Podcast
Questions and Answers
Which of the following drugs is NOT classified as an anthracycline?
Which of the following drugs is NOT classified as an anthracycline?
- Mitoxantrone
- Cyclophosphamide (correct)
- Doxorubicin
- Daunorubicin
Alcohol consumption can aggravate cardiomyopathy by leading to deficiencies in thiamine, magnesium, and selenium.
Alcohol consumption can aggravate cardiomyopathy by leading to deficiencies in thiamine, magnesium, and selenium.
True (A)
What is the most common post-viral cause of myocarditis?
What is the most common post-viral cause of myocarditis?
HHV-6
The pathophysiology of alcohol-induced cardiomyopathy involves the formation of ________ due to ACE polymorphism.
The pathophysiology of alcohol-induced cardiomyopathy involves the formation of ________ due to ACE polymorphism.
Match the following drugs with their associated conditions:
Match the following drugs with their associated conditions:
Which of the following is NOT a feature seen in an ECG of a patient with cardiomyopathy?
Which of the following is NOT a feature seen in an ECG of a patient with cardiomyopathy?
Peripartum cardiomyopathy has the worst prognosis among types of dilated cardiomyopathy.
Peripartum cardiomyopathy has the worst prognosis among types of dilated cardiomyopathy.
What is a significant factor associated with peripartum cardiomyopathy?
What is a significant factor associated with peripartum cardiomyopathy?
Patients with peripartum cardiomyopathy typically present during the ______ or postpartum.
Patients with peripartum cardiomyopathy typically present during the ______ or postpartum.
Match the following diagnostic tools with their main findings in cardiomyopathy:
Match the following diagnostic tools with their main findings in cardiomyopathy:
What is the most likely inheritance pattern for Dilated Cardiomyopathy (DCMP)?
What is the most likely inheritance pattern for Dilated Cardiomyopathy (DCMP)?
Toxin or nutrition-induced cardiomyopathy typically has a good prognosis.
Toxin or nutrition-induced cardiomyopathy typically has a good prognosis.
Name one type of cardiomyopathy that is associated with congenital factors.
Name one type of cardiomyopathy that is associated with congenital factors.
Deficiencies of __________ can lead to toxin/nutrition induced cardiomyopathy.
Deficiencies of __________ can lead to toxin/nutrition induced cardiomyopathy.
Match the following types of cardiomyopathy with their corresponding protein mutations:
Match the following types of cardiomyopathy with their corresponding protein mutations:
Which type of cardiomyopathy involves the heart muscle becoming enlarged and weakened?
Which type of cardiomyopathy involves the heart muscle becoming enlarged and weakened?
Hypertrophic cardiomyopathy can lead to diastolic heart failure.
Hypertrophic cardiomyopathy can lead to diastolic heart failure.
Name one clinical symptom of systolic heart failure seen in dilated cardiomyopathy.
Name one clinical symptom of systolic heart failure seen in dilated cardiomyopathy.
The gold standard for assessing the efficiency of the heart is __________.
The gold standard for assessing the efficiency of the heart is __________.
Which of the following is NOT a recognized type of cardiomyopathy?
Which of the following is NOT a recognized type of cardiomyopathy?
Match the symptoms with their associated type of heart failure:
Match the symptoms with their associated type of heart failure:
What is the primary characteristic of restrictive cardiomyopathy?
What is the primary characteristic of restrictive cardiomyopathy?
Arrhythmogenic RV dysplasia is characterized by abnormal heart function without any structural issues.
Arrhythmogenic RV dysplasia is characterized by abnormal heart function without any structural issues.
Which of the following is a common clinical feature of cardiomyopathy?
Which of the following is a common clinical feature of cardiomyopathy?
Elevation of Troponin I is a normal finding in patients with myocardial injury.
Elevation of Troponin I is a normal finding in patients with myocardial injury.
What imaging technique showcases peripheral enhancement in myocarditis?
What imaging technique showcases peripheral enhancement in myocarditis?
The presence of _____ is significant in diagnosing myocarditis according to the DALLAS criteria.
The presence of _____ is significant in diagnosing myocarditis according to the DALLAS criteria.
Match the clinical features of cardiomyopathy with their consequences:
Match the clinical features of cardiomyopathy with their consequences:
Which of the following is characteristic of asymmetrical septal hypertrophy?
