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COARCTATION OF AORTA
MANAGEMENT
Dr. vikas
Deptt of ctvs, pgimer, chandigarh
SURGICAL MILESTONES AND
GENERAL CONSIDERATIONS
SURGICAL MILESTONES
SURGICAL
PROCEDURE
AUTHOR YEAR COUNTRY
Resection with end to end
anastomosis
Crafoord and
Nylin
1944 Sweden
Interposition graft Gross 1951 USA
Patch augmentation Vosschulte 1957 Germany
Subclavian flap aortoplasty Waldhausen and
Nahrwold
1966 USA
Resection with extended
end to end anast.
Amato 1977 USA
Surgical techniques
General considerations
 2 arterial lines: one in the right
radial artery, another in femoral
or umbilical artery.
 Ambient temperature in the
operating room.
 Heating- cooling blanket. In
neonates and young infants,
temperature maintained near
35oC and in older patients,
33oC to 34oC
General considerations (ctd.)
 Left posterolateral
thoracotomy through
the 4th Intercostal space
 The proximal
hypertension should not
be treated vigorously.
General considerations (ctd.)
 Upper lobe of left lung
retracted anteriorly,
medially and inferiorly
 Pleura overlying
isthmus is opened and
extended superiorly to
LSCA and inferiorly to
the level of 3rd and 4th
IC arteries.
General considerations (ctd.)
 Distal arch, descending aorta, ductus, LCCA,
LSCA dissected ,
 Dissection plane should remain just
superficial to the vascular adventitia to avoid
injury to nerves, thoracic duct and other major
lymphatics.
Intercostal arteries
 Ideally, no Intercostal artery should be sacrificed.
 In infants, gently placed small metal clips
 Older patients may require tourniquets or small
clamps
 May be dilated, aneurysmal.
 If ligated, should be away from Aorta (since origin is
the area of greatest weakness)
PDA
 Doubly ligated and divided
 Preserved in small neonates with borderline
small ventricle
General considerations (ctd.)
 After stabilization, the gradient across the
repaired segment is measure
< 10 mm Hg: acceptable
>10 but <20 mm Hg:
1. no technical error possible, gradients are
because of arch morphology: Accept it
2. morphology adequate, but technical error
possible: revision
> 20 mm Hg: un-acceptable
General considerations (ctd.)
 If anastomosis appears
under tension,
few superficial stitches are passed
through the adventitia of distal aorta and the
aorta is pulled up towards the arch. These
sutures are tethered to the nearby structures.
SPECIFIC SURGICAL TECHNIQUES
RESECTION AND END TO END
ANASTOMOSIS
 A curved side biting
clamp for distal arch
including the left
subclavian artery
 Angled, vascular cross
clamp for descending
aorta 1.5 cm below
ductus insertion
 Second assistant holds
the two clamps 2 cm
apart
Resection and End-to-End
Anastomosis (ctd.)
 Entire posterior suture
line is performed before
the clamps are
approximated
 Interrupted everting
horizontal mattress
sutures anteriorly
Resection and End-to-End
Anastomosis - Concern:
 Relatively high recoarctation rate
(10% to 86%) in age group <1 year:
1. ductal tissue
2. lack of growth at suture line
3. hypoplastic transverse arch
 use of silk suture
 Difficult in older patients:
Arch and DTA ‘fixed’ and difficult to mobilize.
Resection and End-to-End
Anastomosis - Advantages
 Complete removal of ductal tissue
 No prosthetic material
 Its modifications ( Extended resection and
anastomosis or resection-anastomosis with
reverse subclavian flap) used in management
of distal arch hypoplasia
RESECTION WITH EXTENDED
END-TO-END ANASTOMOSIS
 Amato reported 4 infants with hypoplastic
arch in ATS, 1977.
 In 1986, Lansman reported a series of 17
infants who had resection with extended end-
to-end anastomosis. (proximal clamp
between the IA and LCCA
 Elliot (ATS 1987) modification with a single
clamp to occlude the LSCA, LCCA, and even
part of IA (RADICALLY EXTENDED END-
TO-END ANASTOMOSIS)
Resection with extended end-to-end
anastomosis (ctd)
 Descending aorta is
extensively mobilized
(usually first three sets
of Intercostal vessels
are ligated and divided)
 Entire Coarct segment
and ductus are excised
 Incision on the inferior
surface of the
transverse arch.
Extended Resection and end to end
anastomosis
Extended Resection and end to end
anastomosis
Resection with extended end-to-end
anastomosis (Advantages)
 All the coarctation tissue with uncertain potential for
future growth is completely resected
 Left subclavian artery is preserved
 Procedure addresses and corrects hypoplasia of the
transverse arch, the distal aortic arch, and the aortic
isthmus.
 No prosthetic material
 Limits the potential for aneurysm formation
 Preserves normal vascular anatomy
 Paraplegia has not been reported as complication
Hypoplastic arch and extended
resection
- Reports of growth of a hypoplastic arch with
standard end-to-end anastomosis ( Brower et al,
JTCVS 1992) and
subclavian flap aortoplasty (Myers, ATS 1992)
- Some surgeons feel that extended arch repair
should be reserved for infants with transverse aortic
arch to ascending aorta diameter ratio of less than
0.25
- Some recommend extended resection for all infants
under 2 years of age
PROSTHETIC
PATCH AORTOPLASTY
 Vosschulte in
1957(Thorax ; 1961)
described
“isthmusplastic”
procedure that
developed into the
prosthetic patch
aortoplasty
Prosthetic patch aortoplasty (ctd.)
 Aorta incised
longitudinally through
the site of Coarctation
 Elliptical patch of PTFE
with the widest portion
at the level of the aortic
constriction
Advantages over simple resection with
end-to-end anastomosis
 Avoids extensive dissection
 Collateral vessels are all preserved and do
not require ligation and division
 Anterior suture line, easy control of bleeding
 Allows simultaneous enlargement of the
isthmic hypoplasia
 Tension free anastomosis
 Easy to perform, short clamp time
 Posterior aortic wall will grow
Prosthetic patch aortoplasty (ctd.)
