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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