As the number of children who survive single ventricle physiology, it is very important for the pediatrician to understand about them to give them the best care.
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Single ventricle presentation for pediatrician
1. What
Pediatricians
Should
Know
About
Children
With
Single
Ventricle
Laxmi
Ghimire,
MD
PGY3,
KU
Pediatrics
2. ObjecEves
• To
define
single
ventricle
cardiac
defect
• To
idenEfy
pathophysiology
and
clinical
features
of
HLHS
in
brief
• Formulate
management
of
pre
and
postop
care
of
child
with
single
ventricle
• Evaluate
long
term
complicaEons
and
follow
up
of
HLHS
• Describe
the
role
of
pediatricians
in
following
children
with
HLHS
4. HLHS
Epidemiology
• 1.2-‐1.5%
of
all
congenital
heart
defects
• In
US,
≈2000
infants
are
born/
year
with
HLHS
• Without
surgery
HLHS
is
uniformly
fatal
usually
≤2
weeks
of
life.
5. Pathophysiology
of
HLHS
• Ductal
dependent
lesions
• Important
to
keep
duct
open,
why?
– To
provide
systemic
circulaEon
6. Clinical
features
of
HLHS
• Around
24-‐48
hours
aOer
the
ductus
closes-‐
signs
of
cardiogenic
shock
– Cyanosis,
– Tachypnea,
– Respiratory
distress,
– Pallor,
– Lethargy,
– Metabolic
acidosis,
and
– Oliguria.
– Poor
peripheral
perfusion
7. Cardiac
exam
• A
prominent
right
ventricular
impulse
• A
normal
S1
• A
loud
single
S2
• Usually
no
murmur
is
noted
8. Management
of
HLHS
• SupporEve
care
• MulEstage
surgical
intervenEon
(ie,
Norwood,
Glenn,
and
Fontan
procedures)
and
• Cardiac
transplantaEon
9. Medical
preop
management
• Prenatal
diagnosis
• HLHS
is
idenEfied:
deliver
at
an
insEtuEon
where
neonatal
cardiac
surgery,
is
performed
• Try
for
term
delivery,
vaginal
delivery
well
tolerated
10. PreoperaEve
management
1.
Open
the
ductus
arteriosus
– HLHS
is
suspected:
start
PG
E1
infusion
immediately
to
establish
ductal
patency
and
ensure
adequate
systemic
perfusion.
– Refer
11. Pre-‐op
• Intubate
or
not
to
intubate?
• If
short
distance:
do
not
intubate
• OxygenaEon:
Avoid
high
amount
of
oxygen
• Keep
sats
between
75-‐85%,
why?
16. Norwood
Procedure
– Done
in
first
weeks
of
life-‐aOer
infant
stable
– The
goals
•
To
establish
reliable
systemic
circulaEon
without
the
DA
• To
provide
enough
pulmonary
blood
and
prepare
pul
vascular
bed
for
stages
II
and
III.
19. Norwood
postop
• Discharge:
– PCP:
adjust
medicaEon
dose
with
weight
– Oxygen
saturaEon
is
typically
70-‐80%
in
room
air.
– BT
shunt:
conEnuous
murmur
– Thrombosis
of
shunt
20. BidirecEonal
Glenn
procedure
(Stage
II)
• Aprox
4-‐6
months
aOer
Norwood
procedure.
• The
bidirecEonal
Glenn
procedure
=>
anastomosis
between
SVC
and
RPA,
end-‐to-‐side
• ComplicaEons:
Progressive
desaturaEons-‐due
to
venous
collaterals
• No
murmur:
silent.
Thrombosis
of
Glenn
22. Fontan
procedure(stage
III)
• The
Fontan
procedure
:1.5-‐4
yrs
aOer
Glenn
• CompleEon
of
the
Fontan
procedure
– Blood
flow
from
IVC
to
the
PAs
– Systemic
venous
blood
returns
to
the
lungs
passively
without
passing
through
a
ventricle.
• Fontan
looks
simple:
hemodynamics
unclear
25. ComplicaEons
of
Fontan
CirculaEon
• Mild
to
moderate
exercise
intolerance
• Residual
cardiomegaly
• Ventricular
dysfuncEon
• Rhythm
and
conducEon
disturbances
• Hepatomegaly
• LymphaEc
dysfuncEon
with
protein
losing
enteropathy
• Early
and
late
mortality
26. LymphaEc
DysfuncEon
─
Impedes
drainage
of
thoracic
duct.
