Medicine Marrow Pg 331-340 (Cardiology)
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Questions and Answers

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.

    True

    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.

    <p>acetaldehyde</p> Signup and view all the answers

    Match the following drugs with their associated conditions:

    <p>Doxorubicin = Hodgkins lymphoma Daunorubicin = Non-Hodgkins lymphoma Trastuzumab = Carcinoma breast Cyclophosphamide = AML</p> Signup and view all the answers

    Which of the following is NOT a feature seen in an ECG of a patient with cardiomyopathy?

    <p>Sinus bradycardia</p> Signup and view all the answers

    Peripartum cardiomyopathy has the worst prognosis among types of dilated cardiomyopathy.

    <p>False</p> Signup and view all the answers

    What is a significant factor associated with peripartum cardiomyopathy?

    <p>Age at pregnancy over 30 years</p> Signup and view all the answers

    Patients with peripartum cardiomyopathy typically present during the ______ or postpartum.

    <p>third trimester</p> Signup and view all the answers

    Match the following diagnostic tools with their main findings in cardiomyopathy:

    <p>ECG = Small voltage complexes, sinus tachycardia Echo = Cardiomegaly and hypokinesia MRI/Angiogram = Ruling out CAD Angiogram = Assessing coronary artery function</p> Signup and view all the answers

    What is the most likely inheritance pattern for Dilated Cardiomyopathy (DCMP)?

    <p>Autosomal dominant</p> Signup and view all the answers

    Toxin or nutrition-induced cardiomyopathy typically has a good prognosis.

    <p>False</p> Signup and view all the answers

    Name one type of cardiomyopathy that is associated with congenital factors.

    <p>Congenital cardiomyopathy</p> Signup and view all the answers

    Deficiencies of __________ can lead to toxin/nutrition induced cardiomyopathy.

    <p>Thiamine</p> Signup and view all the answers

    Match the following types of cardiomyopathy with their corresponding protein mutations:

    <p>DCMP = Titin ARVD = Desmosome LV non compaction = Tafazzine Ischemia related = No protein mutation</p> Signup and view all the answers

    Which type of cardiomyopathy involves the heart muscle becoming enlarged and weakened?

    <p>Dilated cardiomyopathy</p> Signup and view all the answers

    Hypertrophic cardiomyopathy can lead to diastolic heart failure.

    <p>True</p> Signup and view all the answers

    Name one clinical symptom of systolic heart failure seen in dilated cardiomyopathy.

    <p>Fatigue</p> Signup and view all the answers

    The gold standard for assessing the efficiency of the heart is __________.

    <p>MRI</p> Signup and view all the answers

    Which of the following is NOT a recognized type of cardiomyopathy?

    <p>Coronary artery cardiomyopathy</p> Signup and view all the answers

    Match the symptoms with their associated type of heart failure:

    <p>Fatigue = Systolic heart failure Dyspnoea = Diastolic heart failure Edema = Diastolic heart failure Tachycardia = Systolic heart failure</p> Signup and view all the answers

    What is the primary characteristic of restrictive cardiomyopathy?

    <p>Decreased ability of the heart to fill with blood</p> Signup and view all the answers

    Arrhythmogenic RV dysplasia is characterized by abnormal heart function without any structural issues.

    <p>False</p> Signup and view all the answers

    Which of the following is a common clinical feature of cardiomyopathy?

    <p>Subclinical presentation</p> Signup and view all the answers

    Elevation of Troponin I is a normal finding in patients with myocardial injury.

    <p>False</p> Signup and view all the answers

    What imaging technique showcases peripheral enhancement in myocarditis?

    <p>MRI</p> Signup and view all the answers

    The presence of _____ is significant in diagnosing myocarditis according to the DALLAS criteria.

    <p>inflammation</p> Signup and view all the answers

    Match the clinical features of cardiomyopathy with their consequences:

    <p>Exertional dyspnea = Increased shortness of breath during activity Refractory hypotension = Indicates poor prognosis Giant cell myocarditis = Rapidly progressive heart failure Sinus tachycardia on ECG = Elevated heart rate at rest</p> Signup and view all the answers

    Which of the following is characteristic of asymmetrical septal hypertrophy?

    <p>High velocity jet across LVOT</p> Signup and view all the answers

    Mild mitral regurgitation is a feature associated with asymmetrical septal hypertrophy.