 Resection of the coarctation ridge is no
longer performed
 Recommended for children older than 2 years
and younger than 16 years of age
( higher incidence of recoarctation if <1 year
of age and aneurysm formation if > 16 years
of age)
Prosthetic patch aortoplasty
Disadvantages
 Prosthetic material
 Ductal tissue left
 Aneurysm formation
PROSTHETIC INTERPOSTION
GRAFT
 First described by Robert Gross in 1951. He
used aortic homograft (Ann Surg, 1951)
 In 1960, Morris, Cooley, DeBakey and
Crawford described use of Dacron prosthetic
interposition graft 3% of their 171 patients
(JTCVS, 1960)
Prosthetic interposition graft
Recommendation for prosthetic
interposition graft
 Age > 16 years
 Associated aneurysm
 Complex long segment coarctation
 Recurrent coarctation
 If anastomosis appears under tension
 Thinned aortic wall in post stenotic dilatation
Disadvantages of interposition graft
 Size discrepancy in growing child
 Longer aortic cross clamp time to perform 2
circular anastomoses
SUBCLAVIAN FLAP
AORTOPLASTY
 Introduced by
Waldhausen and
Nahrwold (JTCVS,
1966)
 Successful coarctation
repair was reported in
three patients aged 4
months, 6 months and 3
years.
Subclavian flap aortoplasty (ctd.)
 Aorta clamped proximal
to LSCA
 LSCA ligated distally
 Opened along its lateral
margin
 Incision extended
through the isthmus,
Coarct site into the area
of poststenotic
dilatation.
Subclavian flap aortoplasty (ctd.)
 ‘flap’ sutured in place, creating a
‘roof’ over the area of previous
Coarct
Subclavian flap aortoplasty (ctd.)
 LIGATION OF VERTEBRAL ARTERY: leaving it
intact provides collateral circulation to the arm but
may possibly cause subclavian steal syndrome as
the child grows.
 If possible, LIMA and the Thyrocervical trunk are left
intact to provide collateral circulation to the arm (but
sacrificed if more length is needed)
 Short incision across the Coarct / short flap leads to
restenosis at a later date
Advantages of Subclavian flap
aortoplasty
 Simplicity
 Short cross-clamp time
 Avoidance of prosthetic material
 Easy anastomotic haemostatic control,
 Anastomotic growth owing to the use of an
autogenous noncircumferential flap.
Disadvantages
 Left arm ischemia in older children ( Geiss D,
JTCVS 1980; Wells WJ, ATS 2000)
 Concern for long term growth and function in
the left upper limb. (Todds, JTCVS 1983)
 Recoarctation ( ranging up to 42% in some
series)
Variations of SC flap technique
 REVERSED
SUBCLAVIAN FLAP:
Described by Hart and
Waldhausen for repair
of coarctation proximal
to the left subclavian
artery (ATS 1983)
Variations of SC flap technique (ctd)
Amato technique
 Alternative to reverse
subclavian flap
aortoplasty for distal
arch hypoplasia
 Bases of left common
carotid artery and the
subclavian artery are
sutured together
Variations of SC flap technique (ctd.)
modified EEA with SC flap
Variations of SC flap technique (ctd)
Subclavian Reimplantation
BALLOON DILATION ANGIOPLASTY
AND STENTS
BALLOON DILATION
ANGIOPLASTY
 First demonstrated for
neonatal coarctation in
1979 (Lancet, 1979)
Balloon angioplasty versus surgery
(Shady et al, Circulation 1993;87:793)
 First prospective comparison
 36 patients
 Age ranges 3-10 years
 All <1 cm coarctation
 Both procedure produced 86% reduction in
peak systolic gradient.
 Follow up aortogram in 19 and MRI in 21
Comparison of angioplasty and surgery for
unoperated coarctation of the aorta (Shady et al,
Circulation 1993;87:793)
Complications of balloon angioplasy
 Restenosis (residual gradient 20 mm Hg.):
20-35%
 Aortic dissection or rupture
 Aneurysm formation
 Femoral arterial complication:15%
Indications for balloon angioplasty
 Major systemic illness that significantly
increase the risk of surgical intervention
 Older patients with mild discrete coarctation
of the aorta and poorly developed collaterals.
Balloon dilatation for recurrent (post-
op) coarctation
 In contrast to ‘NATIVE’ coarctation, fibrous
postsurgical perivascular scar allows safe use
of this technique in ‘RECURRENT’ Coarct
 The previous method of surgical repair did
not affect the results.
 Considered as a “PROCEDURE OF CHOICE
FOR RECURRENT COARCTATION OF THE
AORTA AFTER SURGICAL REPAIR”
STENTING
 O’Laughlin et al in 1991
reported the first use of
endovascular stent.
 Recurrent coarctation of
the aorta and also as a
primary therapy for
native coarctation.
Stenting (ctd.)
 Implantation of stent after angioplasty limits
the risk associated with angioplasty and
minimal residual gradient.
 Improved luminal diameter
 Sustained hemodynamic affects
 Stents subsequently dilated as the child
grows
POTENTIAL COMPLIATIONS OF
SURGERY
POTENTIAL COMPLIATIONS OF
SURGERY
 Recoarctation
 Paradoxical hypertension
 Paraplegia
 Recurrent laryngeal nerve injury
 Left arm ischemia
 Hemorrhage
 Aneurysm formation
 Chylothorax
 Horner’s syndrome
 Phrenic nerve injury
 stroke
PARADOXIC POSTOPERATIVE
HYPERTENSION
 “THAT THE CORRECTION OF A
COARCTATION OF THE AORTA, AN
APPARENTLY STRAIGHTFORWARD
CAUSE OF HYPERTENSION, CAN
PROVOKE A POSTOPERATIVE INCREASE
IN BLOOD PRESSURE IS UNEXPECTED
AND ILLOGICAL, HENCE THE NAME”
Pathology of persistent hypertension
 Increased aortic wall stiffness
-Generalized vascular abnormality
-Upstream vascular abnormality
-Altered baroreceptor function
 Persistent humoral hyper-responsiveness
 Residual elevation of LV mass
-Myocardial hypertrophy
-LV hyperkinesis
-Endocardial fibro-elastosis
Pathology of persistent hypertension
(2 hypertensive responses)
 FIRST response occurs immediately
 Due to release of the stretch on the
baroreceptors in the carotid arteries and
aortic arch after removal of the obstruction
 Remains until the baroreceptors are set at a
lower level
 Occurs in 50% of patients
 In most cases subsides within 24 hours
Pathology of persistent hypertension
(2 hypertensive responses) ctd.
