Brom Aoroplasty for Supravalvar Aortic Stenosis

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Brom"s t#ree!patc# aortoplasty is indicated for t#e repair of supravalvar aortic stenosis$
%#ic# can occur as an isolated defect or as a part of &illiams syndrom' T#is syndrome
is c#aracteri(ed by supravalvar aortic stenosis$ elfin craniofacial features$ perip#eral
pulmonary artery stenosis$ and #ypercalcemia'
atient Selection
Brom"s t#ree!patc# aortoplasty is indicated for t#e repair of supravalvar aortic stenosis$
%#ic# can occur as an isolated defect or as a part of &illiams syndrom )1 *' T#is
syndrome is c#aracteri(ed by supravalvar aortic stenosis$ elfin craniofacial features$
perip#eral pulmonary artery stenosis$ and #ypercalcemia' T#e underlying cause is a loss
of function mutation of t#e elastin gene on c#romosome +,11'23' T#ese patients %ill
#ave a systolic cardiac murmur and t%o!dimensional ec#ocardiograp#y can establis# t#e
diagnosis' -ardiac cat#eteri(ation is indicated to evaluate t#e status of t#e coronary
arteries and assess t#e pulmonary arterial tree for related perip#eral stenosis'
.ndications for surgery include symptoms of diminis#ed e/ercise tolerance$ syncope$ or
angina' 0t#er indications %ould include 11 systolic ascending aorta gradient greater
t#an 52 mm 3g$ 21 aortic valve insufficiency$ or 31 evidence of compromised coronary
artery perfusion' T#ese patients s#ould undergo surgery at a relatively early age because
of t#e detrimental effect of t#e #ig# pro/imal aortic pressure on t#e coronary arteries
%it# t#e possibility for accelerated at#erosclerotic c#anges' T#ese patients typically
#ave a t#ic4ening of t#e aortic %all at t#e sinotubular 5unction' T#is abnormal
t#ic4ening may e/tend into t#e ascending aorta and even into t#e aortic arc#' .n some
patients$ t#is abnormal t#ic4ening creates stenosis of t#e coronary ostium'
T#e surgical #istory of operations for supravalvar aortic stenosis is s#o%n in Table 1'
6%ig#t 7c8oon and 9o#n :ir4lin )2 * from t#e 7ayo -linic first reported a one!patc#
teardrop!type repair of supravalvar aortic stenosis in 1;<1' 6onald 6oty )3 * reported
t#e use of an inverted Y!s#aped patc# %#ic# e/tended into t%o of t#e aortic valve
sinuses in 1;++' T#e tec#ni,ue described in t#is monograp# is t#at of A' 8erard Brom$
)4 * %it# t#ree patc#es placed into t#e t#ree aortic valve sinuses' T#is fre,uently also
re,uires a patc# in t#e distal ascending aorta' 7ost recently$ 9o#n 7yers )5 * described
an approac# %it# t#ree incisions into t#e t#ree coronary sinuses and corresponding
counterincisions into t#e distal ascending aorta %#ic# t#en insert into t#e openings
created in t#e pro/imal ascending aorta' T#is tec#ni,ue #as t#e advantage of not
re,uiring autologous patc# material$ but is tec#nically more demanding'
Table 1: Surgical 3istory of 0perations for Supravalvar Aortic Stenosis

Surgeon Tec#ni,ue =umber of Sinuses 0pened Year >eported
7c8oon Single!patc# 1 1;<1
6oty .nverted ?Y? atc# 2 1;++
Brom T#ree!patc# 3 1;@@
7yers
T#ree!sinus
incision
3 1;;3

An asymptomatic$ 5!year!old$ 1@!4g boy 4no%n by genetic testing to #ave &illiams
syndrome on p#ysical e/amination #ad a .ABA. #ars# systolic e5ection murmur and no
diastolic murmur' reoperative ec#ocardiogram s#o%ed an aortic root dimension of 2
cm and t#e dimension at t#e level of stenosis in t#e ascending aorta %as @ mm' ea4
ec#ocardiogram gradient %as +2 mm 3g %it# a cardiac cat#eteri(ation gradient of 52
mm 3g' T#e c#ild #ad no perip#eral pulmonary artery stenosis'
0perative Steps

Cigure 1
An asymptomatic$ 5!year!old$ 1@!4g boy 4no%n by genetic testing to #ave &illiams
syndrome on p#ysical e/amination #ad a .ABA. #ars# systolic e5ection murmur and no
diastolic murmur' reoperative ec#ocardiogram s#o%ed an aortic root dimension of 2
cm and t#e dimension at t#e level of stenosis in t#e ascending aorta %as @ mm' ea4
ec#ocardiogram gradient %as +2 mm 3g %it# a cardiac cat#eteri(ation gradient of 52
mm 3g' T#e c#ild #ad no perip#eral pulmonary artery stenosis'
reoperative transesop#ageal ec#ocardiogram in long!a/is vie% s#o%s ascending aorta
%it# aortic valve to t#e left' T#e mar4ers indicate t#e aoritc root dimension to be 2'2 cm
and #te mid portion of t#e stenosis to be 2'@ cm'


Cigure 2: reoperative cardiac
cat#eteri(ation'
Cigure 3:T#e ?%aist? in t#e ascending aorta is readily apparent' T#e ?%aist? is above
coronary orifices'

