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Lab/ECG/Xray Rounds. The EM Resident Provisionally Known as Sean Caine CCFP-EM March 5, 2008. Case. 29 yo male presents with severe lightheadedness and palpitations during sexual activity Symptoms persisted x 10-15 min Spontaneous resolution. Case continued. Additional hx:

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Lab ecg xray rounds

Lab/ECG/Xray Rounds

The EM Resident Provisionally Known as Sean Caine

CCFP-EM

March 5, 2008


Case

29 yo male presents with severe lightheadedness and palpitations during sexual activity

Symptoms persisted x 10-15 min

Spontaneous resolution


Case continued
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


Case

Normal vitals and physical exam

ECG is obtained….



Objectives
Objectives

  • Background

  • Etiology

  • Pathophysiology

  • Clinical Presentation

  • ECG Findings

  • Treatment


Thank you…

Adam Davidson and Jeff Booker


Background
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


  • Background1
    Background

    • SCD most common for those <35 yrs of age1

      • No age is immune from SCD

    • Annual mortality rates as high as 3-6%3


    Etiology
    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


    15%

    15%

    35%


    Pathophysiology
    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


    Schematic

    Asymmetric septal hypertrophy (ASH)

    Systolic motion of MV anteriorleaflet (SAM)

    LVOT gradient

    Mitral regurgitation (MR)

    Schematic


    2 chamber echo
    2 Chamber Echo

    Systole

    Diastole



    Clinical presentation
    Clinical Presentation

    • Can be completely asymptomatic

    • Presenting Symptoms

      • Dyspnea

      • Exercise intolerance

      • Dizziness

      • Chest pain

      • Presyncope/syncope

      • Sudden Cardiac Death


    Clinical presentation1
    Clinical Presentation

    • Average age at diagnosis is 30-40 yrs

    • 2% diagnosed before the age of 5

    • 7% before the age of 10


    Key features on history
    Key features on history

    Previous hx of syncope

    Family history of SCD or early onset of symptoms


    Physical examination
    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


    The ecg
    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%




    ????? physical activity

    ?????


    Diagnosis
    Diagnosis physical activity

    • Ultimately made by doppler Echo


    Complications
    Complications physical activity

    • Increased incidence of WPW

    • Chronic or paroxysmal afib (10-40%)

    • Moderate risk for infective endocarditis

    • SCD


    Pharmacotherapy
    Pharmacotherapy physical activity

    • β blockers are first line

      • Verapamil or disopyramide prescribed with caution when β 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)


    Nonpharmacologic therapy
    Nonpharmacologic therapy physical activity

    • Septal myomectomy

    • Alcohol septal ablation

    • ICD


    Take home points
    Take Home Points physical activity

    • Be suspicious of unexplained LVH on ECG

      • Especially in symptomatic patients

    • Think HCM with narrow qwaves in lateral leads


    References
    References physical activity

    • Ramaraj R. Hypertrophic Cardiomyopathy: Etiology, Diagnosis, Treatment. Cardiology in Review. 2008; 14(4): 172-179.

    • Dovgalyuk J, Holstege C, Mattu A, Brady WJ. The electrocardiogram in the patient with syncope. American Journal of Emergency Medicine. 2007; 25: 688-701.

    • Kelly BS, Mattu A, Brady WJ. Hypertrophic cardiomyopathy: electrocardiographic manifestations and other important considerations for the emergency physician. American Journal of Emergency Medicine. 2007; 25:72-79.

    • Jouriles NJ. Hypertrophic Cardiomyopathy. Marx: Rosen’s Emergency Medicine: Concepts and Clinical Practice. 6th ed. 2006.

    • Niemann JT. Hypertrophic Cardiomyopathy. Emergency Medicine: A comprehensive study guide. 6th ed. 2004: 379-380.


    Sudden cardiac death
    Sudden Cardiac Death physical activity

    • Risk factors

      • Sustained VT

      • Recurrent syncope (esp w/exertion)

      • Family hx of 1 or more SCD

      • Extreme LVH (>30mm wall thickness)

      • LVOT gradient >30mmHg

      • 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


  • Distinguishing hypertrophic cardiomyopathy (HCM) from "Athlete's Heart"

    LV: left ventricle; LA: left atrium; LVH: left ventricular hypertrophy.Adapted from Maron, BJ, Pellicia, A, Spirito, P. Circulation 1995; 91:1596.



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