 SECOND phase is more pronounced in
diastole appears within 48 to 72 hours
 Occurs in about 1 / 3 of those experiencing
the first phase
 Raised renin and angiotensin
 Adaptation gone awry that ensures adequate
flow to exercising muscles below the
coarctation, above and beyond that delivered
by increasing the systolic pressure.
Hypertension (ctd)
 IV NTG, SNP, Esmolol, eventual conversion
to oral propranolol and captopril
 Hypertension usually resolves within 2 to 4
weeks after surgical correction
 Tendency for the hypertension to persist late
after repair is proportional to the age of the
child
Late hypertension (results)
 Seirafi and colleagues reported only 2 of 48
infants had late hypertension versus 16 of 59
patients operated on after 1 year of age (ATS
1998)
 The incidence of a normal BP was 90% at 5
years and fell to 50% and 25% at 20 and 25
years (Toro-Salazar, Am J Cardiol 2002)
 Normotensive patients any age often have an
exaggerated rise in systolic pressure in
response to exercise (Simsolo, Am Heart J
1998)
Late hypertension (ctd)
 Persistent hypertension after repair of
coarctation of the aorta despite medical
intervention merits investigation to rule out a
recurrent coarctation
COMPLIATIONS OF SURGERY
(ctd) MESENTRIC ARTERITIS
 Due to sudden increase in the arterial
pressure in these arterioles which were
previously accustomed to a very low blood
pressure
 Abdominal pain, distension, tenderness and
GI bleed on occasions
 NPO for first 48 hours after coarctation repair
for the fear of this complication
PARAPLEGIA
 First reported by Gross and Hufnagel as a
complication in animals (N Engl J Med 1945)
 Lerberg reported an incidence of paraplegia
of 1.5% (5/334) and correlated with the length
of aortic cross-clamping (ATS 1982)
 Crawford (JTCVS 1982) had intraoperative
hyperthermia associated with spinal cord
complications
Prevention of spinal cord ischemia
 Limit cross clamp time to less than 30
minutes.
 Do not sacrifice Intercostal arteries.
 Avoid clamping left subclavian artery. If
mandatory, try with partial clamp
 Systemic hypothermia (cooling blanket) to
34oC-35oC
 Topical cooling: wash left pleural cavity with
ice-cold saline.
Prevention of spinal cord and renal
ischemia (ctd.)
 Keep proximal aortic pressure to 100 to 120
mm Hg for infants, 160-200 mm Hg for older
children
 Drugs: methyl prednisolone (25 mg/kg).
Mannitol (1 gm/kg)
 Intrathecal papaverine.
 Avoid SNP during clamp
Prevention of spinal cord and renal
ischemia (ctd.)
 Monitor distal perfusion pressure after
proximal clamp. If < 50 mm Hg, supportive
measure to improve distal perfusion are
required:
- Intra-aortic shunt.
- Extra-aortic shunt. (Gott’s)
- Fem-Fem bypass
Preoperative identification of patients
with poorly developed collaterals.
 Absence of rib notching or palpable para
scapular pulsations in older patients.
 Unilateral rib notching.
 Only mildly diminished femoral pulses
( coarctation is not server enough and hence
collateral development will be poor)
 Decreased Lt or Rt radial pulse
ANEURYSM FORMATION
 Both True and False
aneurysms occur after
all types of repair for
coarctation of the aorta.
 Also occur in patients
with Coarctation not
undergoing surgical
repair (The risk of aneurysm
formation in untreated, native
coarctation has been estimated
to be 20% by the end of the
third decade of life)
Aneurysm formation
Risk factors
 Dacron patch
 Excision of coarctation ridge
 Age > 16 years
 Arch hypoplasia
 Repeat surgery
Aneurysm formation (ctd)
DACRON PATCH
 The incidence of aneurysm formation
appears to be significantly higher after
Dacron patch as compared to PTFE
Aneurysm formation after prosthetic
patch aortoplasty
 Posterior aortic wall opposite the patch
- Different tensile strength of the patch and the
posterior aortic wall, the pulsatile waveform
being completely directed to the posterior
aortic wall
Posterior coarctation membrane
( fibrous shelf)
 - In initial descriptions of
this procedure , the shelf
was excised, however, it
causes disruption of intima
and predisposes to aortic
aneurysm formation
- excision is no longer
recommended
Aneurysm formation (ctd)
Transverse arch hypoplasia
 Thomas et al (Ann Thorac Surg 2003;76:1090-1093)
 between 1970 and 1995 , 38 patients
 Aortic arch hypoplasia associated with
coarctation independently predicts future
aneurysm formation.
