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.

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

3. What
is
hypoplasEc
leO
heart?

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?

12. Systemic arterial oxygen saturation versus systemic oxygen (O2) delivery.
Barnea O et al. Circulation. 1998;98:1407-1413
Copyright © American Heart Association, Inc. All rights reserved.

13. Pre-­‐op
2.
Manipulate
pulmonary
vascular
resistance
(PVR)
Goal:
Qp/Qs:0.8-­‐1
Factors
that
increase
PVR:
-­‐Hypoxia
-­‐Hypercarbia
-­‐HyperinflaEon
-­‐High
hematocrit

14. Pre-­‐op
3.
Correct
metabolic
acidosis
– Metabolic
acidosis
indicates
inadequate
cardiac
output
– Aim
for
base
deficit
of
0

15. Surgical
management
• Norwood(stage
I)
• BidirecEonal
Glenn(stage
II)
• Fontan
(Stage
III)

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.

18. Norwood
Postop
• Norwood
Clinical
pathway

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

21. BidirecEonal
Glenn
(Stage
II)

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

23. Fontan
procedure(Stage
III)

24. Normal circulation Vs Fontan Circulation.
Gewillig M Heart 2005;91:839-846
Copyright © BMJ Publishing Group Ltd & British Cardiovascular Society. All rights reserved.

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)

28. Cumulative hazard by mode of death.
Khairy P et al. Circulation. 2008;117:85-92
Copyright © American Heart Association, Inc. All rights reserved.

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

32. Prevalence of neurodevelopmental impairment in the population with congenital heart
disease (CHD).
Marino B S et al. Circulation. 2012;126:1143-1172
Copyright © American Heart Association, Inc. All rights reserved.

33. Developmental
outcome
CirculaEon.
2002;106:I-­‐95-­‐I-­‐102

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

36. Hepatic fibrosis marker (Forns index) after Fontan operation.
Baek J S et al. Heart 2010;96:1750-1755
Copyright © BMJ Publishing Group Ltd & British Cardiovascular Society. All rights reserved.

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

38. Protein‐Losing Enteropathy after Fontan Operation
Congenital
Heart
Disease
Volume
2,
Issue
5,
pages
288-­‐300,
14
SEP
2007
DOI:
10.1111/j.1747-­‐0803.2007.00116.x
hqp://onlinelibrary.wiley.com/doi/10.1111/j.1747-­‐0803.2007.00116.x/full#f3

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)