Diapositiva 1

1
ADULT WITH CONGENITAL HEART DISEASE
INTRODUCTION OF THE PROBLEM
Pediatric Cardiology Department Adult with
Congenital Heart Disease IRCCS Policlinico San
Donato Dr Massimo Chessa
2
Adults with Congenital Heart Disease
More than 75.000 ACHD in Italy 1200-1600
adolescents enter adulthood every year and
require life-long care More than 85 of infants
are expected to reach adulthood
Pediatric Cardiology Department Adult with
Congenital Heart Disease Dr Massimo Chessa
3
Workload is Increasing 400 in Canada
Pediatric Pts numbers
ACHD Pts numbers
1980
2000
2020
G. Webb
Pediatric Cardiology Department Adult with
Congenital Heart Disease Dr Massimo Chessa
4
An increasing problem
Advances
Diagnosis
Therapy
Survival
Pediatric Cardiology Department Adult with
Congenital Heart Disease Dr Massimo Chessa
5
AN INCREASING PROBLEM
The first cause of such increasing number of
patients is SURGERY
Pediatric Cardiology Department Adult with
Congenital Heart Disease Dr Massimo Chessa
6
Extraordinary Management Advances Improved
Survival Rate
Warnes CA, et al. 32th Bethesda Conference, JACC
2001 371170-5
Pediatric Cardiology Department Adult with
Congenital Heart Disease Dr Massimo Chessa
7
First of all improved Surgical Mortality
Adults 30 20 10 5 4
Children 90 70 40 10 5
Year 1960 1970 1980 1990 2000
Pediatric Cardiology Department Adult with
Congenital Heart Disease Dr Massimo Chessa
8
AN INCREASING PROBLEM
It is important to outline that most pts with CHD
who have had their lives transformed by surgical
intervention, had reparative and not corrective
surgery
Pediatric Cardiology Department Adult with
Congenital Heart Disease Dr Massimo Chessa
9
Surgery is corrective, if.
..ventricular function is normal
..life expetancy is normal!
..there is no need for tharapeutic measures
during f-up
Pediatric Cardiology Department Adult with
Congenital Heart Disease Dr Massimo Chessa
10
Corrective Surgery.
Atrial Septal defect Ventricular Septal
Defect Patent Ductus Arteriosus
..if treated during childhood!!!
Pediatric Cardiology Department Adult with
Congenital Heart Disease Dr Massimo Chessa
11
Residuae
Sequelae
Mechanical
Electrophysiological
Functional
Pediatric Cardiology Department Adult with
Congenital Heart Disease Dr Massimo Chessa
12
An increasing problem
Pediatric Cardiology Department Adult with
Congenital Heart Disease Dr Massimo Chessa
13
Pediatric Cardiology Department Adult with
Congenital Heart Disease Dr Massimo Chessa
14
Why an ACHD is hospitalized
Chessa M, et al. J Cardiovasc Med (Hagerstown).
2006 Sep7(9)701-5.
Pediatric Cardiology Department Adult with
Congenital Heart Disease Dr Massimo Chessa
15
List of diseases treated by Cath or Surgery
Chessa M, et al. J Cardiovasc Med (Hagerstown).
2006 Sep7(9)701-5.
Pediatric Cardiology Department Adult with
Congenital Heart Disease Dr Massimo Chessa
16
Reasons of death 197 pts Mean age at death 37.3
14.6 (18-80)
Oechslin E, et al. Am J Cardiol 2000861111-6
Pediatric Cardiology Department Adult with
Congenital Heart Disease Dr Massimo Chessa
17
Congenital Heart Disease Is a continuum From
Fetal Life Until Adulthood
Pediatric Cardiology Department Adult with
Congenital Heart Disease Dr Massimo Chessa
18
Congenital Heart Disease Is a continuum From
Fetal Life Until Adulthood
CHILDHOOD TRANSITION ADULTHOOD
0
12
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Pediatricians
Pediatricians GUCH Cardiologist Nursing
GUCH Cardiologist Community Cardiologist Primary
Caregivers
Pediatric Cardiology Department Adult with
Congenital Heart Disease Dr Massimo Chessa
19
It is mandatory a correct organization to follow
this kind of patients
Pediatric Cardiology Department Adult with
Congenital Heart Disease Dr Massimo Chessa
20
National Centre for 5 to 10 milion people
Regional Centre with some expertise and facilities
Primary Caregivers
Community Cardiologist
Pediatric Cardiology Department Adult with
Congenital Heart Disease Dr Massimo Chessa
21
A National Adult CHD Centre must include
Physicians with specific training
Dedicated surgeons
Cath Lab with dedicated interventionists
Specific appropriate links for provision of non
cardiac surgery, integrated team of high-risk
obstetricians, anesthetists and adult CHD
cardiologists
EF lab with dedicated specialists
Facilities for Cardio MR and CT scan
22
Types of congenital heart defects of great
complexity these patients should be seen
regularly at the adult congenital heart disease
centres.
Conduits, valved or nonvalved Cyanotic congenital
heart (all forms) Double outlet ventricles Fontan
procedures Mitral atresia Single
ventricle Pulmonary atresia Pulmonary vascular
obstructive diseases Transposition of the great
arteries Tricuspid atresia Truncus
arteriosus Eisenmenger syndrome
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Types of congenital heart defects of moderate
severity these patients should be seen
periodically at general hospitals with some
expertise (Regional GUCH centres)
Aorto-left ventricular fistulae Anomalous
pulmonary venous drainage (partial or
total) Atrioventricular canal defects (partial or
complete) Coarctation of the aorta Ebsteins
anomaly Infundibular right ventricular outflow
obstruction of significance Ostium primum atrial
septal defect Patent dusctus arteriosus Pulmonary
valve regurgitation (moderate to severe) Sinus of
Valsalva fistula/aneurysm  
Sinus venosus atrial septal defect Supra valvar
or subvalvar aortic stenosis Tetralogy of
Fallot Ventricular septal defect with Absent
valve or valves Aortic regurgitation Coarctation
of the aorta Mitral disease Right ventricular
outflow tract obstruction Straddling
tricuspid/mitral valve Subaortic stenosis
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Types of simple congenital heart defects these
patients can usually be cared for in the general
medical community.
Isolated congenital aortic valve disease Isolated
congenital mitral valve disease Isolated patent
foramen ovale or small atrial septal
defect Isolated small ventricular septal
defect Mild pulmonary stenosis Previously legated
or occluded ductus arteriosus Repaired atrial
septal defect without residua Repaired
ventricular septal defect
(modified from Conelly MS, et al. Canadian
Consensus Conference on Adult Congenital Heart
Disease, 1996. Can J Cardiol 1998 14395-452).
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Plan what to do
Pediatric Cardiology Department Adult with
Congenital Heart Disease Dr Massimo Chessa
26
.they born with a CHD but they become Adult with
CHD!!!
Pediatric Cardiology Department Adult with
Congenital Heart Disease Dr Massimo Chessa
27
Infundibular stenosis VSD Overiding aorta RV
hypertrophy
Pediatric Cardiology Department Adult with
Congenital Heart Disease Dr Massimo Chessa
28
Palliation
BTS BTM Waterston Potts shunt Procedura di Brock
Pediatric Cardiology Department Adult with
Congenital Heart Disease Dr Massimo Chessa
29
Palliation