Aneurysm (management)
 Historically, this
complication has been
managed surgically but,
endoluminal repair via
exclusion of these
aneurysms has recently
been established as an,
less invasive
management option
HEMORRHAGE
 Even though none of the technique used
requires intravenous heparin (except if CPB
is used), moderate amount of bleed is
present until clots form within the needle
holes
CHYLOTHORAX
 Caused by traumatic lacerations of
lymphatics and thoracic duct
 Different therapeutic approaches:
- purely conservative (elemental diet or TPN)
- surgical (early or late)
- ligation of thoracic duct
- pleurodesis
- pleuroperitoneal shunts
Recoarctation and complex coarctation
RECOARCTATION
 Recoarctation or residual coarctation is
defined as:
“ A postoperative arm-to-leg peak systolic
pressure gradient exceeding 20mm Hg
across the repaired area”
Recoarctation (ctd)
 Age < 3 months
 Weight < 5 Kg
 Morphology of Coarct
 Suture material
 Technique
 Residual ductal tissue
Recoarctation (ctd)
TECHNIQUE
 PATCH AORTOPLASTY is excellent for older
children but probably should not be used in
infants because of the high recoarctation rate
 EXTENDED RESECTION WITH END TO
END ANASTOMOSIS appears to have the
lowest recoarctation rate
(Mavroudis and Backer, 3rd edition)
Recoarctation (ctd)
 SUTURE MATERIAL AND RESIDUAL
DUCTAL TISSUE:
High incidence of recoarctation in initial
reports due to use of silk sutures, Inadequate
resection of ductal tissue, circumferential
suture line
Recoarctation diagnosis
 Doppler, MRI, DSA
 Most effective approach is “ Physical
examination + MRI”
 Pressure measurement after the exercise
(not resting gradient)
Recoarctation Management
-Balloon angioplasty is now considered the
initial procedure of choice
-25% of patients have short lived improvement
-Repeated procedures can be safely performed
-Stenting- lower recurrence and aneurysm
formation
-Surgery if balloon angioplasty unsuccessful or
not indicated
REOPERATION
 Difficult due to scarring and increased risk of
paraplegia ( since gradient is not high so the
collaterals are not well formed)
 Left heart bypass or hypothermic circulatory
arrest should be seriously considered
 Patch angioplasty, resection and interposition
graft, extra-anatomic bypass graft technique
Complex coarctation of aorta
 Defined as a “ long coarctation or
recoarctation segment, a pseudoaneurysm at
a previous aortic isthmus suture line, or
concomitant hypoplasia of the aortic arch”
Extra-Anatomic Aortic Bypass
Grafting
 Indications:
 coarctation or recoarctation and associated
cardiac problems that required repair through
median sternotomy
 complex coarctation or recoarctation, with
anticipated difficulties with direct anatomic
repair
Extra-Anatomic Aortic Bypass Grafting (ctd)
Ventral aorta repair
 midline sternotomy, extended
into an upper midline
laparotomy
 supraceliac abdominal aorta
dissected and looped
 distal anastomosis to this
portion of the aorta performed
first
 tunneled through a fenestration
in the right hemidiaphragm
 carried anterior to the inferior
vena cava along the lateral
border of the right atrium, and
anastomosed proximally on the
right lateral aspect of the
ascending aorta
Extra-Anatomic Aortic Bypass
Grafting (ctd)
 1980, Vijayanagar et al ( JTCVS 1980)
described exposure of the descending
thoracic aorta through a median
sternotomy and posterior pericardium,
with graft around the left margin of
heart
 Powell et al (Tex Heart Inst J. 1983 )
described a modification of this
technique, which routed the graft
around the right margin of the heart
Associated cardiac defects
VSD + CoA
 Both volume and pressure overload of LV
 Treatment options depend on:
- Size of VSD and degree of shunt
- Type of VSD
- Age
- Severity of coarctation
VSD + CoA
 1. LARGE VSD, SEVERE COARCT IN FIRST
MONTHS WITH INTRACTABLE CCF:
 Emergency
Coarct
Repair
 CCF
persistant
 VSD
closure
 stable
 MFU / VSD
closure
later
 Swiss
cheese
 PA Band
VSD + CoA (ctd.)
 LARGE VSD, SEVERE
CoA,
PRESENTS BEYOND FEW
MONTHS OF LIFE
 SIMULTENOUS REPIAR
VSD + CoA (ctd.)
 SEVERE
COARCT
 SMALL VSD
 ONLY CORCT
REPAIR
 SUBSEQUENT
VSD
REPAIR RARE
VSD + CoA (ctd.)
 LARGE VSD
 COARCT
MODERATE
 VSD REPAIR
 COARCT REPAIR
6-12 MTHS OF
AGE
Coarctation of the aorta and associated cardiac defects:
Congenital Valvular Lesions
 Bicuspid aortic valve is found in 20%to 85%of
patients.
 Significant stenosis and/or regurgitation develops in
up to two thirds of cases, of whom at least 10% will
require aortic valve replacement
 associated with a risk of aortic aneurysm and
dissection
 Bicuspid aortic valve is responsible for many of the
cases of cardiac failure, which accounts for up to
20% of late deaths
2-stage repair through median sternotomy and lateral
thoracotomy (Ann Thorac Surg 1997;64:1309-1311)
 correcting the valvular lesion before the
coarctation
 reduced forward flow through the coronary
arteries during the diastolic phase in the
setting of aortic incompetence
 myocardial blood flow is further substantially
reduced by the acute decrease in systemic
vascular resistance if the coarctation is
repaired first
one-stage approach
 1-stage simultaneous correction of both
lesions through a median sternotomy (Circulation.
2001;104:I-133.)
 number of surgical procedures and length of
hospital stay reduced
Pregnancy after coarctation repair
 Increased incidence of aneurysm formation
and rupture
 ACE inhibitors avoided (fetal skull abnormalities and renal
tubular dysgenesis).
 Beta blockers preferred for control of
hypertension
 Pregnancy postponed till aortic
dilation/aneurysm ruled out
Natural history vs. surgical results
Natural history
 first and largest post-mortem series was published by
Abbott in 1928, who collected findings from all 200
previously documented cases over the age of 2
years, dating from the first report of aortic coarctation
by Paris in 1791.
 Reifenstein, Levine and Goss subsequently reported
104 further cases from the literature dating from 1928
to 1947. The median age of death for all 304 cases
was 31 years, and 76% of deaths were attributed to
complications of the aortic coarctation.
Results of the repair of isolated
coarctation (Kirklin)
 SURVIVAL:
 Early deaths
-Neonates 2-10%
-Older infants and children
1%
 Among a heterogeneous
groups of patients
undergoing repair of isolated
coarctation, the one-month
and 1, 10 and 25 years
survival has been 98%, 97%,
91% and 81% respectively
Incremental risk factors for late death
 Late age at operation
 Associated cardiac anomalies
 Persistent or recurrent hypertension
 Persistent or recurrent coarctation
 Aneurysm formation
 Coronary artery disease
Decision making
TIMING of elective repair
 Debatable.
 Certain considerations are:
1. Higher incidence of re-Coarctation (10 – 30%) if
operated before 1 year of age.
2. Increased prevalence of residual hypertension with
age (6% between 1 to 5 years vs. 30-50% if
operated at later age.)
3. Increased complications (rupture, dissection,
aneurysm) with age
TIMING of elective repair (ctd.)
1. The normal descending aorta attains about
55% of its final diameter by 3 years of age
(Significant obstruction occurs only if the
aortic diameter is reduced by 50%)
2. Concern for ductal remnants till 3 months of
age.
TIMING of elective repair (ctd.)
