Download Perioperative Challenges in Adults with Congenital Heart Disease

Perioperative
Challenges
in
Adults
with
Congenital
Heart
Disease:
A
Pediatric
Perspective.
GD
Williams,
MB.
In
his
assessment
of
the
current
health
care
of
adults
with
congenital
heart
disease
(CHD),
Dr.
G.
Webb
stated
the
following
[1].
“The
care
of
adult
patients
with
congenital
heart
defects
in
the
United
States
is
spotty
at
best,
and
needs
to
improve
greatly
if
the
needs
of
these
patients
are
to
be
met.
The
care
of
American
children
with
congenital
heart
defects
is
generally
excellent.
Pediatric
cardiac
services
are
well
established
and
well
supported.
The
care
of
adults
with
congenital
heart
disease
(CHD)
is
well
established
in
only
a
few
American
centers.
While
there
are
an
increasing
number
of
clinics,
they
are
generally
poorly
resourced
with
relatively
few
patients.
If
located
in
adult
cardiology
programs,
they
are
usually
minor
players.
If
located
in
pediatric
cardiac
programs,
they
are
usually
minor
players
as
well.
Training
programs
for
adult
CHD
(ACHD)
caregivers
are
few,
informal,
and
poorly
funded.
To
improve
the
situation,
we
need
perhaps
25
well‐resourced
and
well‐established
regional
ACHD
centers
in
the
United
States.”
Most
ACHD
centers
in
North
America
and
Europe
do
not
meet
their
national
guideline
standards
[2‐7].
Currently,
a
sizable
proportion
of
North
American
institutions
that
self‐report
to
be
ACHD
centers
are
associated
with
pediatric
hospitals
(International
Society
for
Adult
Congenital
Heart
Disease,
http://www.achaheart.org/).
Most
outpatient
and
inpatient
services
and
surgery
are
conducted
in
facilities
that
provide
both
pediatric
and
adult
care
[2].
A
recent
pan‐Canadian
survey
of
quality
of
ACHD
care
reported
8
of
the
15
ACHD
clinics
were
part
of
a
university
center
that
had
an
established
pediatric
cardiac
surgery
program.
These
ACHD
clinics
had
larger
numbers
of
yearly
clinic
visits,
annual
percutaneous
volumes
and
surgical
volumes
compared
to
clinics
without
an
affiliated
pediatric
cardiac
surgery
program
at
their
site.
They
also
tended
to
have
larger
numbers
of
registered
patients,
larger
numbers
of
active
patients,
more
dedicated
nursing
support
and
more
physicians
with
ACHD
Fellowships
[3].
National
practice
guidelines
generally
concur
regarding
the
provision
of
anesthesia
services
to
ACHD
patients.
“Surgery
in
ACHD
patients
(including
anesthesia
and
intensive
care)
is
very
different
from
conventional
adult
cardiac
surgery,
and
this
provides
a
strong
case
for
concentrating
resources
into
specialist
units
for
both
treatment
and
training.
It
should
be
appreciated
that
even
minor
non‐cardiac
surgery
may
carry
a
high
risk;
consultation
with
specialists,
and
careful
preoperative
planning
and
intra‐
operative
monitoring,
are
vital
to
avoid
complications
[8].”
“Surgical
procedures
that
require
general
anesthesia
or
conscious
sedation
in
adults
with
moderate
or
complex
CHD
should
be
performed
in
a
regional
ACHD
center
with
an
anesthesiologist
familiar
with
ACHD
patients.
An
ACHD
center
should
have
several
cardiac
anesthesiologists
[9].”
From
a
pediatric
cardiac
anesthesiologist’s
perspective,
optimal
perioperative
management
requires
an
understanding
that
ACHD
patients
are
not
simply
“big
children
with
CHD”.
These
two
patient
groups
are
dissimilar
for
the
following
reasons.
(i)
Adults
are
naturally
different
from
children;
(ii)
ACHD
presents
some
unique
management
challenges;
and
(iii)
non‐CHD
comorbidities
differ
between
adults
and
children.
When
children
with
CHD
attain
adulthood
and
independence,
they
have
to
tackle
a
variety
of
new
life‐
issues.
Transition
of
medical
care
refers
to
a
shift
in
the
responsibility
of
health
care
management
from
the
family
to
the
patient.
Often
this
is
poorly
done
and
there
is
substantial
loss
of
patients
to
follow‐up
[10].
Specialized
ACHD
clinics
are
hard
to
find
and
waiting
times
for
ACHD
services
may
be
long
[3].
Non‐
compliance
with
medical
therapy
is
a
common
problem.
Adults
with
CHD
may
have
felt
unwell
their
entire
life
and
may
suffer
from
depression
[11].
