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Hypertrophic cardiomyopathy: A clinical and genetic update

doi: 10.1097/01.NPR.0000429833.69138.5b
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INSTRUCTIONS Hypertrophic cardiomyopathy: A clinical and genetic update


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Hypertrophic cardiomyopathy: A clinical and genetic update

General Purpose: To provide information on HCM. Learning Objectives: After reading the article and taking this test, the reader should be able to: 1. Discuss the pathophysiology and criteria for diagnosis of HCM. 2. Recognize the clinical presentation and recommended treatment of a patient with HCM.

  1. A recognizable trait of HCM is LVH usually greater than or equal to
    1. 5 mm.
    2. 7 mm.
    3. 12 mm.
    4. 15 mm.
  2. Which statement about HCM is true?
    1. HCM is the second most common inherited heart condition.
    2. Most individuals with HCM reach a normal life expectancy.
    3. The ethnic group most commonly affected by HCM is Blacks.
    4. HCM is a major cause of SCH in children under the age of 15.
  3. The most commonly reported symptom of HCM in otherwise young, healthy adults without any underlying cardiac conditions is
    1. dyspnea.
    2. chest pressure.
    3. VT.
    4. palpitations.
  4. A drop in BP in HCM patients may cause
    1. atrial dysrhythmias.
    2. chest pain.
    3. VT.
    4. syncope.
  5. VT in HCM patients may result from
    1. diastolic dysfunction.
    2. right atrial hypertrophy.
    3. mitral regurgitation.
    4. septal hypertrophy.
  6. Physical exam on a patient with HCM reveals a
    1. cardiac murmur that decreases during Valsalva.
    2. point of maximal impulse that is forceful and sustained.
    3. palpable S3 gallop.
    4. decrescendo diastolic murmur.
  7. The murmur that is most prominent above the left sternal border is from
    1. subvalvular aortic stenosis.
    2. rheumatic valvulitis.
    3. valvular aortic stenosis.
    4. supravalvular aortic stenosis.
  8. An ECHO can be used for all of the following except to
    1. measure the shape of the heart.
    2. visualize valve function.
    3. measure the partial pressure of oxygen in the heart.
    4. visualize blood flow.
  9. Which ECHO finding is consistent with the major diagnostic criteria for HCM?
    1. posterior wall thickness of 10 mm
    2. SAM
    3. anterior septum thickness of 8 mm
    4. free wall thickness of 12 mm
  10. The genetic basis of HCM may be confirmed via
    1. ECHO.
    2. MRI.
    3. cardiac catheterization.
    4. cardiac exercise stress test.
  11. An electrophysiology study will provide specific details on
    1. the presence of inducible dysrhythmias.
    2. structural changes within the heart.
    3. underlying myocardial ischemia.
    4. the heart's degree of functionality.
  12. Diastolic dysfunction may cause
    1. palpitations.
    2. shortness of breath.
    3. loss of consciousness.
    4. ventricular dysrhythmias.
  13. The abnormal BP response during exercise stress testing for an HCM patient is defined by a
    1. fall in the systolic BP.
    2. fall in the diastolic BP.
    3. rise in the systolic BP.
    4. rise in the diastolic BP.
  14. Which dysrhythmia is very common in HCM patients?
    1. junctional tachycardia
    2. premature atrial contractions
    3. atrial fibrillation
    4. wandering atrial pacemaker
  15. Which class of drugs has traditionally been used to treat HCM symptoms?
    1. angiotensin-converting enzyme inhibitors
    2. catecholamines
    3. anticoagulants
    4. beta-blockers
  16. Verapamil helps HCM symptoms by
    1. increasing left ventricular contractility.
    2. causing ventricular relaxation.
    3. augmenting outflow obstruction.
    4. causing a positive chronotropic effect.
  17. What procedure has significantly eased symptoms in more than 90% of HCM patients?
    1. alcohol septal ablation
    2. heart transplantation
    3. an ICD
    4. septal myectomy
  18. Which statement about the genetics of HCM is true?
    1. It is inherited in an autosomal recessive manner.
    2. The MYBPC3 is the gene most frequently associated with HCM.
    3. The current methods of genetic testing can detect all types of mutations.
    4. There is a 25% risk of transmitting HCM to offspring in those with positive cardiogenetic test results.


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