Lab/ECG/Xray RoundsLab/ECG/Xray RoundsThe EM Resident Provisionally The EM Resident Provisionally

Known as Sean CaineKnown as Sean Caine

CCFP-EMCCFP-EMMarch 5, 2008March 5, 2008

Case Case

29 yo male presents with severe 29 yo male presents with severe lightheadedness and palpitations lightheadedness and palpitations

during sexual activityduring sexual activity

Symptoms persisted x 10-15 minSymptoms persisted x 10-15 min

Spontaneous resolutionSpontaneous resolution

Case continuedCase continuedAdditional hx:Additional hx:

2 prev visits to ED in past month for similar 2 prev visits to ED in past month for similar symptomssymptoms

11stst episode episodeLightheaded while mowing the lawnLightheaded while mowing the lawnD/C home from ED after ECG and normal labsD/C home from ED after ECG and normal labs

2nd episode2nd episodeSyncopal watching TVSyncopal watching TVAdmitted for observation Admitted for observation Had serial cardiac enzymes and cardiac monitoringHad serial cardiac enzymes and cardiac monitoringDischarged home after negative work upDischarged home after negative work upInstructed to f/u with GPInstructed to f/u with GP

Case Case

Normal vitals and physical examNormal vitals and physical exam

ECG is obtained….ECG is obtained….

Hypertrophic Cardiomyopathy Hypertrophic Cardiomyopathy (HCM)(HCM)

ObjectivesObjectives BackgroundBackground

EtiologyEtiology

PathophysiologyPathophysiology

Clinical PresentationClinical Presentation

ECG FindingsECG Findings

TreatmentTreatment

Thank you…Thank you…

Adam Davidson and Jeff BookerAdam Davidson and Jeff Booker

BackgroundBackground aka Idiopathic hypertrophic subaortic aka Idiopathic hypertrophic subaortic

stenosis, obstructive cardiomyopathystenosis, obstructive cardiomyopathy

Notable cause of sudden cardiac death Notable cause of sudden cardiac death among athletesamong athletes

Accounting for 2-36% SCD among athletesAccounting for 2-36% SCD among athletes

Prevalence of 0.1-0.2% of general populationPrevalence of 0.1-0.2% of general population

Accounts for up to 60% of unexplained LVHAccounts for up to 60% of unexplained LVH

MOST COMMONMOST COMMON genetic cardiovascular genetic cardiovascular disorderdisorder

BackgroundBackground

SCD most common for those <35 yrs SCD most common for those <35 yrs of ageof age11

– No age is immune from SCDNo age is immune from SCD

Annual mortality rates as high as 3-Annual mortality rates as high as 3-6%6%33

EtiologyEtiology

30-50% due to familial inheritance30-50% due to familial inheritance1,51,5

Autosomal dominant inheritance with Autosomal dominant inheritance with variable penetration variable penetration

mutations of 1 of 11 possible genes mutations of 1 of 11 possible genes encoding proteins for myocardial encoding proteins for myocardial sarcomeresarcomere

35%

15%

15%

PathophysiologyPathophysiology

Hypertrophied, nondilated left ventricle Hypertrophied, nondilated left ventricle in the absence of another causein the absence of another cause

Histology: Histology: – Hypertrophied muscle cells Hypertrophied muscle cells – Cellular disorganization in “whorled Cellular disorganization in “whorled

pattern”pattern”– Abnormal fibrous tissue Abnormal fibrous tissue – Scarring mimics healed MIScarring mimics healed MI

SchematicSchematic

Asymmetric septal Asymmetric septal hypertrophy hypertrophy (ASH)(ASH)

Systolic motion of MV Systolic motion of MV anteriorleaflet anteriorleaflet (SAM)(SAM)

LVOT gradientLVOT gradient

Mitral regurgitation Mitral regurgitation (MR)(MR)

2 Chamber Echo2 Chamber Echo

DiastoleSystole

Morphologic Variants of Morphologic Variants of HCMHCM

Clinical PresentationClinical Presentation

Can be completely asymptomaticCan be completely asymptomatic

Presenting Symptoms Presenting Symptoms – DyspneaDyspnea– Exercise intoleranceExercise intolerance– DizzinessDizziness– Chest painChest pain– Presyncope/syncopePresyncope/syncope– Sudden Cardiac DeathSudden Cardiac Death

