Lab/ECG/Xray Lab/ECG/Xray Rounds Rounds The EM Resident Provisionally The EM Resident Provisionally Known as Sean Caine Known as Sean Caine CCFP-EM CCFP-EM March 5, 2008 March 5, 2008
Mar 18, 2016
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 symptoms2 prev visits to ED in past month for similar symptoms
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 pattern”Cellular disorganization in “whorled 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 historyPrevious 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 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; 91:1596.
25 yo male severe palpitations, 25 yo male severe palpitations, lightheadedness, presyncope playing lightheadedness, presyncope playing
basketballbasketball