MYOCARDIOPATIAS Y MUERTE SUBITA CARDIACA
ESTADO ACTUAL
Gaetano Thiene, MD, FRCP University of Padua, Italy
Cadiz, May 24, 2013
• 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
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%
Institute of Pathology University of Padua
ARVC
Basso et al,
Lancet 2009
ARVC :
A DESMOSOMAL
DISEASE
9
Left dominant variant Male, 27 year old, Athlete (Hockey player) SD during a game
10 Corrado et al, Heart 2009;95;766-773
Tissue Characterization by CE- Magnetic Resonance Imaging
11
Cardiovascular Pathology, University of Padua
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
Male, 11 yrs SD at rest
VS thickness 25 mm
Cardiovascular Pathology, University of Padua
Gori F, Basso C, Thiene G. N Engl J Med 2000; 342:593-594
Enterovirus Myocarditis
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
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
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
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
Arrhythmic
MECHANISM
Cardiac Arrest
(VF/CHF)
SUBSTRATE TRIGGER
Sports disqualification,
lifestyle
Disease prevention and cure
ICD, HTx
Drug therapy, ablation
Pathophysiology of Sudden Cardiac Death in the Young
Giotto's Raising of Lazarus (c.1304), Padua, Scrovegni Chapel
“Lazare, veni foras” John’s Gospel chapter 11, lines 43-44