 Current trend at some centers :
Operate at any time after the age of 3
months
 General consensus:
Operate asymptomatic patients at about 1
year of age or at the time of diagnosis is
made later after 1 year of age.
Decision making
 Coarctation should be repaired at the earliest to
minimize the incidence of late hypertension
 Resection/extended resection and end to end
anastomosis / subclavian flap for neonates
 Patch aortoplasty with PTFE for children > 2 years of
age
 For children with 2-16 year age group, interposition
graft placement
 Balloon dilation is the initial procedure of choice for
recoarctation, if unsuccessful, patch aortoplasty or
interposition graft
 Complex coarctation of the aorta single stage repair
THANK YOU

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Coarctation of aorta

  • 1. COARCTATION OF AORTA MANAGEMENT Dr. vikas Deptt of ctvs, pgimer, chandigarh
  • 3. SURGICAL MILESTONES SURGICAL PROCEDURE AUTHOR YEAR COUNTRY Resection with end to end anastomosis Crafoord and Nylin 1944 Sweden Interposition graft Gross 1951 USA Patch augmentation Vosschulte 1957 Germany Subclavian flap aortoplasty Waldhausen and Nahrwold 1966 USA Resection with extended end to end anast. Amato 1977 USA
  • 4. Surgical techniques General considerations  2 arterial lines: one in the right radial artery, another in femoral or umbilical artery.  Ambient temperature in the operating room.  Heating- cooling blanket. In neonates and young infants, temperature maintained near 35oC and in older patients, 33oC to 34oC
  • 5. General considerations (ctd.)  Left posterolateral thoracotomy through the 4th Intercostal space  The proximal hypertension should not be treated vigorously.
  • 6. General considerations (ctd.)  Upper lobe of left lung retracted anteriorly, medially and inferiorly  Pleura overlying isthmus is opened and extended superiorly to LSCA and inferiorly to the level of 3rd and 4th IC arteries.
  • 7. General considerations (ctd.)  Distal arch, descending aorta, ductus, LCCA, LSCA dissected ,  Dissection plane should remain just superficial to the vascular adventitia to avoid injury to nerves, thoracic duct and other major lymphatics.
  • 8. Intercostal arteries  Ideally, no Intercostal artery should be sacrificed.  In infants, gently placed small metal clips  Older patients may require tourniquets or small clamps  May be dilated, aneurysmal.  If ligated, should be away from Aorta (since origin is the area of greatest weakness)
  • 9. PDA  Doubly ligated and divided  Preserved in small neonates with borderline small ventricle
  • 10. General considerations (ctd.)  After stabilization, the gradient across the repaired segment is measure < 10 mm Hg: acceptable >10 but <20 mm Hg: 1. no technical error possible, gradients are because of arch morphology: Accept it 2. morphology adequate, but technical error possible: revision > 20 mm Hg: un-acceptable
  • 11. General considerations (ctd.)  If anastomosis appears under tension, few superficial stitches are passed through the adventitia of distal aorta and the aorta is pulled up towards the arch. These sutures are tethered to the nearby structures.
  • 13. RESECTION AND END TO END ANASTOMOSIS  A curved side biting clamp for distal arch including the left subclavian artery  Angled, vascular cross clamp for descending aorta 1.5 cm below ductus insertion  Second assistant holds the two clamps 2 cm apart
  • 14. Resection and End-to-End Anastomosis (ctd.)  Entire posterior suture line is performed before the clamps are approximated  Interrupted everting horizontal mattress sutures anteriorly
  • 15. Resection and End-to-End Anastomosis - Concern:  Relatively high recoarctation rate (10% to 86%) in age group <1 year: 1. ductal tissue 2. lack of growth at suture line 3. hypoplastic transverse arch  use of silk suture  Difficult in older patients: Arch and DTA ‘fixed’ and difficult to mobilize.
  • 16. Resection and End-to-End Anastomosis - Advantages  Complete removal of ductal tissue  No prosthetic material  Its modifications ( Extended resection and anastomosis or resection-anastomosis with reverse subclavian flap) used in management of distal arch hypoplasia
  • 17. RESECTION WITH EXTENDED END-TO-END ANASTOMOSIS  Amato reported 4 infants with hypoplastic arch in ATS, 1977.  In 1986, Lansman reported a series of 17 infants who had resection with extended end- to-end anastomosis. (proximal clamp between the IA and LCCA  Elliot (ATS 1987) modification with a single clamp to occlude the LSCA, LCCA, and even part of IA (RADICALLY EXTENDED END- TO-END ANASTOMOSIS)
  • 18. Resection with extended end-to-end anastomosis (ctd)  Descending aorta is extensively mobilized (usually first three sets of Intercostal vessels are ligated and divided)  Entire Coarct segment and ductus are excised  Incision on the inferior surface of the transverse arch.
  • 19. Extended Resection and end to end anastomosis
  • 20. Extended Resection and end to end anastomosis
  • 21. Resection with extended end-to-end anastomosis (Advantages)  All the coarctation tissue with uncertain potential for future growth is completely resected  Left subclavian artery is preserved  Procedure addresses and corrects hypoplasia of the transverse arch, the distal aortic arch, and the aortic isthmus.  No prosthetic material  Limits the potential for aneurysm formation  Preserves normal vascular anatomy  Paraplegia has not been reported as complication
  • 22. Hypoplastic arch and extended resection - Reports of growth of a hypoplastic arch with standard end-to-end anastomosis ( Brower et al, JTCVS 1992) and subclavian flap aortoplasty (Myers, ATS 1992) - Some surgeons feel that extended arch repair should be reserved for infants with transverse aortic arch to ascending aorta diameter ratio of less than 0.25 - Some recommend extended resection for all infants under 2 years of age
  • 23. PROSTHETIC PATCH AORTOPLASTY  Vosschulte in 1957(Thorax ; 1961) described “isthmusplastic” procedure that developed into the prosthetic patch aortoplasty
  • 24. Prosthetic patch aortoplasty (ctd.)  Aorta incised longitudinally through the site of Coarctation  Elliptical patch of PTFE with the widest portion at the level of the aortic constriction
  • 25. Advantages over simple resection with end-to-end anastomosis  Avoids extensive dissection  Collateral vessels are all preserved and do not require ligation and division  Anterior suture line, easy control of bleeding  Allows simultaneous enlargement of the isthmic hypoplasia  Tension free anastomosis  Easy to perform, short clamp time  Posterior aortic wall will grow
  • 26. Prosthetic patch aortoplasty (ctd.)  Resection of the coarctation ridge is no longer performed  Recommended for children older than 2 years and younger than 16 years of age ( higher incidence of recoarctation if <1 year of age and aneurysm formation if > 16 years of age)
  • 27. Prosthetic patch aortoplasty Disadvantages  Prosthetic material  Ductal tissue left  Aneurysm formation
  • 28. PROSTHETIC INTERPOSTION GRAFT  First described by Robert Gross in 1951. He used aortic homograft (Ann Surg, 1951)  In 1960, Morris, Cooley, DeBakey and Crawford described use of Dacron prosthetic interposition graft 3% of their 171 patients (JTCVS, 1960)
  • 30. Recommendation for prosthetic interposition graft  Age > 16 years  Associated aneurysm  Complex long segment coarctation  Recurrent coarctation  If anastomosis appears under tension  Thinned aortic wall in post stenotic dilatation
  • 31. Disadvantages of interposition graft  Size discrepancy in growing child  Longer aortic cross clamp time to perform 2 circular anastomoses
  • 32. SUBCLAVIAN FLAP AORTOPLASTY  Introduced by Waldhausen and Nahrwold (JTCVS, 1966)  Successful coarctation repair was reported in three patients aged 4 months, 6 months and 3 years.