– Leakage
in
intersEEum
=>
lymph
edema/
pulmonary
edema,
a
very
lethal
complicaEon
in
the
early
postoperaEve
period.
– Chylothorax
or
chylopericardium
– Leakage
into
the
gut
leads
to
protein
losing
enteropathy
(PLE)
27. OutpaEent
follow
up
• Periodic
follow-‐up
visits
aOer
stage
I,
II,
and
III
operaEons
are
mandatory.
• SubstanEal
(5-‐15%)
interstage
mortality
(between
stages
I
and
II)
•
Careful
observaEon,
follow-‐up
and
home
surveillance
•
Interstage
mortality
between
stage
II
(bidirecEonal
Glenn)
and
stage
III
(Fontan)
is
lower
that
aOer
stage
I
(Norwood)
29. Prognosis
• Overall
survival
Norwood
is
~75%.
•
Survival
aOer
the
bidirecEonal
Glenn/hemi-‐Fontan
and
Fontan
operaEons
is
nearly
90-‐95%.
• Survival
rate
post
staged
reconstrucEon
is
70%
at
5
years.
30. PaEent
educaEon
• General:
HLHS
is
a
complex
heart
defect
that
requires
mulEple
hospitalizaEons,
surgeries,
catheter
intervenEons
and
long-‐term
follow-‐
up.
• MedicaEon
– Educate
parents
regarding
cardiac
medicaEons,
interacEons
• Feeding
– Many
require
NG
or
G-‐tube
tube
feeding
– Increased-‐calorie
formula
is
required
for
adequate
growth.
• Follow-‐up
care
– Importance
of
follow-‐up
care.
31. Role
of
Pediatricians
• Cardiac
care
• Neuro-‐behavioral
care
• GastrointesEnal
care
• Growth
34. Liver
funcEon
aOer
Fontan
• Deranged
hepaEc
funcEon
– ProlongaEon
of
the
PT
– Low
factor
V
level.
• No
relaEonship
between
cardiac
funcEonal
measurements
and
liver
funcEon.
35. Fontan
and
Liver
problems
• 1/3-‐2/3
paEents
would
have
liver
problem
by
teenage
years
• Good
Eme
to
monitor
liver
funcEon=
11
yrs
Baek J S et al. Heart 2010;96:1750-1755
37. LymphaEc
DysfuncEon:
Protein
Losing
Enteropathy
•
Fontan
circulaEon
– Impedes
drainage
of
thoracic
duct.
– Leakage
into
the
gut
leads
to
protein
losing
enteropathy
(PLE),
the
most
frequent
lymphaEc
problem
in
long
term
follow
up
39. Poor
Growth
and
Single
Ventricle
• Significantly
underweight
and
shorter
• Shorter
stature
:
~25%
Fontan
survivors
Vs
13.4%
in
the
healthy
pediatric
populaEon.
Cardiology
in
the
Young,
10,
pp
447-‐457.
2000
American
Heart
Journal
-‐2010
Vol.
160,
P
1092-‐1098.
40. Weight in Children With Single Ventricle Physiology
J Am Coll Cardiol. 2007;50(19):1876-1883.
41. Height in Children With Single Ventricle Physiology
J Am Coll Cardiol. 2007;50(19):1876-1883.
42. The
most
important
component
of
care
of
single
ventricle
children..
………CoordinaEon
of
care
between
primary
care
physician
and
subspecialEes.
43. Take
home
messages
• Single
ventricle
distorts
the
normal
physiology
• Surgeries
are
complicated
• Pediatrician
can
do
a
lot
make
sure
they
are
growing
well
and
geung
appropriate
care
• AnEcipate
the
complicaEons
of
Fontan
• Cardiac,
Neuro,
GI,
poor
growth
are
the
most
common
long
complicaEons
encountered.
Coordinate
care
with
other
specialEes.
44. Thank
you!!
• Dr.
Schroeder
• Dr.
Goertz
• Dr.
Meyer
• Dr.
Almadhoun
45. Bibliography
• Park,
M.
Textbook
of
Pediatric
Cardiology.
5th
EdiEon.
2007
• Moss
&
Adam’s
Heart
diseases
in
Infants,
Children
and
adolescents.
8th
EdiEon.
2012
• Rao,
S.
HypoplasEc
LeO
Heart
Syndrome.
E-‐medicine(Accessed
12/20/2013)