Perceived
parental
overprotection
is
associated
with
heart‐
focused
anxiety
in
adults
with
CHD
[12].
Older
patients
may
be
living
with
the
diagnosis
of
incurable
heart
disease.
Many
adults
with
congenital
heart
disease
have
a
specific
need
for
advice
about
socio‐legal
questions
(insurance,
provision
for
old
age,
severe
disability),
types
of
education
(school,
study,
occupation),
employability,
physical
resilience
(e.g.
performance
ability,
sporting
activity),
acquisition
of
a
driver’s
license,
suitability
to
fly
and
often
also
issues
concerning
sexuality,
pregnancy
and
inheritance
of
heart
defects
[13].
Many
patients
wish
to
discuss
end
of
life
matters
with
their
health
professionals
[14].
Awareness
of
these
psycho‐social
factors
is
important
when
interacting
with
the
ACHD
patient.
There
are
several
reasons
why
CHD
in
adults
is
not
the
same
as
CHD
is
children.
Some
elderly
patients
with
Eisenmenger’s
syndrome
have
unrepaired
septal
defects
that
were
deemed
inoperable
during
an
earlier
era.
Certain
lesions
(eg:
recurrent
pulmonary
vein
stenosis,
hypoplastic
left
heart
syndrome)
have
(or
had)
a
high
infant
mortality
and
are
under‐represented
in
the
current
adult
population.
Adults
may
have
undergone
surgical
procedures
that
later
fell
from
favor.
Examples
include
the
atrial
switch
(Mustard,
Senning),
Potts’
and
Waterston
aorto‐pulmonary
shunts
and
the
atrio‐pulmononary
Fontan.
Adults
are
likely
to
develop
the
late
cardiovascular
complications
of
CHD
because
their
hearts
have
been
subjected
to
multiple
cardiac
surgeries
and
have
endured
abnormal
pressure
and
volume
loads,
cyanosis,
hypoxia
and
dysrhythmias
for
a
long
time.
For
example,
the
prevalence
of
atrial
fibrillation
and
ventricular
arrhythmias
increases
markedly
after
45
years
of
age
in
patients
with
repaired
Tetralogy
of
Fallot
[15].
In
earlier
times,
biventricular
repairs
(eg:
Tetralogy
of
Fallot)
were
delayed
until
late
childhood
and
this
may
limit
the
potential
for
ventricular
remodeling.
There
are
gender‐related
differences
in
CHD
that
are
less
apparent
in
children.
For
example,
women
with
CHD
are
more
likely
to
develop
pulmonary
hypertension
and
less
likely
to
have
infective
endocarditis
[16].
Complications
commonly
associated
with
ACHD
include
heart
failure
[17],
arrhythmia
[18,
19],
infective
endocarditis
[20],
impaired
pulmonary
function
[21],
pulmonary
hypertension
[22],
neurological
impairment,
cerebrovascular
accidents
[23]
and
thromboembolic
events.
Some
patients
(for
example,
those
palliated
with
the
Fontan
procedure)
are
prone
to
other
organ
injury
such
as
liver,
gut
or
kidney
dysfunction
from
poor
systemic
output,
chronic
venous
congestion,
hypoxia,
drug
effects,
abnormal
coagulation,
sepsis
and
other
factors.
There
may
also
be
complications
related
to
surgery,
other
invasive
diagnostic
and
therapeutic
cardiac
procedures
and
heart
or
heart‐lung
transplantation
[24].
CHD
is
associated
with
non‐cardiac
anomalies
and
genetic
disorders
and
there
may
be
complications
related
to
these
conditions.
For
example,
patients
with
Down
syndrome
have
an
increased
risk
of
Alzheimer’s
disease
[25].
In
addition,
adults
with
CHD
may
have
concomitant
non‐cardiac
disease
such
as
obesity,
hypertension,
coronary
artery
disease,
diabetes
mellitus
and
chronic
kidney
disease.
Considering
the
factors
outlined
above,
it
is
not
surprising
that
ACHD
guidelines
recommend
that
the
anesthesiologist
should
have
expertise
in
adult
cardiac
anesthesia
and
the
management
of
CHD.
Physicians
with
this
expertise
are
in
short
supply
and
may
not
be
available.
In
such
a
situation,
pediatric
cardiac
anesthesiologists
are
often
asked
to
provide
ACHD
care
because
they
are
familiar
with
the
management
of
CHD.
Currently,
it
appears
cardiac
surgery
outcomes
are
best
when
a
congenital
heart
surgeon
performs
the
heart
surgery
[26‐28].
Some
studies
suggest
the
cardiac
surgery
should
occur
in
a
pediatric
hospital
[26]
while
others
note
a
high
incidence
of
perioperative
complications
in
the
pediatric
setting
[29]
and
a
higher
mortality
compared
to
care
in
an
adult
hospital
[28].
