiomyopathy, non-dilated left ventricular cardiomyopathy, restrictive cardiomyopathy and, arrhythmogenic right ventricular cardiomyopathy.

Hypertrophic Cardiomyopathy (HCM)

  • Characterized by an inappropriate thickening of the left ventricle's walls, often in the basal portion of the interventricular septum.
  • Prevalence is 0.16-0.29% in the general adult population.
  • LV wall thickness of 15mm or more in adults is a clinical marker.
  • In children, the condition is diagnosed if the LV wall thickness is more than two standard deviations greater than average for a given age.
  • Approximately 60% of patients have a family history relating to myosin and myosin binding protein mutations.
  • Often asymptomatic.
  • Can lead to mitral regurgitation, systolic dysfunction and myocardial ischemia.

Dilated Cardiomyopathy (DCM)

  • Characterized by dilation and impaired contraction (systolic function) of the left or both ventricles.
  • Is not due to abnormal loading conditions.
  • Pathological anatomy shows ventricular enlargement and dilation.
  • Often, there are normal or reduced ventricular wall thickness.
  • Normal cardiac valves, with mitral and tricuspid anulus dilation.

Takotsubo Cardiomyopathy

  • Also known as stress cardiomyopathy, apical ballooning syndrome, broken heart syndrome.
  • Characterized by an acute, temporary, and reversible systolic dysfunction of the left ventricle, typically triggered by emotional or physical stress.
  • Often involves transient mid-segmental dyskinesia.
  • Presentation varies; chest pain, shortness of breath, dizziness, weakness or fainting.
  • Clinical findings may include abnormal heart sounds, irregular pulse, abnormal/low blood pressure.

Restrictive Cardiomyopathy (RCM).

  • Characterized by extremely rigid ventricular walls (increased myocardial stiffness).
  • Impairs ventricular filling, but often has normal or only mildly impaired systolic function.
  • Is the least common type of cardiomyopathy in most regions.
  • Can arise due to differing aetiological processes (e.g. inflammatory processes, deposition of amyloid, iron, or other substances, infiltrative processes), affecting the structure of the myocardial tissues.

Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC).

  • Characterized by progressive fibrofatty replacement of the right ventricle causing ventricular arrhythmias, and sudden cardiac death.
  • Can also involve the left ventricle.
  • Aetiology linked to mutations of genes important for desmosomes, including genes for desmoplakin, plakoglobin, and plakophilin-2.
  • This disorder may manifest itself with symptoms such as palpitations, fainting, and eventually sudden cardiac arrest, in younger patients.

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Description

Test your knowledge on hypertrophic cardiomyopathy (HCM) and its various implications, including diastolic dysfunction, left ventricular hypertrophy, and associated arrhythmias. This quiz covers key concepts such as LV outflow tract obstruction, myocyte architecture, and inheritance patterns related to HCM.

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