A Balanced Set of Clinical Measures and the discussions they have provoked
Hypertrophic Cardiomyopathy an update - WordPress.com fileStress Echo : normal BP response to...
Transcript of Hypertrophic Cardiomyopathy an update - WordPress.com fileStress Echo : normal BP response to...
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James Kadouch,VP MD SGLA
Carolina Underwriter Forum Charlotte NC March 16th 2017
Hypertrophic Cardiomyopathyan update
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HCM : the tip of the iceberg
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Case 1: medical history
A 36 year old non smoker male applies for life insurance.In 2007, SCD of his twin brother at 24 yo autopsy revealed HCM familial screening led to the diagnosis of HCM in his case too.He is asymptomatic except some palpitations now and then.On examination, the only abnormality is a systolic murmur 3/6 heard at the apex.
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Case 1: recent cardiac evaluation
EKG shows a sinus rhythm 65 bpm ; thin q waves I,II,III,Avf,V4,V5,V6 ; inverted TW V4,V5,V6 ; LVH ( voltage criteria ).
Cardiac MRI : mid interventricular septum thickened 20 mm with late gadolinium enhancement.
24 hours Holter EKG monitoring : 25400 isolated and monomorphic PVCs without SVT or non SVT.
Stress Echo : normal BP response to exercise, no provoked LVOT obstruction.
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Case 1 : question
What would you do ?
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Agenda
1 Classification of cardiomyopathies
2 Definition
3 Etiology - Pathophysiolgy
4 Clinical manifestations and diagnosis
5 Differential diagnosis
6 Natural history
7 SCD risk stratification
8 Therapy
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Classification
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Unknown cause(primary)
Dilated
Hypertrophic
Restrictive
Arrhythmogenic right ventriculardysplasia
Non classified ( LV non compaction, stress cardiomyopathy)
Specific disease of myocardial muscle (secondary)
Infectious
Metabolic
Systemic disease
Hereditary
Toxic
Source : Br Heart J 1980; 44:672-673
WHOs cardiomyopathies classification
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Ventricle dilatation and systolic dysfunctionDilated (congestive, DCM, IDC)
Inappropriate myocardial hypertrophy without HTN oraortic stenosis
Hypertrophic (IHSS, HCM, HOCM)
Abnormal filling and diastolic dysfunctionRestrictive (infiltrative)
Functionnal classification
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A) Normal left ventricle
B) Dilated Cardiomyopathy
C) Hypertrophic Cardiomyopathy (HCM)
D) Restrictive cardiomyopathy
Different types of cardiomyopathies
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Definition
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In an adult, HCM is defined by a wall thickness 15 mm in one or more LV myocardial segmentsas measured by any imaging technique (echocardiography, cardiac magnetic resonance imaging (CMR) or computed tomography (CT))that is not explained solely by loading conditions.
In children as in adults, the diagnosis of HCM requires an LV wall thickness more than two standard deviations greater than the predicted mean
Hypertrophic Cardiomyopathy : definition
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Diverse hypertrophy locations in HCM
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Etiology
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Etiology
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Basic unit of striated muscle : sarcomere
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Hypertrophic cardiomyopathy with left ventricular outflow tract obstruction
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Dynamic gradient outflow 30 mm Hg at rest or provokedSystole
Abnormal diastolic LV filling and LV filling pressuresDiastole
myocardial mass, filling pressures, O demand Microvascular dysfunction Concomitant epicardial obstructive CAD Myocardial bridging of coronary arteries (LAD)
Myocardial ischemia
Pathophysiology
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Clinical manifestations and diagnosis
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Asymptomatic, Echocardiographic findings, abnormal ECG, family history
Dyspnea 90% cases Chest pain 75% cases Fatigue, syncope Palpitations, dizziness less frequent
Symptomatic
Clinical patterns
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Systolic ejection murmur left lower sternal border
Sudden cardiac death Heart failure Stroke
Complications
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Clinical recognition of HCM
Sports/Other screening(4%)
Adabag et al. AJC 2006;98:1507
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ECG abnormalities precede development of LVH
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ECG abnormalities
Abnormal EKG in 92 % cases.
LVH aspect and repolarization abnormalities in 70 % cases.
Marked left axis deviation favoring an intraventricular gradient.
Thin and deep Q waves in inferior/ lateral territory in 20 to 50 % cases.
In apical HCM, giant inverted T waves, > than 10 mm depth, and very big QRS complexes inthe lateral precordial chest leads.
Atrial fibrillation in 10 % cases.
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ECG 1 HCM
Inverted T waves
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Cornell index positive
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ECG 2 HCM
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QRS V2 and V3 > 45 mm
Inverted T waves
Cornell index positive
Concave ST elevationII-III-aVF
Perpendicular QRS axis
Thin Q waves
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ECG abnormalities suggesting specific diagnoses
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ECG abnormalities suggesting specific diagnoses
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Echocardiography : Long axis parasternal view
LVLA
RV
AO
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Transthoracic echocardiography (normal heart)
RV
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LV
LA
AO
LV
RV
IVS
LVPW
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Long axis parasternal view : HCM
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Long axis parasternal view
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Long axis parasternal view
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4 Chambers apical view
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5 Chambers apical view
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Late-peaking dagger-shaped appearance
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Cardiac MRI (HCM)
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Cardiac MRI (HCM)
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Differential diagnosis
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Amyloidosis, glycogen storage disease, Anderson-Fabrydisease, infant of a diabetic mother.Similar clinical aspect
Noonans syndrome, Friedreichs ataxia, familial restrictivecardiomyopathyGenetic
HTN , hypertrophy elderly people, athletesExcessive physiologic response
Other causes of hypertrophy
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Natural history
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Annual mortality 3% / year in HCM centers, but 1% / year in general population.
