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SC BROWN
PAEDIATRIC CARDIOLOGY
UFS
Introduction

congenital heart disease - lifelong care
○ frequency 1%
- USA: 2.9mil pa
- Netherlands 20 000 GUCH – only 8000 seen
- SA: 47million = 470 000

challenges:
○ best location
○ appropriate antenatal care
○ securing infrastructure

3 most common problems
 neglected patient
 weak infrastructure
 family planning & management of pregnancy
neglected patient
lost to follow up
 non education

○ patient
○ families

adult physicians: no idea
 SOLUTIONS
○ educate patient & families
○ orderly transfer
infrastructure
more survivors of CHD
 few clinics for GUCH

○ no experience
○ shortage of skilled personnel
no orderly way of training
 multidisciplinary approach

contraception & pregnancy

GUCH – 50% female
 problems
○ contraceptive options
○ cardiac risks
conditions in which pregnancy risk is
WHO 4
* Pulmonary arterial hypertension of any cause
* Severe systemic ventricular dysfunction
NYHA III–IV or LVEF ≤ 30%
* Previous peripartum cardiomyopathy with any residual impairment of
left ventricular function
* Severe left heart obstruction
* Marfan syndrome with aorta dilated > 40 mm
LVEF, left ventricular ejection fraction; NYHA, New York Heart
Association
Heart 2006;92:1520-25
GUCH: Facts & figures
age distribution (Florida)
Single
ventricle
5%
PFO
Ebstein 2%
Diagnosis
TGA
9%
PuV
8%
2%
CoA
5%
Tet
21%
AVSD
5%
ASD
9%
AoV
9%
other
12%
VSD
8%
TA
5%
number of re-operations
Problems in GUCH

ventricular function
○ poor imaging

arrhythmias
○ SVT
○ early electrical cardioversion

cyanosis





policythaemia – phlebotomy (hct > 65)
haemostasis
renal function
gallstones
orthopaedic

pulmonary vascular disease
○ irreversible & progressive disease

pregnancy
○ Eisenmenger > 50% mortality risk (after delivery)
○ cyanotic CHD – 12% successful pregnancy

genetic counselling
○ 2 – 50% risk if mother CHD

comorbidity
○ 15 – 20% chromosomal abnormalities


endocarditis
psychosocial
○ anxiety
○ depression

employment
surgical issues


planning
specific challenges
 preservation myocardial function
 blood salvage techniques
○ autologous transfusion
○ pre-op phlebotomy in cyanotic patients
○ routinely cell saver system intraoperatively
 redo sternotomy
○ difficult
○ consider cannulation of femoral vessels, bypass before opening
sternum
 aortopulmonary collaterals
○ complicate peri- 7 post operative management
○ excessive return LA:
- obscures visualisation, volume overlaod etc
 anaesthesia
Case example

31y female
 Tricuspid Atresia
 Fontan @ 6y

29y: atrial flutter – cv
 disability pension
 exercise intolerance & ascites
referred – transplant liver & heart by physician
 RAPA Fontan

 extracardiac conduit + Maze
 great improvement in quality of life
• physician initial care did not understand Fontan circulation &
complexity
• patient + family did not appreciate the need for expert care or where
to find it
reasons for inappropriate care
adults CHD lost to follow-up
 patients & families not educated

○ what to expect
○ where to go

adult physicians not educated re
complexity of GUCH
NEED


network of specialist centers
team of experts
○ nurses – physicians


training
recommendations





unit located adult environment with multidisciplinary provision associated with
strong paediatric cardiology group
each center to serve 5-10million population
referral relationships with primary caregivers
association with transplant centre
medical expertise






Cardiologist – paed + adult
Electro physiologist
MRI, CT
Minimum 2 congenital heart surgeons
Obstetrics for high risk pregnancies
Specialist nurses
European Heart J 2003. 24:1035-84
Why the sharks win
Why the sharks win
Why the sharks win
And the BULLS ?