T#e operative approac# is t#roug# a median sternotomy %it# t#e use of
cardiopulmonary bypass and bicaval venous cannulation' T#e first dose of cold blood
cardioplegia is antegradeD t#e remaining doses are retrograde'

Cigure 5: 7ain pulmonary artery in5ection' T#ere is no perip#eral pulmonary artery
stenosis'
Cigures < E +: 3ig# aortic cannulation near innominate artery'

Cigure ;: ?S#ield?!s#aped patc#es for t#ree coronary incisions and ascending aorta
incision'
Cigure 12: ?=ormal? si(ed distal ascending aortaD 14!mm si(er'

Cigures 12 E 13: -ardioplegia needle

Cigures 1< E 1+: Cirst incision is in noncoronary sinus ! t#is opens aorta and improves access to visuali(e cornoary orifices for t#e ne/t incisions' T#is is an important
point as t#e surgeonFs vie% of t#e coronary orifices from above is severely restricted until t#is first incision is made'

Cigures 22 E 21: T#ird incision #as been made to t#e rig#t of t#e left coronary orifice'
T#ey yello% plastic suture boot on t#e pic4up is pointing out t#e left coronary orifice'
Cigures 22 E 23: ro/imal ascending aorta no% opens %idely$ revealing t#e rig#t and left coronary orifices and t#e normal trileaflet aortic valve belo%'

Cigures 24 E 25: An incision is performed in t#e distal ascending aorta so t#at t#e
orifice of t#e completed distal reconstruction %ill correspond in si(e to t#e pro/imal
reconstructed aorta'
Cigures 2< E 2+: A s#ield!s#aped patc# is being placed in t#e incision in t#e pro/imal ascending aorta ! noncoronary cusp' Suturing is %it# +!2 polypropylene suture' T#e patc#
s#ould not be too %ide$ becaus t#is %ill lead to commissural stretc#ing and loss of central coaptation %it# reultant aortic valve insufficiency'

Cigures 2@ E 2;: -ompleted noncoronary cusp patc#' Cigure 32: A portion of t#ic4 intimal #yperplasia ad5acent to t#e left coronary orifice is
s#arply resected %it# a no' 11 blade'

Cigures 32 E 33: S#ield!s#aped #omograft patc# is being sutured into t#e incision in t#e
distal ascending aorta to augment t#e ascending aorta'
Cigure 34: 1<!mm dilator passes easily t#roug# reconstructed pro/imal ascending aorta'
T#is also s#o%s t#e t#ree complteed patc#es in t#e pro/imal aorta'

Cigures 3< E 3+: Aortic anastomosis is being performed %it# <!2 running polypropylene
suture'
Cigures 3@ E 3;: -ompleted aortic anastomosis' atient is no% off bypass and #as been dcannulated' ostoperative TGG s#o%s trivial aortic insufficiency' ostoperative left
ventricular and aortic pressure measurements %ere e,ual$ indicating no residual gradient'
Tips E itfalls
3ig# ascending aortic cannulation ad5acent to t#e innominate artery for ma/imum
e/posure of t#e ascending aorta and to allo% lengt# for positioning of t#e patc# in t#e
ascending aorta'
0ne dose of antegrade cardioplegia follo%ed by retrograde cardioplegia'
ulmonary #omograft or pericardial patc#es Htanned in glutaralde#yde1 for t#e Is#ieldsJ'
T#ese patc#es s#ould not be too large K t#is %ill lead to aortic insufficiency'
Cirst coronary sinus incision in noncoronary sinus to facilitate e/posure of t#e incisions
into t#e coronary orifice sinuses'
.n most cases$ t#e rig#t coronary artery sinus s#ould be opened to t#e left of t#e coronary
orifice and t#e left coronary artery sinus to t#e rig#t of t#e coronary orifice' 0ccasionally
one may not #ave enoug# room bet%een a coronary orifice and t#e aortic valveD in t#is
case t#e incision site s#ould be tailored to t#e situation'
Gnsure similar orifice si(es bet%een t#e pro/imal and distal ascending aorta after patc#
augmentation by t#e use of a si(ing dilator'
Transesop#ageal ec#ocardiograp#y to assess completeness of repair$ coronary blood flo%
postoperatively$ %all motion abnormalities$ and ade,uacy of aortic valve'
>esults
T#e results of t#e t#ree!patc# aortoplasty tec#ni,ue are s#o%n in Table 2' T#e report by
Stamm and associates from Boston -#ildren"s 3ospital$ in particular$ compared t#e
results of single!patc#$ bifurcated!patc#$ and t#ree!sinus reconstruction )+ *' >esults
improved greatly after t#e introduction of t#e t#ree!sinus tec#ni,ue$ a more symmetric
reconstruction of t#e aortic root' >esidual gradients %ere lo%er$ #emodynamics %ere
superior$ and t#ere %as a reduction in bot# mortality rate and need for reoperation'
Table 2' >esults of t#ree!sinus incision tec#ni,ue

Aut#or
Year
=umber of
atients
7edian Age
HYears1
L &illiams
Syndrome
7ortalit
y
7ean >esidual
8radient
7yers )5 * 1;;
3
2 ! Brom
4 ! 7yers
3'@ 3;
2
1
12mm 3g
3a(e4amp )<
*
1;;
;
13 15'@ 3@ 2 12mm 3g
Stamm )+ * 1;;
;
4 ! 7yers
2 ! Brom
+'4 <1
2
2
12mm 3g
Total
25 1