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Congenital Cardiac Malformations in Relation to Central

Congenital Cardiac Malformations in Relation to Central

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Congenital cardiac malformationsin relation
to
central venous access
Christine ThompsonAbstract
During
the
third
and
seventh weeks
of
gestation, teratogenic exposure
may
lead
to
fetal abnormality such
as
congenital heart defects
or
intrauterinedeath. Congenital heart defects
are
present from birth,
but may
appear
at
any tiine,
or
only revealed postniortetn. Often defects
are
present
by
degree.Some defects
are
life-threatening, while other, less severe conditions,
may
have tttininial physiological iinpact. Left superior vena cava exists
in
earlyembryonic development,
but the
vessel degenerates
as the
cardiovascularsystem matures. When
not
associated with other malformations,
an
incidenceof persistent left-sided superior vena cava (PLSVC)
has no
clinicalsignsor symptoms. However,
tt may not be as
innocuous
asit
appears
due to
itsassociation with
the
cyanotic defect, tetralogy
of
Fallot (TOF). Usinga casehistory
as an
illustration
it can be
shown that
all
cases
of
defect
or
chromosomal suspicion should
be
documented
as
there
maybe
implicationsfor future interventions.
Key words:
Cardiovascular system
and
disorders
Heart disorders
A
46-year-old
man (KW) of
unusual appearanceand manner, with
a
previous medical historyof non-insulin dependence diabetes
and
newlydiagnosed lyniphoma
was
referred
for
insertionof
a
tunnelled central venous line
for the
administrationof chemotherapy. There
was
significant maternal historyof insulin dependence diabetes
and
epilepsy
and his
fatlierdescribed mild cyanosis when extremely cold
and
reported
a
reluctance
to
exercise
in
childhood.
Procedure
The preferred right-sided approach
via the
internal jugularvein
was
attempted. Under ultrasound visualization
the
veinappeared
to be
small
and,
although
it
could
be
cannulated,venospasm appeared
to
occur
and the
guide wire could
not
be advanced.
The
left internal jugular vein appeared largeand compressible
and
therefore
a
left-sided approach
was
attempted.
The
vessel
was
cannulated,
the
guide wire
and
dilator passed easily, there were
no
electrocardiograph changesand
tbe
catheter flinctioned.
A
post-procedural chest X-rayshowed
the
catheter coursing along
the
left heart bonier,initially suggesting misplacement; however,
the
patient
was
asymptoniatic.
A
contrast injection study confirmed
the
Ciiristint' Thompson, (.llinicjl Niirsi' SpetKihst.Tunellcd ('entr.il('.itlietcr ServKC, Cianiuvt'l (ifntT.il Hospit.il, (il.isj;ii\v
Accepted for piihlicalion: January 2006
catheter passing through
a
persistent left-sided superiorvena cava (PLSVC^), through
the
coronary sinns
and
withthe
tip in the
upper right atrium.
An
abnormal, narrowedright superior vena cava
(SVC) and a
sliglitiy constrictedpulmonary artery
was
detected. Later review
of his
medicalnotes revealed childhood surgery
for
repair
of
hypospadias
but
no evidence
of
chmmosomal studies.
Tbe
catheter remainedin position
for 5
months
and the
patient completed
his
courseof chemotherapy without incident.The patient's unusual appearance
and
manner suggestedthere
may be
genetic
or
thromosoinal nnpiications,
but in
view
of his
diagnosis,
a
decision
was
made
to
investigate
no
further until
his
prognosis could
be
determined.RW
was
found
to
sufl^er from
a
non-cyanotic congenitalheart defect(C"HD}.whieh
is one of
over
35
doeunientedhuman congenital cardiac malformations. These recognizedlesions also have many variations.
For
example, RW's particularcondition (PLSVC)
can he
complicated
by
unroofing
of the
coronary sinus wherein cyanosis would
be
present; this willbe discussed later
in the
text (I'orth, 2002). Incidences
of
CHI), also referred
to as
congenital cardiac malformation,are found
in
equal measure
in
every continent.