Which of the following is characteristic of asymmetrical septal hypertrophy?
Mild mitral regurgitation is a feature associated with asymmetrical septal hypertrophy.
Mild mitral regurgitation is a feature associated with asymmetrical septal hypertrophy.
What is the typical cause of angina when coronary arteries appear normal?
What is the typical cause of angina when coronary arteries appear normal?
The ejection systolic murmur in asymmetrical septal hypertrophy is best auscultated at the ________.
The ejection systolic murmur in asymmetrical septal hypertrophy is best auscultated at the ________.
Match the following conditions with their associated types of obstruction:
Match the following conditions with their associated types of obstruction:
What is the most frequent cause of sudden cardiac death (SCD)?
What is the most frequent cause of sudden cardiac death (SCD)?
The severity of Hypertrophic Cardiomyopathy is more pronounced in males compared to females.
The severity of Hypertrophic Cardiomyopathy is more pronounced in males compared to females.
What mutation is primarily associated with Hypertrophic Cardiomyopathy?
What mutation is primarily associated with Hypertrophic Cardiomyopathy?
Hypertrophic Cardiomyopathy is defined as left ventricular (LV) hypertrophy of at least ______ mm without dilation.
Hypertrophic Cardiomyopathy is defined as left ventricular (LV) hypertrophy of at least ______ mm without dilation.
Match the following pediatric conditions with their characteristics:
Match the following pediatric conditions with their characteristics:
Which of the following symptoms is NOT commonly associated with increased left atrial pressure (LAP)?
Which of the following symptoms is NOT commonly associated with increased left atrial pressure (LAP)?
A reverse split S2 heart sound indicates a normal left ventricular function.
A reverse split S2 heart sound indicates a normal left ventricular function.
What would you expect to observe in the pulse of a patient with aortic stenosis?
What would you expect to observe in the pulse of a patient with aortic stenosis?
Increased left atrial pressure can lead to symptoms such as __________ on exertion and syncope.
Increased left atrial pressure can lead to symptoms such as __________ on exertion and syncope.
Match the following clinical signs with their descriptions:
Match the following clinical signs with their descriptions:
Which condition is characterized by apical hypertrophy and is observed only in Japanese individuals?
Which condition is characterized by apical hypertrophy and is observed only in Japanese individuals?
Diastolic dysfunction in cardiomyopathy is associated with an increase in left ventricular chamber size.
Diastolic dysfunction in cardiomyopathy is associated with an increase in left ventricular chamber size.
What happens to the pulmonary capillary wedge pressure (PCWP) in patients with hypertrophic cardiomyopathy?
What happens to the pulmonary capillary wedge pressure (PCWP) in patients with hypertrophic cardiomyopathy?
Hypertrophic cardiomyopathy is often associated with an increased risk of __________ due to atrial stretch and dilatation.
Hypertrophic cardiomyopathy is often associated with an increased risk of __________ due to atrial stretch and dilatation.
Match the following features of hypertrophic cardiomyopathy with their descriptions:
Match the following features of hypertrophic cardiomyopathy with their descriptions:
Which factor is considered a relieving factor for cardiac issues?
Which factor is considered a relieving factor for cardiac issues?
All murmurs decrease in intensity with Valsalva, except for murmurs in hypertrophic cardiomyopathy.
All murmurs decrease in intensity with Valsalva, except for murmurs in hypertrophic cardiomyopathy.
What is the primary mechanism of preload in hypertrophic cardiomyopathy?
What is the primary mechanism of preload in hypertrophic cardiomyopathy?
The electrical changes seen in the ECG of patients with left ventricular hypertrophy (LVH) may include ________ inversions.
The electrical changes seen in the ECG of patients with left ventricular hypertrophy (LVH) may include ________ inversions.