Information
is
lacking
about
outcomes
after
non‐cardiac
surgery
in
ACHD
patients.
Typically,
a
pediatric
cardiac
anesthesiologist
becomes
involved
because
(i)
the
adult
patient
with
CHD
is
undergoing
a
cardiac
procedure,
performed
by
a
CHD
specialist
(surgeon
or
cardiologist)
at
a
pediatric
hospital;
or
(ii)
the
pediatric
anesthesiologist
has
been
asked
by
his
adult
hospital
colleagues
to
assist
in
the
management
of
an
ACHD
case
that
will
be
performed
at
an
adult
hospital.
When
the
procedure
is
to
occur
in
a
pediatric
hospital,
the
pediatric
anesthesiologist
should
comprehensively
review
the
ACHD
case
and
decide
whether
she/he
is
comfortable
managing
its
non‐pediatric
aspects.
If
necessary,
an
anesthesiolgist
with
adult
expertise
should
be
consulted.
This
is,
of
course,
labor
intensive
but
seems
an
appropriate
strategy
until
each
ACHD
center
has
built
up
a
cadre
of
experienced
ACHD
anesthesiologists.
The
perioperative
management
of
ACDH
patients
in
a
pediatric
hospital
requires
considerable
pre‐
planning
and
coordination
of
effort.
All
equipment
and
pharmaceuticals
should
be
appropriate
for
the
patient’s
size
and
age.
Special
attention
should
be
paid
to
items
that
are
seldom
used
but
important
‐
for
example,
code
carts,
ECMO
backup,
ICU
emergency
sternotomy
sets,
etc.
Nursing,
physician
and
staff
education
and
collaboration
are
vital.
With
proper
preparation,
care
for
ACHD
patients
at
our
pediatric
hospital
has
anecdotally
been
a
positive
experience
for
patients
and
providers;
others
report
a
similar
finding
[30].
More
problematic
for
our
institution,
is
the
sharing
of
patient
management
between
the
adult
and
pediatric
hospitals.
Although
physically
connected,
there
are
many
impediments
to
seamless
transfer
of
care.
These
include
cumbersome
administrative
processes,
non‐compatible
computer
systems,
limited
accessibility
to
patient
data
and
electronic
documentation,
different
hospital
equipment
and
pharmacy
support,
dissimilar
clinical
protocols
and
styles
of
practice.
Communication
channels
between
pediatric
and
adult
services
may
be
mediocre
because
they
are
infrequently
used.
To
overcome
these
issues,
it
is
our
practice
to
involve
an
adult
cardiac
anesthesiologist
in
a
pediatric
hospital
procedure
if
the
ACHD
patient
is
to
receive
early
postoperative
care
in
the
adult
hospital.
Again,
this
is
labor
intensive
but
well
worth
the
effort.
Many
ACHD
patients
will
require
anesthesia
for
non‐cardiac
procedures.
There
appears
to
be
minimal
indication
for
performing
these
procedures
in
a
pediatric
hospital.
Far
better
to
use
adult
hospital
facilities
and
adult
surgeons
and
anesthesiologists,
with
a
pediatric
cardiac
anesthesiologist
consulted
on
CHD
perioperative
management.
Like
many
other
ACHD
centers,
the
process
at
our
institution
(Stanford
University,
CA)
is
still
evolving.
The
ACHD
clinic
is
located
within
the
adult
hospital
and
staffed
with
ACHD‐trained
cardiologists.
ACHD
cardiac
surgery
is
performed
at
the
pediatric
hospital
by
the
pediatric
CHD
team.
After
initial
postoperative
care
by
pediatric
cardiologists
and
intensivists
in
the
pediatric
CVICU,
patients
are
transferred
to
an
adult
hospital
ward
and
managed
by
ACHD
cardiologists.
Other
invasive
procedures
are
done
at
the
adult
hospital
with
collaborative
involvement
of
CHD
and
adult
specialists.
The
longer‐term
proposal
is
to
move
all
management
to
the
adult
hospital
and
utilize
pediatric
CHD
expertise
as
required.
A
recent
editorial
[4]
comments
that
it
will
be
a
long
journey
before
reaching
the
end
of
the
road
to
better
ACHD
care.
Although
most
agree
there
is
a
need
to
strengthen
the
human
resource
infrastructure
of
ACHD
care
in
the
USA,
how
and
when
that
will
occur
remains
unclear.
Until
there
is
a
full
complement
of
personnel
competent
at
ACHD
care,
it
will
often
be
pediatric
subspecialists
(including
pediatric
cardiac
anesthesiologists)
who
are
asked
to
provide
their
services.
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