HCM GUIDELINES ESC 2014 - Livemedia.grstatic.livemedia.gr/livemedia/documents/al18598_us147... ·...
Transcript of HCM GUIDELINES ESC 2014 - Livemedia.grstatic.livemedia.gr/livemedia/documents/al18598_us147... ·...
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HCM GUIDELINES – ESC 2014HYPERTROPHIC CARDIOMYOPATHY
ASYMPTOMATIC PATIENT
Division of Inherited Cardiac DiseasesHeart Center for the Young and Athletes
A Dpt of Cardiology – University of Athens
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ASYMPTOMATIC PTS WITH TYPICAL HCM
INHERITED CV DISEASESSD FAMILIES
Clinical Cardiology
GeneticsMOLECULARCARDIOLOGY
FAMILY SCREENING
TYPICALDISEASE
SUBCLINICALDISEASE
GENE CARRIER
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HYPERTROPHIC CARDIOMYOPATHY
PREVENT THE COMPLICATION
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Asymptomatic HCMpts characteristics and complication
►Sudden cardiac death
►CVA
►LVOTgradient
►Aneurysm
►Mildly impaired LV function
►Endocarditis
►End stage HCM - systolic heart failure
Asymptomatic
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ESC
GUIDELIN
ES IN
HCM 2014MRI – LGE
APICAL ANUERYSM
EF <50%
DOUBLE MUTATION
ABPR
EPS ?
ARBITRATORS
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HCM pts in sinus rhythm and dilated left atrium
HCM GUIDELINES
2014
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HOCM ASYMPTOMATIC
MYECTOMY OR SEPTAL ABLATION
NOT RECOMMENDED .
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TREATMENT in asymptomatic
►B BLOCKERS ?
►VERAPAMIL ?
►HIGH RISK - ICD
► LA > 45mm - ANTICOAGULATION ?
► HOCM – MYECTOMY OR SEPTAL ABLATION NOT RECOMMENDED
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Network for the management of inherited CV diseases and SCD prevention in the Young
INHERITED CV DISEASESSD FAMILIES
Clinical Cardiology
GeneticsMOLECULARCARDIOLOGY
FAMILY SCREENING
TYPICALDISEASE
SUBCLINICALDISEASE
GENE CARRIER
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ESCHCM
GUIDELINES
2014
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ESCHCM
GUIDELINES
2014
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Pedigree
Symptoms
Physical
exam
ECG
Laboratory
Echo/MRI
Other
Autsomal dominant
Autosomal recessive
X-linked-Dystrophin, Danon.
Maternal
Deafness (AFD, Epicardin)
Muscle pains/weakness (Dystrophin)
Paraesthesia
Muscle weakness
Postural hypotension
Rash (lentigenes, angiokeratomata)
Ocular
Premature conduction disease
Pseudo-infarct pattern
Creatinine kinase (dystrophic, Danon, Desmin)
Serum creatinine
Proteinuria
Ferritin
Lactate
Pattern of hypertrophy
Valve disease
Pericardial effusion
Pattern of gadolinium hyperenhancement
Exercise test: premature acidosis
Endomyocardial biopsy
ESC
HCM
2014
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DIAGNOSTIC WORK UP IN CARDIOMYOPATHIES
SIMPLE LAB TESTS
RED FLAGS
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DANON DISEASE
II:3 II:4
III:1 III:2
I:1 I:2
II:2 II:1
I:3 I:4
II:5
LIVER
ENZYMES
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HCM- FABRY DISEASE
Σ
Ν
–
8
3
y
8
3
y
AF-
ΑΕΕ-
Θάνα
τος
83y
Α
ρρ
υθ
μί
ες
67
y
3
7
y
3
8
y
60
y
PA
F-
M
R-
AY
AY AY
angiokeratoma
CONCENTRIC MILD LVH
PREECXITATION
LYMPHOEDEMA
INHERITANCE GENETICS
LAB TEST PROTEINOURIA
A GALACTOSIDASE
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ASYMPTOMATIC HCM PTSWITH SUBCLINICAL FORM
INHERITED CV DISEASESSD FAMILIES
Clinical Cardiology
GeneticsMOLECULARCARDIOLOGY
FAMILY SCREENING
TYPICALDISEASE
SUBCLINICALDISEASE
GENE CARRIER
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SUBCLINICAL FORM OF HCM DUE TO EVOLUTION
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Mutation:
Arg286Cys
Q waves
SUBCLINICAL FORM OF HCM DUE TO PENETRATION
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athletic screeningpre surgery evaluation , family screening
• ATHLETE FOOTBALL• Age 16 y old
• asymptomatic
• o/e = unremarkable
• Medical history (-)
• Family history (-)
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Contrast echo
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MRI: no fibrosis
LVEDD:50
LVWmax:9-10
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Cor Angio : normal
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Abn ECG
Abn ECGCHD –48y
CHD 78y
IHD (+)
SD (-)
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Athlete with abnormal ECGTHE PUZZLE and the weight of evidence
• ASYMPTOMATIC
• YOUNG
• ECHO – LVEDD=50 mm,TDI:Ea=10
• ABNORMAL ECG (+)
• NORMAL HOLTER rhythm
• NORMAL C/P EXERCISE TEST
• NO FAMILY HISTORY OF HCM / SCD
• CLINICAL FAMILY SCREENING (+)
• DECODITIONING (?)
SUSPECTEDHCM
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MAJOR RISK FACTORS
• LVH .30 OR Z SCORE >6
• UNEXPLAINED SYNCOPE
• NSVT
• FHSD
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HCM
Abn EC
Abn ECG
CHD –48y
CHD
78y
IHD (+)
SD (-)
GENETICS
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ASYMPTOMATIC HCM NORMAL GENE CARRIERS
INHERITED CV DISEASESSD FAMILIES
Clinical Cardiology
GeneticsMOLECULARCARDIOLOGY
FAMILY SCREENING
TYPICALDISEASE
SUBCLINICALDISEASE
GENE CARRIER
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Normal Gene carriers
► Normal ECHO
► Normal ECG
► Athletic activity ????? u
LOW RISK
USA - EU
Close f/u
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TAKE HOME MESSAGEHCM ASYMPTOMATIC PTS
► DIAGNOSIS : MULTIFACTORIAL
► GENETIC TESTING IS RECOMMENDED
► RISK STRATIFICATION – ESC RISK MODEL 2014
► Dilated LA - risk of AF
► Apical Aneurysm –risk of VA’s
► Mildly impaired EF – risk of VA’s
► MANAGEMENT OF SUBCLINICAL FORMS like the typical
► NORMAL GENE CARRIERS SEEM SAFE
HCM
ESC
2014
F/U every year
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Καθ. Δ Τουσουλης