The
mostcommon congenital defects
are
ventricular septal detects(VSDs)2()-25%,
atria!
septal defects (ASDs) 8-13%, and patentductus arteriosus (I'DAs)
6-11%
(Hanied
and
Maher, 2000).Although these lesions will
be
referred
to in tbe
course
of
thisarticle,
the
author will concentrate specifically
on
PLSVC,
an
acyanotic malformation which
has an
incidence
of
0.3—0.5%of the population
and is the
most coninion congenital venousanomaly
of the SVC
system (Leibowitz
et
al, 1992; Bartram
et
al, 1997),
and the
cyanotic defect with which
it has a
15-25%)association, tetralogy
of
Fallot
(TOF)
(Soto
et al.
1992;Warellet al, 2003; lirzezinski
et al,
2005).
What Is a congenital heart defect?
A
CHI.) can be
defined
as the
structural, funetional
or
positional defect of the heart occurring either
in
isolation
or in
a combination ofseveral lesions collectively Defects
are
presentfrom birth,
but may
appear
at any
time after birth,
or not at
all, often only being revealed postmortem (Lilly, 1998). Somedefects
are
life-threatening
and
require iniiiiediate surgicalcorrection
or
palliation, while other, less severe conditions,may have niininial physiological impact (Lilly, 1998).CHDs
can be
broadly classified into
two
categories: thosecausing little
or no
cyanosis, classed
as
acyanotic.
and
thosec-ausing cyanosis (Lilly, 1998; Porth, 2002).Eight coniinon lesions account
for 85%) of all
casesworldwide
and are
listed
in
'liihlf
/.The
remaining
15%i
276
British Journal
of
Nursmg, 2<X16,Vol 15,
No 5
 
CARDIAC NURSING
accounts
for a
variety rare detects, inckidiiig PLSVC^ (Lilly,
199S;
Porth, 2002).
Cyanotic and acyanotic defects
In the presence of CHDs, pressure gradients between chambersmay
be
created which lead
to
haemodynamic compromise.This effect
is
produced
by
alterations
in
pulmonary bloodflow and the abnormal shunting (diversion) of blood from
one
system
to
another through aberrant openings between bothsides
of
the heart. l.,eft-to-right shunts
are
mainly acyanoticconditions
and
push blood from
tbe
arterial circulation intotbe venous circulation resulting
in
oxygenated blood beingrecycled through
the
right heart, increasing
the
load
and
leading
to
enlargement
of
the right heart. Conversely, right-to-left shunts produce cyanosis
as a
result
of
deoxygenatedblood being forced into
tbe
systemic circulation
via the
leftheart
as in
TOF (Forth. 20(12).
Causes
of
CHD
(;H1)
occurs with equal frequency
in
males
and
femalesand
IS the
cause
ot S0% ot
deaths withm
the
first year
of
life (Hamed
and
Maher, 2(10(1). Approximately 13%
of
thoseborn with
a
cardiac detect will bave another non-cardiac,chromosomal ahnormality (Forth, 2002).The prevalence
ot'
maltorniation among
the
siblings
and
near relatives
of
sucha cbild
IS
sigiiiticantly higher
[ban in the
general population(Hamed
and
Maher, 2000).The crticial phase
tor
tetal heart development is between
the
third
and
seventh week
of
gestation (dittenberger-De Grootet al, 2005). During that tune, teratogemc
(a
tactor increasingthe incidence
of
maltoniKition development) exposure
may
lead
to
tetal abnormality such as C^Hl),
or
intrauterine death
if
exposure
is
prolonged (Lilly,
I
99K;
Hamed
and
Maher,200(l).The involvement
of
a
heredity tactor associated with theselesions
is
widely acknowledged within
the
literature, withmany sutierers having
a
genetic predisposition
to
disease.Embryonic mesoderm
is the
source
of
the cardiogenic place,giving rise
to the
future myocardium,
and
endocardium,which lines
the
heart. Genetic cascades
are
triggered thatgive rise
to
interactions between competing chemicalsignals
111 tbe
primitive beart
and
several mutated genes
Table
1.