Higher risk of SCD in children up to 6% / year (decreased dramatically during last decade)
In most cases, hypertrophy increases.
Usually slow clinical deterioration.
Evolution to dilated cardiomyopathy in 10-15% cases.
Natural history of HCM
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Mortsubite
Insuffisancecardiaque
ICterminale FA et AVC
Benign andStable ( normal
longevity )
PrognosticProfiles
Suddendeath
Heartfailure End Stage
AF and stroke
Clinical course and natural history
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SCD risk stratification
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SCDClinical
Morphology
Hemodynamicstatus Arrythmia
Ischemia
Genetic
SCD evaluation in HCM
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1. Unexplained recent syncope
2. Family history of SD attributable to HCM in 1 1st or 2nd degree relatives
3. Massive LV hypertrophy (wall thickness 30 mm)
4. Hypotensive or blunted blood pressure response to exercise (max SBP rest SBP < 20or 25 mm Hg)
5. Multiple repetitive non sustained ventricular tachycardia on ambulatory (Holter) ECG monitoring
Major established risk markers of SD in HCM
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A number of studies have reported a significant association with LVOTO and SCD.
Several unanswered questions remain, including the prognostic importance of provocable LVOTO and the impact of treatment (medical or invasive) on SCD.
Left ventricular outflow tractobstruction
LGE on CMR imaging is believed to represent myocardialfibrosis or scarring
Recent meta-analysis of 7 studies showed the presence ofLGE was associated with an increased risk for SCD (OR:3.41)
Late gadolinium enhancement
Some sarcomeric gene mutations may confer a higher risk ofSDGene mutations
Source : 2011 ACCF/AHA Guideline diagnosis and treatment of HCM.Circulation 2011;124:2761-96.
Other potential SCD risk modifiers
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Young age ( 30 mm
Absence of BP elevation during exercise
Profile of high risk of SCD
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No or moderate symptoms
No family history of SCD
No syncope
No NSVT ( Holter )
Intraventricular gradient < 30 mm Hg
Normal or mild enlargement of LA size
Normal exercise BP response
Moderate LVH ( < 20 mm )
Low risk patients of SCD
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Source : Adapted from Maron B, Circulation 2010 et Guide ALD5 CMH, HAS 2011
Elev
HCM : SCD prevention
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Source : http://www.doc2do.com/hcm/webHCM.html
ESC Risk Score
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http://www.doc2do.com/hcm/webHCM.html
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Therapy
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Asymptomatic : surveillance
Moderate symptoms : medical therapy ( beta-blockers, calcium-blockers, disopyramide )
With LVOTO :myectomy or alcohol septal ablation No LVOTO : heart transplant
Severe symptoms and ineffective medical therapy
Therapy for patients with low risk of SCD
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Therapy for patients with high risk of SCD : Implantable CardioverterDefibrillator ( ICD )
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Ventricular septal myectomy ( Morrow procedure )
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Alcohol Septal Ablation
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Cases study
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Case 1 : risk factors of SCD
Young age Brothers SCD
PVCsLate GDE
IVS thickness 20 mmNo SVT or non SVT
No LVOT obstructionNormal systolic BP to exercise
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Case 2 : medical history
A 27 year old Japanese soccer player applies for life insurance.He had had an isolated syncopal episode while intensely training a year ago, but his medical history was otherwise unremarkable. On examination, he appeared fit. His vital signs were normal. The apical pulse was sustained on palpation and was not displaced. Auscultation revealed an S4 heart sound.
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Case 2 : TTE
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LV
LARA
RV
RV
LV
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Kaplan-Meier 20 years survival curves in patients with apical HC
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American Journal of Cardiology 2013; 112:1271
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Case 3 : medical history
Non smoker male 40 yo , no HTN, no Rx, with a brother on dialysis.
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Case 3 : TTE
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MERCI
Slide Number 1HCM : the tip of the iceberg Case 1: medical historyCase 1: recent cardiac evaluationCase 1 : question AgendaSlide Number 7Slide Number 8Slide Number 9Slide Number 10Slide Number 11Slide Number 12Slide Number 13Slide Number 14EtiologySlide Number 16Slide Number 17Slide Number 18Slide Number 19Slide Number 20Slide Number 21Slide Number 22Slide Number 23Slide Number 24Slide Number 25Slide Number 26Slide Number 27Slide Number 28Slide Number 29Long axis parasternal view : HCMLong axis parasternal viewSlide Number 324 Chambers apical view5 Chambers apical viewLate-peaking dagger-shaped appearance Slide Number 36Cardiac MRI (HCM) Slide Number 38Slide Number 39Slide Number 40Slide Number 41Slide Number 42Slide Number 43Slide Number 44Slide Number 45Slide Number 46Slide Number 47Slide Number 48Slide Number 49Slide Number 50Slide Number 51Slide Number 52Slide Number 53Slide Number 54Slide Number 55Slide Number 56 Case 1 : risk factors of SCD Case 2 : medical history Case 2 : TTEKaplan-Meier 20 years survival curves in patients with apical HCCase 3 : medical history Case 3 : TTESlide Number 63