  • 33. Subclavian flap aortoplasty (ctd.)  Aorta clamped proximal to LSCA  LSCA ligated distally  Opened along its lateral margin  Incision extended through the isthmus, Coarct site into the area of poststenotic dilatation.
  • 34. Subclavian flap aortoplasty (ctd.)  ‘flap’ sutured in place, creating a ‘roof’ over the area of previous Coarct
  • 35. Subclavian flap aortoplasty (ctd.)  LIGATION OF VERTEBRAL ARTERY: leaving it intact provides collateral circulation to the arm but may possibly cause subclavian steal syndrome as the child grows.  If possible, LIMA and the Thyrocervical trunk are left intact to provide collateral circulation to the arm (but sacrificed if more length is needed)  Short incision across the Coarct / short flap leads to restenosis at a later date
  • 36. Advantages of Subclavian flap aortoplasty  Simplicity  Short cross-clamp time  Avoidance of prosthetic material  Easy anastomotic haemostatic control,  Anastomotic growth owing to the use of an autogenous noncircumferential flap.
  • 37. Disadvantages  Left arm ischemia in older children ( Geiss D, JTCVS 1980; Wells WJ, ATS 2000)  Concern for long term growth and function in the left upper limb. (Todds, JTCVS 1983)  Recoarctation ( ranging up to 42% in some series)
  • 38. Variations of SC flap technique  REVERSED SUBCLAVIAN FLAP: Described by Hart and Waldhausen for repair of coarctation proximal to the left subclavian artery (ATS 1983)
  • 39. Variations of SC flap technique (ctd) Amato technique  Alternative to reverse subclavian flap aortoplasty for distal arch hypoplasia  Bases of left common carotid artery and the subclavian artery are sutured together
  • 40. Variations of SC flap technique (ctd.) modified EEA with SC flap
  • 41. Variations of SC flap technique (ctd) Subclavian Reimplantation
  • 43. BALLOON DILATION ANGIOPLASTY  First demonstrated for neonatal coarctation in 1979 (Lancet, 1979)
  • 44. Balloon angioplasty versus surgery (Shady et al, Circulation 1993;87:793)  First prospective comparison  36 patients  Age ranges 3-10 years  All <1 cm coarctation  Both procedure produced 86% reduction in peak systolic gradient.  Follow up aortogram in 19 and MRI in 21
  • 45. Comparison of angioplasty and surgery for unoperated coarctation of the aorta (Shady et al, Circulation 1993;87:793)
  • 46. Complications of balloon angioplasy  Restenosis (residual gradient 20 mm Hg.): 20-35%  Aortic dissection or rupture  Aneurysm formation  Femoral arterial complication:15%
  • 47. Indications for balloon angioplasty  Major systemic illness that significantly increase the risk of surgical intervention  Older patients with mild discrete coarctation of the aorta and poorly developed collaterals.
  • 48. Balloon dilatation for recurrent (post- op) coarctation  In contrast to ‘NATIVE’ coarctation, fibrous postsurgical perivascular scar allows safe use of this technique in ‘RECURRENT’ Coarct  The previous method of surgical repair did not affect the results.  Considered as a “PROCEDURE OF CHOICE FOR RECURRENT COARCTATION OF THE AORTA AFTER SURGICAL REPAIR”
  • 49. STENTING  O’Laughlin et al in 1991 reported the first use of endovascular stent.  Recurrent coarctation of the aorta and also as a primary therapy for native coarctation.
  • 50. Stenting (ctd.)  Implantation of stent after angioplasty limits the risk associated with angioplasty and minimal residual gradient.  Improved luminal diameter  Sustained hemodynamic affects  Stents subsequently dilated as the child grows
  • 52. POTENTIAL COMPLIATIONS OF SURGERY  Recoarctation  Paradoxical hypertension  Paraplegia  Recurrent laryngeal nerve injury  Left arm ischemia  Hemorrhage  Aneurysm formation  Chylothorax  Horner’s syndrome  Phrenic nerve injury  stroke
  • 53. PARADOXIC POSTOPERATIVE HYPERTENSION  “THAT THE CORRECTION OF A COARCTATION OF THE AORTA, AN APPARENTLY STRAIGHTFORWARD CAUSE OF HYPERTENSION, CAN PROVOKE A POSTOPERATIVE INCREASE IN BLOOD PRESSURE IS UNEXPECTED AND ILLOGICAL, HENCE THE NAME”
  • 54. Pathology of persistent hypertension  Increased aortic wall stiffness -Generalized vascular abnormality -Upstream vascular abnormality -Altered baroreceptor function  Persistent humoral hyper-responsiveness  Residual elevation of LV mass -Myocardial hypertrophy -LV hyperkinesis -Endocardial fibro-elastosis
  • 55. Pathology of persistent hypertension (2 hypertensive responses)  FIRST response occurs immediately  Due to release of the stretch on the baroreceptors in the carotid arteries and aortic arch after removal of the obstruction  Remains until the baroreceptors are set at a lower level  Occurs in 50% of patients  In most cases subsides within 24 hours
  • 56. Pathology of persistent hypertension (2 hypertensive responses) ctd.  SECOND phase is more pronounced in diastole appears within 48 to 72 hours  Occurs in about 1 / 3 of those experiencing the first phase  Raised renin and angiotensin  Adaptation gone awry that ensures adequate flow to exercising muscles below the coarctation, above and beyond that delivered by increasing the systolic pressure.