Clinical PresentationClinical Presentation

Average age at diagnosis is 30-40 yrsAverage age at diagnosis is 30-40 yrs

2% diagnosed before the age of 52% diagnosed before the age of 5

7% before the age of 107% before the age of 10

Key features on historyKey features on history

Previous hx of syncopePrevious hx of syncope

Family history of SCD or early onset of Family history of SCD or early onset of symptomssymptoms

Physical ExaminationPhysical Examination

S4 gallopS4 gallop

Harsh crescendo-decrescendo midsystolic Harsh crescendo-decrescendo midsystolic murmur loudest at apexmurmur loudest at apex– LOUDERLOUDER with valsalva and standing with valsalva and standing– SOFTENS SOFTENS with lying, squatting, or isometric with lying, squatting, or isometric

exerciseexercise

Bifid arterial pulse,double/triple apical Bifid arterial pulse,double/triple apical impulse,palpable atrial gallopimpulse,palpable atrial gallop

THE ECGTHE ECG

Abnormal in 90%Abnormal in 90%

ECG FindingsECG Findings LVH: 30% LVH: 30%

– and associated ST and T wave changesand associated ST and T wave changes

Deep Deep NARROWNARROW Q waves (typically I, aVL, Q waves (typically I, aVL, V5, V6)V5, V6)

Left atrial enlargement: 25-50%Left atrial enlargement: 25-50%

30 yo male with 20 min of lightheadedness 30 yo male with 20 min of lightheadedness running to busrunning to bus

31 yo female syncope preceded by 31 yo female syncope preceded by palpitations during physical activitypalpitations during physical activity

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DiagnosisDiagnosis

Ultimately made by doppler EchoUltimately made by doppler Echo

ComplicationsComplications

Increased incidence of WPWIncreased incidence of WPW

Chronic or paroxysmal afib (10-40%)Chronic or paroxysmal afib (10-40%)

Moderate risk for infective Moderate risk for infective endocarditisendocarditis

SCDSCD

PharmacotherapyPharmacotherapy ββ blockers are first line blockers are first line

– Verapamil or disopyramide prescribed with Verapamil or disopyramide prescribed with caution when caution when ββ blocker poorly tolerated blocker poorly tolerated

Afterload reducing agents with systolic Afterload reducing agents with systolic dysfunction/CHFdysfunction/CHF– ACEI, diuretics, digoxinACEI, diuretics, digoxin

Amiodarone should be used for ventricular Amiodarone should be used for ventricular dysrhythmiasdysrhythmias

Avoid nitrates (decrease preload and LVOT)Avoid nitrates (decrease preload and LVOT)

Nonpharmacologic therapyNonpharmacologic therapy

Septal myomectomySeptal myomectomy

Alcohol septal ablationAlcohol septal ablation

ICDICD

Take Home PointsTake Home Points

Be suspicious of unexplained LVH on Be suspicious of unexplained LVH on ECGECG– Especially in symptomatic patientsEspecially in symptomatic patients

Think HCM with narrow qwaves in Think HCM with narrow qwaves in lateral leadslateral leads

ReferencesReferences1. 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; 25:72-79.

4. Jouriles NJ. Hypertrophic Cardiomyopathy. Marx: Rosen’s 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.

Sudden Cardiac DeathSudden Cardiac Death

Risk factorsRisk factors Sustained VTSustained VT Recurrent syncope (esp w/exertion)Recurrent syncope (esp w/exertion) Family hx of 1 or more SCDFamily hx of 1 or more SCD Extreme LVH (>30mm wall thickness)Extreme LVH (>30mm wall thickness) LVOT gradient >30mmHgLVOT gradient >30mmHg Abnormal BP response to exerciseAbnormal BP response to exercise Nonsustained VT ambulatory monitorNonsustained VT ambulatory monitor

0-1 RF = 1% annual SCD rate0-1 RF = 1% annual SCD rate 2 or more= increased risk of SCD and 2 or more= increased risk of SCD and

prophylactic ICD should be consideredprophylactic ICD should be considered

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

Parameter Findings in HCM Findings in Athlete's Heart

LV wall thickness and morphology

Can be >16 mm; can be heterogeneous or asymmetric across segments

Typically <16 mm, especially in women; symmetric

Diastolic LV cavity <45 mm (except in late, dilated phase) >55 mm

LA size Enlarged Normal

LV diastolic filling pattern

Impaired relaxation (E:A ratio <1, prolonged diastolic deceleration time)

Normal

Response to deconditioning

None LV wall thickness decreases

EKG findingsVery 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; 91:1596.

25 yo male severe palpitations, 25 yo male severe palpitations, lightheadedness, presyncope playing lightheadedness, presyncope playing

basketballbasketball