Podcast
Questions and Answers
What is a consequence of diastolic dysfunction in left ventricular hypertrophy?
What is a consequence of diastolic dysfunction in left ventricular hypertrophy?
Which pathology is NOT commonly associated with an increased prevalence of HCM?
Which pathology is NOT commonly associated with an increased prevalence of HCM?
What characterizes the left ventricle outflow tract obstruction (LVOT) in obstructive HCM?
What characterizes the left ventricle outflow tract obstruction (LVOT) in obstructive HCM?
What is the effect of the Venturi effect in HCM?
What is the effect of the Venturi effect in HCM?
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In patients with HCM, what does preserved LV systolic function imply?
In patients with HCM, what does preserved LV systolic function imply?
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What leads to abnormal myocyte architecture in HCM?
What leads to abnormal myocyte architecture in HCM?
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What effect does LV hypertrophy have on late phases of diastole?
What effect does LV hypertrophy have on late phases of diastole?
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What condition may occur due to the presence of mild to moderate pulmonary hypertension in HCM?
What condition may occur due to the presence of mild to moderate pulmonary hypertension in HCM?
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What is the minimum QT interval associated with an increased risk of cardiac arrhythmias in short QT syndrome?
What is the minimum QT interval associated with an increased risk of cardiac arrhythmias in short QT syndrome?
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Which class of cardiomyopathy primarily affects only the heart muscle?
Which class of cardiomyopathy primarily affects only the heart muscle?
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Which of the following is NOT a subtype of primary cardiomyopathy?
Which of the following is NOT a subtype of primary cardiomyopathy?
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In hypertrophic cardiomyopathy, what is the defining feature regarding the left ventricular wall thickness in adults?
In hypertrophic cardiomyopathy, what is the defining feature regarding the left ventricular wall thickness in adults?
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What type of pattern of inheritance is most common for hypertrophic cardiomyopathy?
What type of pattern of inheritance is most common for hypertrophic cardiomyopathy?
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Which of the following is a characteristic of the MOGES classification?
Which of the following is a characteristic of the MOGES classification?
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Which of the following statements about hypertrophic cardiomyopathy is incorrect?
Which of the following statements about hypertrophic cardiomyopathy is incorrect?
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What does the 'O' in the MOGES classification stand for?
What does the 'O' in the MOGES classification stand for?
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What percentage of patients with hypertrophic cardiomyopathy have a familial disease associated with gene mutations?
What percentage of patients with hypertrophic cardiomyopathy have a familial disease associated with gene mutations?
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Which of the following conditions is most likely to evolve into another cardiomyopathy phenotype in an individual patient?
Which of the following conditions is most likely to evolve into another cardiomyopathy phenotype in an individual patient?
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What is a major consequence of increased left ventricle filling pressure in patients with hypertrophic cardiomyopathy?
What is a major consequence of increased left ventricle filling pressure in patients with hypertrophic cardiomyopathy?
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Which symptom is experienced by more than 90% of symptomatic patients with hypertrophic cardiomyopathy?
Which symptom is experienced by more than 90% of symptomatic patients with hypertrophic cardiomyopathy?
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What is a characteristic auscultatory finding in obstructive hypertrophic cardiomyopathy?
What is a characteristic auscultatory finding in obstructive hypertrophic cardiomyopathy?
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Which ECG finding is commonly associated with hypertrophic cardiomyopathy?
Which ECG finding is commonly associated with hypertrophic cardiomyopathy?
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Which of the following arrhythmias is least likely to occur in patients with hypertrophic cardiomyopathy?
Which of the following arrhythmias is least likely to occur in patients with hypertrophic cardiomyopathy?
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What is a common result of myocardial hypertrophy in relation to oxygen demand?
What is a common result of myocardial hypertrophy in relation to oxygen demand?
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Which physical examination finding is typically observed in patients with hypertrophic cardiomyopathy?
Which physical examination finding is typically observed in patients with hypertrophic cardiomyopathy?