  • 57. Hypertension (ctd)  IV NTG, SNP, Esmolol, eventual conversion to oral propranolol and captopril  Hypertension usually resolves within 2 to 4 weeks after surgical correction  Tendency for the hypertension to persist late after repair is proportional to the age of the child
  • 58. Late hypertension (results)  Seirafi and colleagues reported only 2 of 48 infants had late hypertension versus 16 of 59 patients operated on after 1 year of age (ATS 1998)  The incidence of a normal BP was 90% at 5 years and fell to 50% and 25% at 20 and 25 years (Toro-Salazar, Am J Cardiol 2002)  Normotensive patients any age often have an exaggerated rise in systolic pressure in response to exercise (Simsolo, Am Heart J 1998)
  • 59. Late hypertension (ctd)  Persistent hypertension after repair of coarctation of the aorta despite medical intervention merits investigation to rule out a recurrent coarctation
  • 60. COMPLIATIONS OF SURGERY (ctd) MESENTRIC ARTERITIS  Due to sudden increase in the arterial pressure in these arterioles which were previously accustomed to a very low blood pressure  Abdominal pain, distension, tenderness and GI bleed on occasions  NPO for first 48 hours after coarctation repair for the fear of this complication
  • 61. PARAPLEGIA  First reported by Gross and Hufnagel as a complication in animals (N Engl J Med 1945)  Lerberg reported an incidence of paraplegia of 1.5% (5/334) and correlated with the length of aortic cross-clamping (ATS 1982)  Crawford (JTCVS 1982) had intraoperative hyperthermia associated with spinal cord complications
  • 62. Prevention of spinal cord ischemia  Limit cross clamp time to less than 30 minutes.  Do not sacrifice Intercostal arteries.  Avoid clamping left subclavian artery. If mandatory, try with partial clamp  Systemic hypothermia (cooling blanket) to 34oC-35oC  Topical cooling: wash left pleural cavity with ice-cold saline.
  • 63. Prevention of spinal cord and renal ischemia (ctd.)  Keep proximal aortic pressure to 100 to 120 mm Hg for infants, 160-200 mm Hg for older children  Drugs: methyl prednisolone (25 mg/kg). Mannitol (1 gm/kg)  Intrathecal papaverine.  Avoid SNP during clamp
  • 64. Prevention of spinal cord and renal ischemia (ctd.)  Monitor distal perfusion pressure after proximal clamp. If < 50 mm Hg, supportive measure to improve distal perfusion are required: - Intra-aortic shunt. - Extra-aortic shunt. (Gott’s) - Fem-Fem bypass
  • 65. Preoperative identification of patients with poorly developed collaterals.  Absence of rib notching or palpable para scapular pulsations in older patients.  Unilateral rib notching.  Only mildly diminished femoral pulses ( coarctation is not server enough and hence collateral development will be poor)  Decreased Lt or Rt radial pulse
  • 66. ANEURYSM FORMATION  Both True and False aneurysms occur after all types of repair for coarctation of the aorta.  Also occur in patients with Coarctation not undergoing surgical repair (The risk of aneurysm formation in untreated, native coarctation has been estimated to be 20% by the end of the third decade of life)
  • 67. Aneurysm formation Risk factors  Dacron patch  Excision of coarctation ridge  Age > 16 years  Arch hypoplasia  Repeat surgery
  • 68. Aneurysm formation (ctd) DACRON PATCH  The incidence of aneurysm formation appears to be significantly higher after Dacron patch as compared to PTFE
  • 69. Aneurysm formation after prosthetic patch aortoplasty  Posterior aortic wall opposite the patch - Different tensile strength of the patch and the posterior aortic wall, the pulsatile waveform being completely directed to the posterior aortic wall
  • 70. Posterior coarctation membrane ( fibrous shelf)  - In initial descriptions of this procedure , the shelf was excised, however, it causes disruption of intima and predisposes to aortic aneurysm formation - excision is no longer recommended
  • 71. Aneurysm formation (ctd) Transverse arch hypoplasia  Thomas et al (Ann Thorac Surg 2003;76:1090-1093)  between 1970 and 1995 , 38 patients  Aortic arch hypoplasia associated with coarctation independently predicts future aneurysm formation.
