MYOCARDIOPATIAS Y MUERTE SUBITA CARDIACA ESTADO … · University of Padua ARVC . Basso et al,...
Transcript of MYOCARDIOPATIAS Y MUERTE SUBITA CARDIACA ESTADO … · University of Padua ARVC . Basso et al,...
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MYOCARDIOPATIAS Y MUERTE SUBITA CARDIACA
ESTADO ACTUAL
Gaetano Thiene, MD, FRCP University of Padua, Italy
Cadiz, May 24, 2013
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• Cardiomyopathies at risk of sudden death
• Mechanism and substrates of arrhythmic cardiac arrest
• Prevention of sudden death by early diagnosis
MYOCARDIOPATIAS Y MUERTE SUBITA CARDIACA
ESTADO ACTUAL
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Causes of Sudden Death in Young People (≤35 yrs)
Total n.590 (185 F) Veneto Region, Italy, 1980-2011
Coronary atherosclerosis 108 18,3%
Myocarditis 72 12,2%
Arrhythmogenic cardiomyopathy 58 9,8%
Hypertrophic cardiomyopathy 55 9,3%
Mitral valve prolapse 45 7,6%
Coronary artery anomalies 27 4,5%
Aortic rupture 20 3,4%
… … …
Unexplained 101 17,0%
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Institute of Pathology University of Padua
ARVC
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Basso et al,
Lancet 2009
ARVC :
A DESMOSOMAL
DISEASE
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9
Left dominant variant Male, 27 year old, Athlete (Hockey player) SD during a game
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10 Corrado et al, Heart 2009;95;766-773
Tissue Characterization by CE- Magnetic Resonance Imaging
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11
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Cardiovascular Pathology, University of Padua
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HC is now recognized to result from dominant mutations in genes encoding proteins of the
contractile apparatus
Spirito et al. N Engl J Med, 1997, 336: 775-785
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Male, 11 yrs SD at rest
VS thickness 25 mm
Cardiovascular Pathology, University of Padua
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Gori F, Basso C, Thiene G. N Engl J Med 2000; 342:593-594
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Enterovirus Myocarditis
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Epstein Barr virus (EBV)
Adenovirus
Cytomegalovirus (CMV)
Enterovirus (EV)
EV/CMV
EV/EBV
EV/CMV/EBV
Parvovirus B19 (PV
B19)/EBV
Myocarditis and Juvenile SD VIRAL ETIOLOGY
40%60%
virus negative
virus positive
1
1
1
2
11
2
1
2
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Sudden death in Athletes Veneto Region, Italy, 1981-2004
• No. cases: 55 (50 males)
• Causes:
• ARVC 12 (22%)
• Coronary artery anomalies 11 (20%)
• Coronary atherosclerosis 10 (18%)
• HCM 1 (2%)
• Unexplained 1 (2%)
• ……
Cardiovascular Pathology University of Padua Medical School-Padua,I
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Relative Risk of Sport-related SD
in ARVC
0
0,1
0,2
0,3
0,4
0,5
0,6
0,7
0,8
ARVC
SD
per
10
0,0
00
pers
on-y
ear
s
Athletes
Non-athletes
RR = 5.4 CI = 2.5-11.2
p < 0.001
Corrado et al., J Am Coll Cardiol 2003
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P=0.00
5
Su
dd
en
ca
rdia
c d
ea
th p
er
10
0,0
00
ath
lete
-ye
ars
SUDDEN DEATH IN THE ATHLETES
FROM CARDIOMYOPATHIES
0
0,2
0,4
0,6
0,8
1
1,2
1,4
Corrado et al. JAMA 2006
1979- 1986
Pre/early-screening
1987-1994
Mid-screening
1995-2002
Late-screening
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Arrhythmic
MECHANISM
Cardiac Arrest
(VF/CHF)
SUBSTRATE TRIGGER
Sports disqualification,
lifestyle
Disease prevention and cure
ICD, HTx
Drug therapy, ablation
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Pathophysiology of Sudden Cardiac Death in the Young
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Giotto's Raising of Lazarus (c.1304), Padua, Scrovegni Chapel
“Lazare, veni foras” John’s Gospel chapter 11, lines 43-44
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