    <p>True</p> Signup and view all the answers

    What is the typical cause of angina when coronary arteries appear normal?

    <p>microvascular dysfunction</p> Signup and view all the answers

    The ejection systolic murmur in asymmetrical septal hypertrophy is best auscultated at the ________.

    <p>lower left sternal border (LLSB)</p> Signup and view all the answers

    Match the following conditions with their associated types of obstruction:

    <p>Aortic stenosis = Valvular obstruction Hypertrophic Cardiomyopathy (HCM) = Subvalvular obstruction Williams' syndrome = Supravalvular obstruction Asymmetrical septal hypertrophy = Subvalvular obstruction</p> Signup and view all the answers

    What is the most frequent cause of sudden cardiac death (SCD)?

    <p>Hypertrophic Cardiomyopathy</p> Signup and view all the answers

    The severity of Hypertrophic Cardiomyopathy is more pronounced in males compared to females.

    <p>False</p> Signup and view all the answers

    What mutation is primarily associated with Hypertrophic Cardiomyopathy?

    <p>Myosin binding protein C</p> Signup and view all the answers

    Hypertrophic Cardiomyopathy is defined as left ventricular (LV) hypertrophy of at least ______ mm without dilation.

    <p>15</p> Signup and view all the answers

    Match the following pediatric conditions with their characteristics:

    <p>Pompe's disease = Glycogen storage disease Type 2, Short PR interval Fabry's disease = Lysosomal storage disease that can mimic HCM Noonan syndrome = Genetic condition associated with congenital heart defects Friedreich's ataxia = Neuromuscular disorder presenting with scoliosis and heart issues</p> Signup and view all the answers

    Which of the following symptoms is NOT commonly associated with increased left atrial pressure (LAP)?

    <p>Weight gain</p> Signup and view all the answers

    A reverse split S2 heart sound indicates a normal left ventricular function.

    <p>False</p> Signup and view all the answers

    What would you expect to observe in the pulse of a patient with aortic stenosis?

    <p>Jerky pulse</p> Signup and view all the answers

    Increased left atrial pressure can lead to symptoms such as __________ on exertion and syncope.

    <p>dyspnea</p> Signup and view all the answers

    Match the following clinical signs with their descriptions:

    <p>Jerky pulse = Characterized by a sudden drop and rise in pulse wave Pulsus bisferiens = Two distinct peaks in systole S3 heart sound = Indicates reduced filling of the ventricle S4 heart sound = Caused by vigorous atrial contraction in a non-compliant ventricle</p> Signup and view all the answers

    Which condition is characterized by apical hypertrophy and is observed only in Japanese individuals?

    <p>Yamaguchi disease</p> Signup and view all the answers

    Diastolic dysfunction in cardiomyopathy is associated with an increase in left ventricular chamber size.

    <p>False</p> Signup and view all the answers

    What happens to the pulmonary capillary wedge pressure (PCWP) in patients with hypertrophic cardiomyopathy?

    <p>It increases.</p> Signup and view all the answers

    Hypertrophic cardiomyopathy is often associated with an increased risk of __________ due to atrial stretch and dilatation.

    <p>atrial fibrillation</p> Signup and view all the answers

    Match the following features of hypertrophic cardiomyopathy with their descriptions:

    <p>Asymmetric hypertrophy = Involves only the left side, affecting septal and anterior wall Increased cardiac output = Ranges from 25% to 40% in some cases Dynamic obstruction = Present in some cases, affecting left ventricular outflow tract Deep/giant T inversions = Associated with Yamaguchi disease in the ECG</p> Signup and view all the answers

    Which factor is considered a relieving factor for cardiac issues?

    <p>Hydration</p> Signup and view all the answers

    All murmurs decrease in intensity with Valsalva, except for murmurs in hypertrophic cardiomyopathy.

    <p>True</p> Signup and view all the answers

    What is the primary mechanism of preload in hypertrophic cardiomyopathy?

    <p>Decreased preload due to reduced venous return.</p> Signup and view all the answers

    The electrical changes seen in the ECG of patients with left ventricular hypertrophy (LVH) may include ________ inversions.

    <p>deep giant T</p> Signup and view all the answers

    Match the following factors with their effect on the intensity of murmurs:

    <p>Valsalva = Decreases intensity Isoproterenol = Increases intensity Hand grip = Increases intensity Amyl nitrite = Decreases intensity</p> Signup and view all the answers

    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.

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