Most common
Malformation
Ventricular septal detectPatent ductus arteriosusAtrial septal defectPulmonary valve stenosisAortic valve stenosisCoarctation
of
tine aortaTetralogy
of
FallotTransposition
ot
great arteries
lesions
Cyanotlc/acyanotic
AcyanoticAcyanoticAcyanoticAcyanoticAcyanoticCyanoticCyanoticCyanoticAdapted from: Lilly (1998); Porth (2002)
are thought responsible
for
numerous C^HHs (Bruneau,
2003;
Ciittenberger-De Groot
et al,
2005). Botb intrinsictranscription factors that are required
for
induction of cardiacdifferentiation,
in
particular
the
family NKX2-5, which
are
recessive genes, and theT-box transcription factor TBX5
are
implicated
in the
development
ot a
wide variety
of
CHUssucb
as
ASD,VSD, Ebsteins anomaly
of the
tricuspid valve,aortic valve stenosis atrioventricular
(AV)
block,
and TOF.
Further,
as
ditlerent areas
ot the
developing lieart
are
moresensitive
to the
effect
of
these agents
it is now
possibleto predict
at
what stage
of
development
the CHI) was
established, as shown
in
Tahlc
2
(Bruneau, 2003).Although
no one
specific cause
of
all t\'pes
of
congenitalheart detects
has yet
been identified,
the
currently prevailingnotion, running
in
tandem with
the
above,
is
that
the
occurrence
ot a ("HI) is
more likely
to be the
result
of a
genetic
and
environmental interaction rather than that
ot a
single chromosomal abnormality.
A
triad
of
predisposition,vulnerability
and
teratogemc agents
may all be
necessary
to
produce sucb detects (Lilly. 1998; Hamed
and
Maher, 2000;Porth, 2002).There
are
many risk factors
tor
CHI), and theseare ldentitied
in
'Ihhlcs
J
and
4.
In addition
to the
above,
the
relevance
of low
socioeconomic status reflecting maternal nutrition
and the
issue
of
matrilineal transmission have also been recognizedas significant factors
m the
development
of CHD
(Hamedand Maher, 2(.)00).
Table
2.
Gestatlonal
age at
Oestational
aee
(days)
1
5
Events HeartdifferentiatesSome
of
ttie
Not
knownCHD occuring
which
a
congenital heart defect
20
Heart tubeforms.Heart beatsCardia bifida,Laterality defect28Early chambersform andloop
to
rightHypoplastic5
L and
R tieart
(CHD)
may
32
Chamberbrmation.septatesdefectivevalves.
AV
canal defects.tetraiogyof Fallot
ASD
=
Atrial septal defect:
AV -
Aortic valve; DORV
=
Double outlet right ventricle: VSD
=
Ventricuiar septai defect
arise
50+Valves form.Septation occurs.conductionsystems form,connects
to
great vesselsASD.VSD,DORV.tetralogycf Faliot.conduction defectsSource: Bruneau (2003)
i Jouriijl ot" Nursiiis;. 2niMi.Vi>)
l.S, No 5
277
 
Table 3. Congenital heart defect with chromosomalabnormalities
Chromosomal Name of syndromeabnormality
Type of defect resulting
21
18
13XOtrisomytrisomytrisomysyndromeDown
--
Turners syndrome"s syndrome
VSD
VSD. PDA.DORVDextocardia. VSD,Coarctationof the
DORV = Double outlet right ventride: PDA
=•
Patent ductus arteriosus; VSD
Adaptedfrom: Lilly (1998);Hamed and
PDA
aorta, stenosis
= Ventricuiar septal defect
Maher (2000); Portfi (2002)
Normal embryonic cardiac development
111 rhe limiian embryo, the mesodcrmal germ layer givesrise
to
the entire cardiovascular system. The heart developstrom two simple epithelial tubes which tlise to form a singlechambered heart.Twenty-three days tollowing conception the single, simpleepithelial heart tube lies within
the
embryo's pericardialcavity and
is
layered with cells which are known
as the
cardiac mantle, which eventually give rise to the epicardiumand myocardium. As development progresses, the crania!one-third
ot
the tube dilates
to
form the aortic sac whichwill give rise
to
the aortic arches. The caudal one-thirdalso dilates
to
form the early embryonic ventricle aroundthe 2Sth day. The remaining mid-portion forms the bulbuscordis, which has three distinct areas of development:Tlie proximal one-third gives rise ro the bocly of the riglitventricle• The distal section, known
as the
trunciis arteriosus,develops into the aortic root and ascending aortaThe remaining mid portion, the conus cordis, connects theprimitive right ventricle to the truncus arteriosus.The conus cordis partitions to torm the outflow tracts otthe right and left ventricles.l)-looping (where the heart tube grows longer and bendsto tbe right) is responsible tor die initial positioning of tbeprimitive ventricle.Distal to tbe primitive rigbt ventricle !s an area known asthe conotriincai region (maldevelopment
of
this area leadsto TOF) containing tbe conus cordis and truncus arteriosus.'fbe conotruncal region and tbe primitive rigbt ventricle arecollectively known as tbe bulbus cordis.