Match the following factors with their effect on the intensity of murmurs:
Match the following factors with their effect on the intensity of murmurs:
Study Notes
Hypertrophic Cardiomyopathy
- Common genetic cardiovascular disease worldwide
- Frequent cause of sudden cardiac death (SCD)
- Autosomal dominant inheritance
- Myosin binding protein C > myosin heavy chain mutation
- Incidence is equal in males and females
- Females tend to experience faster progression to heart failure
- 95-99% of cases are asymptomatic
- Left ventricular (LV) hypertrophy ≥ 15 mm without dilation in the absence of other cardiac/systemic diseases that could cause hypertrophy
- Complications include progression to heart failure, arrhythmias (atrial fibrillation), and sudden cardiac death (SCD)
Pediatric Conditions that Mimic HCM
- Pompe's disease: glycogen storage disease Type 2, short PR interval
- Fabry's disease
- Noonan syndrome
- Friedreich's ataxia
Clinical Features
- 95-99% of cases are asymptomatic
- Increased LAP, increased LVEDP, resulting in:
- Dyspnea on exertion/fatigue
- Orthopnea
- Paroxysmal nocturnal dyspnea (PND)
- Angina
- LVOT obstruction symptoms
- Palpitations
- Arrhythmias (atrial fibrillation)
- Syncope
Signs
- Pulse may be jerky, bisferiens
- Pulsus bisferiens: 2 peaks in systole (percussion wave > tidal wave)
- Peak resulting from obstruction by SAM
- Heart sounds:
- S1: Normal
- S2: Reverse split (LV hypertrophy → increased time to eject blood)
- S3: + (decreased filling of a non-compliant ventricle)
- S4: ++ (healthy atria in sinus rhythm contracting vigorously across a normal non-stenosed AV valve into a hypertrophied non-compliant ventricle)
Murmurs
- Ejection systolic murmur (ESM) at LLSB
- Pansystolic murmur (PSM) at apex
Dynamic Auscultation
- Principle: Intensity of ESM & LV-aorta pressure gradient
- Factors affecting gradient:
- Preload
- Afterload
- Inotropy (force of contraction)
Additional Notes
- BP: Normal
- JVP: Normal
- Double apical impulse: due to SAM
- Triple apical impulse: due to presystolic accentuation
Characteristic Features
- Asymmetric hypertrophy:
- Involves only the left side
- Affects the septal and anterior wall (while sparing the apex and posterior wall)
- Occurs due to the absence of increased afterload
- Inappropriate hypertrophy:
- Implies the condition is present in 1/3rd of cases
- LVOT obstruction:
- Absent (normal) in some cases (1/3rd)
- May be present as a dynamic obstruction in other cases (1/3rd)
Yamaguchi Disease
- A variant of HCM
- Characterized by apical hypertrophy
- Only observed in Japanese individuals
- Often associated with deep/giant T inversions in ECG
Pathophysiology
- Diastolic dysfunction:
- Concentric LV hypertrophy (LVH) - decreased LV chamber size
- Increased LV end-diastolic pressure (LVEDP)
- Increased LA pressure
- Atrial stretch and dilatation
- Increased cardiac output (25% to 40%)
- Increased risk of atrial fibrillation
- Increased PCWP
- Pulmonary edema
- Dyspnea
- Tachycardia-related dyspnea on exertion
Table of Cardiac Factors
Factor | Preload | Afterload | Inotropy |
---|---|---|---|
Preferred state | High | High | Low |
Mechanism in HCM | Decreased preload ↓ cavity size ↑LVOT obstruction ↑ gradient ↑ severity | Decreased afterload ↓ resistance ↓blood remains in LV ↓LV cavity size ↓LVOT width ↑LVOT obstruction | Increased inotropy Septum and mitral valve drawn into LVOT ↑ LVOT obstruction |
Relieving Factors
- Hydration (Increased preload)
- Venodilators
- Diuretics
Aggravating Factors
- Aggressive in BP
- Moderate-severe activity
- Inotropes: C/I in HCM to elevate BP (e.g. Digoxin)
Intensity of Murmurs
Factor | To ↓ Intensity | To ↑ Intensity |
---|---|---|
Valsalva | Standing (↓ preload) | |
Amyl nitrite | Vasodilator: (↓ afterload) | |
Isoproterenol | ↑ force of contraction | |
Hand grip / Phenylephrine | ↑ afterload |
Investigations
- ECG:
- LVH
- ST-T changes
- Deep giant T inversions (Yamaguchi disease)
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Description
This quiz covers Hypertrophic Cardiomyopathy (HCM), a common genetic cardiovascular disorder that can lead to sudden cardiac death. Explore its inheritance patterns, clinical features, and pediatric conditions that may mimic HCM. Understand the implications of this condition and key points about its management and diagnosis.