• PA distorsion
• PH
• Cyanosis
• LV overload
• RVP overload

Biventricular disfunction with risk of sudden
death
Pediatric Cardiology Department Adult with
Congenital Heart Disease Dr Massimo Chessa
30
Complete reparation Different approaches during
the years Right ventriculotomy and transanular
patch
Combined approach Transatrial-transpulmonary
sometime it is necessary a small ventriculotomy
with a transanular patch
Pediatric Cardiology Department Adult with
Congenital Heart Disease Dr Massimo Chessa
31
Survival rate 86 at 32 yrs 85 at 36
yrs Murphy JG et al. N Engl J Med 1993329593-9
Pediatric Cardiology Department Adult with
Congenital Heart Disease Dr Massimo Chessa
32
Residual problems

• Residual VSD
• Residual Infundibular Stenosis
• PA branches stenosis
• Severe PR
• Ao dilatation
• Arrhythmias and SD

Pediatric Cardiology Department Adult with
Congenital Heart Disease Dr Massimo Chessa
33
What to do?
This is the most difficult and also the most
important step planning a correct follow-up
strategy.
Mandatory a) share follow-up protocols b) have
a shared database
Pediatric Cardiology Department Adult with
Congenital Heart Disease Dr Massimo Chessa
34
What to do
Tetralogy of Fallot (FU annual ) All patients
require endocarditis prophylaxis 22qD FISH
test (bloods to be drawn at Paul Wood Ward by
GUCH SHO) if patient or spouse contemplate
pregnancy Di George syndrome more common when
right aortic arch or pulmonary atresia present
Pediatric Cardiology Department Adult with
Congenital Heart Disease Dr Massimo Chessa
35
What to do
Tetralogy of Fallot (FU annual ) Each
Visit Sustained palpitations? Presyncope? Exer
tional dyspnoea? Exam To and fro murmur in the
pulmonary area? Tricuspid regurgitation? Dia
stolic murmur of aortic regurgitation? Right
heart failure? BP and which arm If unrepaired
tetralogy, check resting O2 saturations (after 5
mins of rest) ECG Rhythm, QRS duration (if
maximum gt 180 ms, or QRS increase from recent
ECGs is more than 3.5 ms/year discuss with
consultant) Holter If clinically indicated,
not routinely
Pediatric Cardiology Department Adult with
Congenital Heart Disease Dr Massimo Chessa
36
What to do
Tetralogy of Fallot (FU annual ) 2-3
yearly CXR Cardiothoracic ratio, right of left
aortic arch, dilated ascending aorta? dilated
main or central pulmonary arteries? Echo RV
size and function Long-axis function Degree of
pulmonary and tricuspid regurgitation Gradient
across right ventricular outflow tract Estimated
systolic RV pressure (from tricuspid
regurgitation) Residual VSD LV size and
function RA/LA size and function
37
What to do
Tetralogy of Fallot (FU annual )
Baseline MRI Assessment Exercise test with
maximum O2 uptake and anaerobic threshold Signal
Average ECG Tests to be arranged following
consent (on a Friday day-case basis) Please
inform Drs . (or copy your letter to them
in their absence)
Pediatric Cardiology Department Adult with
Congenital Heart Disease Dr Massimo Chessa
38
What to do

• Tetralogy of Fallot (FU annual )
• Discuss with consultant if
• New symptoms (exertional dyspnoea, palpitations,
  presyncope) develop progressive RV dilation,
  early RV dysfunction is present
• New onset tricuspid regurgitation is documented
• and if QRS gt 180 ms, or QRS increase gt than 3.5
  ms/year (from recent ECGs)

Pediatric Cardiology Department Adult with
Congenital Heart Disease Dr Massimo Chessa
39
Paracas-Perù
Thank you for your attention Massimo Chessa