Grown
Up
Congenital
Heart
(disease)
Ad. Pat
Ped. Pat
30
70
1965
1500.000
↑ 5% anno
1250.000
1000.000
750.000
500.000
250.000
1970
1980
1990
2000
2010
JACC 2001 Warnes
JACC 2006 Williams
Ad. Pat
Ped. Pat
40
60
2005
RVEF
70%
60
50
40
30
20
10
0
8
10
12
14
16
18
20
22
24
26 ys
Millane JACC 2000
Vander Velde Eur J Epidemiol 2005
ACE/ARB treatment
Losartan … 7 pts
Enalapril ….. 14 pts
Lester AmJ C 2001
Beta/Blocker treatment
… 8 pts
Josephson Can J Card 2006
Hecther AmJC 2001
…..31 pts
Doughan Am J Card 2007
Enalapril …. 9 pts
Robinson Ped Card 2002
…. 14 pts
Bouallal Card Young 2010
Losartan .. 37 pts
Dore Circ 2005
Ramipril … 17 pts
Therrien InT J Card 08
No high-quality data support the use
It is difficult to conclude that B/blockade is beneficial
Cardiac conditions at highest risk
Type of procedure at risk
20 %
18
16
14
12
10
8
6
4
2
0
SVT
VT
Endocardits
Engelfriet Eur H J 2005
CVA
The principal reasons for 373 medical admissions to the Royal Brompton Hospital GUCH unit in 1997.
Sommerville Heart 2002
40%
35
30
25
20
15
10
5
0
Arr
Ac HF
Inf
Sync
TE
Chest
pain
Hem
Ao An
Card Arr
Oth
Kaemmerer Am JC 2008
The updated recommendations dramatically change long
established practice for primary care physicians,cardiologists,
dentists, and their patients. For ethical reasons, these
practitioners need to discuss the potential benefit and harm of
antibiotic prophylaxis with their patients before a final decision
is made. Following informed review and discussion, some
patients (and also physicians) may wish to continue with
routine prophylaxis in the individual case, and these views
should be respected.
Br Med Bull. 2008;85:151-80.
Adult congenital heart disease: a 2008 overview.
Bédard E, Shore DF, Gatzoulis MA.
Source
Adult Congenital Heart Center and Center for Pulmonary Arterial
Hypertension, Royal Brompton Hospital, Sydney Street, London SW3
6NP, UK
Current ACC/AHA GL are much more restrictive concernig
the use of prophylactic antibiotics. However , we advocate a
prudent approach for endocarditis prohylaxis in patients
with CHD
Good oral hygiene and
regular dental review
have an essential role in
reducing the risk of IE.
It appears plausible that a large proportion
of IE-causing bacteraemia may derive
from daily routine activities such as tooth
brushing, flossing….
In the majority of patients, no potential
index procedure preceding the first clinical
appearance of IE can be identified
Transient bacteraemia occurs frequently
in the context of these daily routine
activities
Atrial septal defect
Interventional options:
surgery or device closure
Unresolved issues:
surgery vs device closure
30 %
25
20
Mayo
Toronto
15
10
5
0
< 20
21-30
31-40
41-50
51-60
61-70
> 70 ys
JACC 2001 Warnes
Y
40
> 50 y
< 50 y
35
30
25
21
20
15
79
10
5
0
ASD
VSD
ToF
CoAo
TGA
Marfan
Fontan
Cyan. Def
Engelfriet Eur H J 2005
Ebstein’s anomaly
RV to PA conduits
VO2 ml/kg/m
50
45
40
35
30
25
20
15
10
5
0
Normali
Normali
NYHA I
NYHA I
NYHA II
NYHA II
NYHA III
GUCH età media 33 y
NYHA III
Pazienti HF età media 59 y
….all should have a measurement of exercise physiology
Diller Circulation 2005
Non cardiac
Peri op
CV
Analyzing the mortality causes in
GUCH pts it appears that
cardiovascular death is the most
frequent: 65% of all deaths, excluding
the perioperative cardiac surgery
deaths.
17%
18%
65%
Non cardiac
Peri op
Other CV
17%
26%
HF
SD
The most common mode is SD, followed
by progressive HF
18%
21%
18%
Am J Cardiol 2000 Oechslin
Specific criteria for ICD implantation for primary
prevention have not been well defined yet
ICD therapy in adult patients with ToF
Witte Europace 2008
ICD in Tetralogy of Fallot
Khairy Circ 2008
SD and ICD in TGA With Intra-atrial
Baffles: A Multicenter Study
Khairy Circ Arr 2008
...inappropriate anti-tachycardia pacing
delivery (20%) and inappropriate
cardioversion (25 %) ...
Conclusion :Tetralogy of Fallot patients
have a higher risk of inappropriate
therapies …..
36 patients (29.8%)
experienced
complications, of which 6
(5.0%) were acute, 25 (20.7%)
were late lead-related, and 7
(5.8%) were late generatorrelated complications.
Conclusions:…late leadrelated complications are
common.
14 patients (37.8%)
experienced complications: 5
(13.5%) acute, 1 (2.7%) late
generator related, and 12
(32.4%) late lead related.
Cardiologo
pediatra
GUCH
Cardiologo
dell’adulto
IGG
RETE INTERAZIENDALE
per la GESTIONE dei GUCH
Ambulatorio per i GUCH
ASL 3 - Nervi
ICLAS
Cardiologo Pediatra
GUCH
Cardiologo dell’Adulto
ASl 3
IGG
RETE INTERAZIENDALE
per la GESTIONE dei GUCH
Ambulatorio per i GUCH
ASL 3 - Nervi
ASl 3
ICLAS
Cardiologo Pediatra
GUCH
Cardiologo dell’Adulto