As
growth continues,the conotruncal region moves centrally with torsion andtwisting, giving rise
to
the anatomical curve
oi
the aortaand the pulmonary artery and aligns the heart in its normalposition. By the 56th day, valves and septation has occurred,the conduction system is forming and
it
is connected to thegreat vessels
{H-^iirc
/)
(Larson, l'/JS; Lilly, 1998; Porth, 201)2;Gittenberger-Ue Groot et al, 2005).
Persistent left-sided superior vena cava
Ibe worldwide incKlence of PLSVCJ is approximately 0.3%(Leibowitz et
al,
1992; Bartram et
al,
1997). In those with othercongenital cardiac defects the incidence is considerably higher,between 3% and H*34% and is tbe most common anomalyof systemic venous return {Leibowitz et al, 1992; 13artram et al,1997). Diagnosis is often incidental and in approximately 25%of
cases
it
is associated with TOF and other cardiac as well asnon-cardiac malformations (Bartram et al, 1997).The basis of the I'LSVC
is
poorly understood.
It
resultsfrom in utero failure of the left cardinal vein to develop andcan take various forms. In most cases
it
results in
a
bilateralSVC, often with
a
venous bridge joining the two, or theymay
be
completely separate from eacb other (Soto
et al,
1992). Occasionally the right
SVC.
is absent and the venousreturn trom the upper body enters the coronary sinus to tberight atrium (vvww.pediheart.org). PLSVC draining into theright atritiin via an enlarged coronary sinus, and rarely withan absent rigbt SVC, will rarely produce any pbysiologicalderangement
as
venous blood continues
to
return
to
theright atrium and, therefore,
the
condition requires
no
treatment, as in the case of the patient l^W (Leibowitz et al,1992; Gcrber and Kuzuzo, 2002)
{Fi^^mr
2).
More infrequently,
a
FLSVC]
can be
connected
to tbe
roof of tbe lett atriinn instead of the coronary sinus whichthen continues as an inter-artrial conduit that ailows venousreturn to the left atrium. This is known as 'unroofing' and
is
clinically signiticant (Sabiston and Spencer. 1995). A I'LSVCdraining into
the
lett atrium
is
associated with
an
ASDwhich produces right-to-left shunting and haemodynamiccompromise resulting
in
cyanosis which
is
usually mild andperipheral, and is the main clinical sign (Bartram et al. 1997;
Table 4. Environmental factors significantly increasingmalformation
Maternal infectionsMaternal drugsMaternal diseaseOther maternalfactors
ASD = Atrial septal defect:
Rubella, mumps, intrauterine infectionAnti convulsants (phenytoin), vitamin D,anti-depressantsDiabetes, systemic lupus erythematosis, hypertension.phenylketonuria, phenylalanaemiaMaternal agePre-natal alcohol consumptionSmoking accompanied by low birth weightExposure to X-raysExposure to toxins
VSD = Ventricular septal delect
incidence of cardiovascular
Pulmonary stenosis, VSD, ASD,endocardial fibroelastosisAortic stenosisTransposition of greatarteries, congenital heart blockChromosomal defects (down s syndrome}Fetal alcohoi syndromeAdapted from; Lilly (1998); Hamed and Maher (2000); Porth (2002)
278
Uri[isiiJ,n]rii,jl
i;. 20(lf,.Voi 15. No 5

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