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What is a potential first clinical manifestation in hypertrophic cardiomyopathy patients?
What is a potential first clinical manifestation in hypertrophic cardiomyopathy patients?
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What is a key pathological feature of Arrhythmogenic Cardiomyopathy?
What is a key pathological feature of Arrhythmogenic Cardiomyopathy?
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Which of the following describes the histological findings associated with the right ventricular free wall in Arrhythmogenic Cardiomyopathy?
Which of the following describes the histological findings associated with the right ventricular free wall in Arrhythmogenic Cardiomyopathy?
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What can result from the enlargement and dysfunction of the ventricles in Arrhythmogenic Cardiomyopathy?
What can result from the enlargement and dysfunction of the ventricles in Arrhythmogenic Cardiomyopathy?
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Which statement about the biventricular variant of Arrhythmogenic Cardiomyopathy is true?
Which statement about the biventricular variant of Arrhythmogenic Cardiomyopathy is true?
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What distinguishes the left ventricular free wall histology in Arrhythmogenic Cardiomyopathy?
What distinguishes the left ventricular free wall histology in Arrhythmogenic Cardiomyopathy?
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What is a primary electrical disturbance associated with Arrhythmogenic Cardiomyopathy?
What is a primary electrical disturbance associated with Arrhythmogenic Cardiomyopathy?
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In terms of gross examination, what is observed in the right ventricular walls in Arrhythmogenic Cardiomyopathy?
In terms of gross examination, what is observed in the right ventricular walls in Arrhythmogenic Cardiomyopathy?
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Which of the following symptoms is NOT commonly associated with Arrhythmogenic Cardiomyopathy?
Which of the following symptoms is NOT commonly associated with Arrhythmogenic Cardiomyopathy?
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What is the primary reason biopsy should be reserved for selected patients in the diagnosis of AC?
What is the primary reason biopsy should be reserved for selected patients in the diagnosis of AC?
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Which treatment is prioritized for the prevention of sudden cardiac death (SCD) in ARVC patients?
Which treatment is prioritized for the prevention of sudden cardiac death (SCD) in ARVC patients?
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In which situation is the use of antiarrhythmic drugs particularly indicated for ARVC patients?
In which situation is the use of antiarrhythmic drugs particularly indicated for ARVC patients?
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Which patient characteristics are considered when determining eligibility for heart transplantation in ARVC?
Which patient characteristics are considered when determining eligibility for heart transplantation in ARVC?
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What diagnostic technique is recommended for obtaining samples in the assessment of arrhythmogenic right ventricular cardiomyopathy (ARVC)?
What diagnostic technique is recommended for obtaining samples in the assessment of arrhythmogenic right ventricular cardiomyopathy (ARVC)?
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What is assessed through echocardiography in patients with potential left ventricular obstruction?
What is assessed through echocardiography in patients with potential left ventricular obstruction?
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Which statement regarding exercise stress echocardiography is accurate?
Which statement regarding exercise stress echocardiography is accurate?
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What limitation is associated with stress tests in the context of hypertrophic cardiomyopathy?
What limitation is associated with stress tests in the context of hypertrophic cardiomyopathy?
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Which echocardiographic parameter is specifically used for understanding hemodynamics related to left ventricular outflow tract?
Which echocardiographic parameter is specifically used for understanding hemodynamics related to left ventricular outflow tract?
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How does cardiovascular magnetic resonance (CMR) imaging assist in cardiac evaluation?
How does cardiovascular magnetic resonance (CMR) imaging assist in cardiac evaluation?
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What critical assessment does the Valsalva maneuver provide in echocardiography?
What critical assessment does the Valsalva maneuver provide in echocardiography?
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What is a recognized abnormal blood pressure response during exercise for assessing severe obstruction?
What is a recognized abnormal blood pressure response during exercise for assessing severe obstruction?
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What does late gadolinium enhancement (LGE) indicate in cardiovascular magnetic resonance imaging?
What does late gadolinium enhancement (LGE) indicate in cardiovascular magnetic resonance imaging?
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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.
