Lab/ECG/Xray Rounds

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Lab/ECG/Xray Rounds

• The EM Resident Provisionally Known as Sean Caine
• CCFP-EM
• March 5, 2008

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Case

• 29 yo male presents with severe lightheadedness
  and palpitations during sexual activity
• Symptoms persisted x 10-15 min
• Spontaneous resolution

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Case continued

• Additional hx
• 2 prev visits to ED in past month for similar
  symptoms
• 1st episode
• Lightheaded while mowing the lawn
• D/C home from ED after ECG and normal labs
• 2nd episode
• Syncopal watching TV
• Admitted for observation
• Had serial cardiac enzymes and cardiac monitoring
• Discharged home after negative work up
• Instructed to f/u with GP

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Case

• Normal vitals and physical exam
• ECG is obtained.

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Hypertrophic Cardiomyopathy (HCM)
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Objectives

• Background
• Etiology
• Pathophysiology
• Clinical Presentation
• ECG Findings
• Treatment

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• Thank you
• Adam Davidson and Jeff Booker

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Background

• aka Idiopathic hypertrophic subaortic stenosis,
  obstructive cardiomyopathy
• Notable cause of sudden cardiac death among
  athletes
• Accounting for 2-36 SCD among athletes
• Prevalence of 0.1-0.2 of general population
• Accounts for up to 60 of unexplained LVH
• MOST COMMON genetic cardiovascular disorder

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Background

• SCD most common for those lt35 yrs of age1
• No age is immune from SCD
• Annual mortality rates as high as 3-63

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Etiology

• 30-50 due to familial inheritance1,5
• Autosomal dominant inheritance with variable
  penetration
• mutations of 1 of 11 possible genes encoding
  proteins for myocardial sarcomere

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Pathophysiology

• Hypertrophied, nondilated left ventricle in the
  absence of another cause
• Histology
• Hypertrophied muscle cells
• Cellular disorganization in whorled pattern
• Abnormal fibrous tissue
• Scarring mimics healed MI

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Schematic

• Asymmetric septal hypertrophy (ASH)
• Systolic motion of MV anteriorleaflet (SAM)
• LVOT gradient
• Mitral regurgitation (MR)

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2 Chamber Echo
Diastole
Systole
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Morphologic Variants of HCM
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Clinical Presentation

• Can be completely asymptomatic
• Presenting Symptoms
• Dyspnea
• Exercise intolerance
• Dizziness
• Chest pain
• Presyncope/syncope
• Sudden Cardiac Death

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Clinical Presentation

• Average age at diagnosis is 30-40 yrs
• 2 diagnosed before the age of 5
• 7 before the age of 10

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Key features on history

• Previous hx of syncope
• Family history of SCD or early onset of symptoms

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Physical Examination

• S4 gallop
• Harsh crescendo-decrescendo midsystolic murmur
  loudest at apex
• LOUDER with valsalva and standing
• SOFTENS with lying, squatting, or isometric
  exercise
• Bifid arterial pulse,double/triple apical
  impulse,palpable atrial gallop

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THE ECG

• Abnormal in 90
• ECG Findings
• LVH 30
• and associated ST and T wave changes
• Deep NARROW Q waves (typically I, aVL, V5, V6)
• Left atrial enlargement 25-50

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30 yo male with 20 min of lightheadedness running
to bus
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31 yo female syncope preceded by palpitations
during physical activity
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Diagnosis

• Ultimately made by doppler Echo

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Complications

• Increased incidence of WPW
• Chronic or paroxysmal afib (10-40)
• Moderate risk for infective endocarditis
• SCD

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Pharmacotherapy

• ß blockers are first line
• Verapamil or disopyramide prescribed with caution
  when ß blocker poorly tolerated
• Afterload reducing agents with systolic
  dysfunction/CHF
• ACEI, diuretics, digoxin
• Amiodarone should be used for ventricular
  dysrhythmias
• Avoid nitrates (decrease preload and LVOT)

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Nonpharmacologic therapy

• Septal myomectomy
• Alcohol septal ablation
• ICD

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Take Home Points

• Be suspicious of unexplained LVH on ECG
• Especially in symptomatic patients
• Think HCM with narrow qwaves in lateral leads

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References

1. Ramaraj R. Hypertrophic Cardiomyopathy Etiology,
   Diagnosis, Treatment. Cardiology in Review. 2008
   14(4) 172-179.
2. Dovgalyuk J, Holstege C, Mattu A, Brady WJ. The
   electrocardiogram in the patient with syncope.
   American Journal of Emergency Medicine. 2007 25
   688-701.
3. Kelly BS, Mattu A, Brady WJ. Hypertrophic
   cardiomyopathy electrocardiographic
   manifestations and other important considerations
   for the emergency physician. American Journal of
   Emergency Medicine. 2007 2572-79.
4. Jouriles NJ. Hypertrophic Cardiomyopathy. Marx
   Rosens Emergency Medicine Concepts and Clinical
   Practice. 6th ed. 2006.
5. Niemann JT. Hypertrophic Cardiomyopathy.
   Emergency Medicine A comprehensive study guide.
   6th ed. 2004 379-380.

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Sudden Cardiac Death

• Risk factors
• Sustained VT
• Recurrent syncope (esp w/exertion)
• Family hx of 1 or more SCD
• Extreme LVH (gt30mm wall thickness)
• LVOT gradient gt30mmHg
• Abnormal BP response to exercise
• Nonsustained VT ambulatory monitor
• 0-1 RF 1 annual SCD rate
• 2 or more increased risk of SCD and prophylactic
  ICD should be considered

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Distinguishing hypertrophic cardiomyopathy (HCM)
from "Athlete's Heart"
Parameter Findings in HCM Findings in Athlete's Heart
LV wall thickness and morphology Can be gt16 mm can be heterogeneous or asymmetric across segments Typically lt16 mm, especially in women symmetric
Diastolic LV cavity lt45 mm (except in late, dilated phase) gt55 mm
LA size Enlarged Normal
LV diastolic filling pattern Impaired relaxation (EA ratio lt1, prolonged diastolic deceleration time) Normal
Response to deconditioning None LV wall thickness decreases
EKG findings Very high QRS voltages Q waves deep negative T waves Criteria for LVH but without unusual features
Family history of HCM Present (except de novo mutations) Absent
LV left ventricle LA left atrium LVH left
ventricular hypertrophy.Adapted from Maron, BJ,
Pellicia, A, Spirito, P. Circulation 1995
911596.
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25 yo male severe palpitations, lightheadedness,
presyncope playing basketball