  • 72. Aneurysm (management)  Historically, this complication has been managed surgically but, endoluminal repair via exclusion of these aneurysms has recently been established as an, less invasive management option
  • 73. HEMORRHAGE  Even though none of the technique used requires intravenous heparin (except if CPB is used), moderate amount of bleed is present until clots form within the needle holes
  • 74. CHYLOTHORAX  Caused by traumatic lacerations of lymphatics and thoracic duct  Different therapeutic approaches: - purely conservative (elemental diet or TPN) - surgical (early or late) - ligation of thoracic duct - pleurodesis - pleuroperitoneal shunts
  • 76. RECOARCTATION  Recoarctation or residual coarctation is defined as: “ A postoperative arm-to-leg peak systolic pressure gradient exceeding 20mm Hg across the repaired area”
  • 77. Recoarctation (ctd)  Age < 3 months  Weight < 5 Kg  Morphology of Coarct  Suture material  Technique  Residual ductal tissue
  • 78. Recoarctation (ctd) TECHNIQUE  PATCH AORTOPLASTY is excellent for older children but probably should not be used in infants because of the high recoarctation rate  EXTENDED RESECTION WITH END TO END ANASTOMOSIS appears to have the lowest recoarctation rate (Mavroudis and Backer, 3rd edition)
  • 79. Recoarctation (ctd)  SUTURE MATERIAL AND RESIDUAL DUCTAL TISSUE: High incidence of recoarctation in initial reports due to use of silk sutures, Inadequate resection of ductal tissue, circumferential suture line
  • 80. Recoarctation diagnosis  Doppler, MRI, DSA  Most effective approach is “ Physical examination + MRI”  Pressure measurement after the exercise (not resting gradient)
  • 81. Recoarctation Management -Balloon angioplasty is now considered the initial procedure of choice -25% of patients have short lived improvement -Repeated procedures can be safely performed -Stenting- lower recurrence and aneurysm formation -Surgery if balloon angioplasty unsuccessful or not indicated
  • 82. REOPERATION  Difficult due to scarring and increased risk of paraplegia ( since gradient is not high so the collaterals are not well formed)  Left heart bypass or hypothermic circulatory arrest should be seriously considered  Patch angioplasty, resection and interposition graft, extra-anatomic bypass graft technique
  • 83. Complex coarctation of aorta  Defined as a “ long coarctation or recoarctation segment, a pseudoaneurysm at a previous aortic isthmus suture line, or concomitant hypoplasia of the aortic arch”
  • 84. Extra-Anatomic Aortic Bypass Grafting  Indications:  coarctation or recoarctation and associated cardiac problems that required repair through median sternotomy  complex coarctation or recoarctation, with anticipated difficulties with direct anatomic repair
  • 85. Extra-Anatomic Aortic Bypass Grafting (ctd) Ventral aorta repair  midline sternotomy, extended into an upper midline laparotomy  supraceliac abdominal aorta dissected and looped  distal anastomosis to this portion of the aorta performed first  tunneled through a fenestration in the right hemidiaphragm  carried anterior to the inferior vena cava along the lateral border of the right atrium, and anastomosed proximally on the right lateral aspect of the ascending aorta
  • 86. Extra-Anatomic Aortic Bypass Grafting (ctd)  1980, Vijayanagar et al ( JTCVS 1980) described exposure of the descending thoracic aorta through a median sternotomy and posterior pericardium, with graft around the left margin of heart  Powell et al (Tex Heart Inst J. 1983 ) described a modification of this technique, which routed the graft around the right margin of the heart
  • 88. VSD + CoA  Both volume and pressure overload of LV  Treatment options depend on: - Size of VSD and degree of shunt - Type of VSD - Age - Severity of coarctation
  • 89. VSD + CoA  1. LARGE VSD, SEVERE COARCT IN FIRST MONTHS WITH INTRACTABLE CCF:  Emergency Coarct Repair  CCF persistant  VSD closure  stable  MFU / VSD closure later  Swiss cheese  PA Band
  • 90. VSD + CoA (ctd.)  LARGE VSD, SEVERE CoA, PRESENTS BEYOND FEW MONTHS OF LIFE  SIMULTENOUS REPIAR
  • 91. VSD + CoA (ctd.)  SEVERE COARCT  SMALL VSD  ONLY CORCT REPAIR  SUBSEQUENT VSD REPAIR RARE
  • 92. VSD + CoA (ctd.)  LARGE VSD  COARCT MODERATE  VSD REPAIR  COARCT REPAIR 6-12 MTHS OF AGE
  • 93. Coarctation of the aorta and associated cardiac defects: Congenital Valvular Lesions  Bicuspid aortic valve is found in 20%to 85%of patients.  Significant stenosis and/or regurgitation develops in up to two thirds of cases, of whom at least 10% will require aortic valve replacement  associated with a risk of aortic aneurysm and dissection  Bicuspid aortic valve is responsible for many of the cases of cardiac failure, which accounts for up to 20% of late deaths
  • 94. 2-stage repair through median sternotomy and lateral thoracotomy (Ann Thorac Surg 1997;64:1309-1311)  correcting the valvular lesion before the coarctation  reduced forward flow through the coronary arteries during the diastolic phase in the setting of aortic incompetence  myocardial blood flow is further substantially reduced by the acute decrease in systemic vascular resistance if the coarctation is repaired first
  • 95. one-stage approach  1-stage simultaneous correction of both lesions through a median sternotomy (Circulation. 2001;104:I-133.)  number of surgical procedures and length of hospital stay reduced
  • 96. Pregnancy after coarctation repair  Increased incidence of aneurysm formation and rupture  ACE inhibitors avoided (fetal skull abnormalities and renal tubular dysgenesis).  Beta blockers preferred for control of hypertension  Pregnancy postponed till aortic dilation/aneurysm ruled out
  • 97. Natural history vs. surgical results
  • 98. Natural history  first and largest post-mortem series was published by Abbott in 1928, who collected findings from all 200 previously documented cases over the age of 2 years, dating from the first report of aortic coarctation by Paris in 1791.  Reifenstein, Levine and Goss subsequently reported 104 further cases from the literature dating from 1928 to 1947. The median age of death for all 304 cases was 31 years, and 76% of deaths were attributed to complications of the aortic coarctation.
  • 99.
  • 100.
  • 101. Results of the repair of isolated coarctation (Kirklin)  SURVIVAL:  Early deaths -Neonates 2-10% -Older infants and children 1%  Among a heterogeneous groups of patients undergoing repair of isolated coarctation, the one-month and 1, 10 and 25 years survival has been 98%, 97%, 91% and 81% respectively
  • 102. Incremental risk factors for late death  Late age at operation  Associated cardiac anomalies  Persistent or recurrent hypertension  Persistent or recurrent coarctation  Aneurysm formation  Coronary artery disease
  • 104. TIMING of elective repair  Debatable.  Certain considerations are: 1. Higher incidence of re-Coarctation (10 – 30%) if operated before 1 year of age. 2. Increased prevalence of residual hypertension with age (6% between 1 to 5 years vs. 30-50% if operated at later age.) 3. Increased complications (rupture, dissection, aneurysm) with age
  • 105. TIMING of elective repair (ctd.) 1. The normal descending aorta attains about 55% of its final diameter by 3 years of age (Significant obstruction occurs only if the aortic diameter is reduced by 50%) 2. Concern for ductal remnants till 3 months of age.
  • 106. TIMING of elective repair (ctd.)  Current trend at some centers : Operate at any time after the age of 3 months  General consensus: Operate asymptomatic patients at about 1 year of age or at the time of diagnosis is made later after 1 year of age.
  • 107. Decision making  Coarctation should be repaired at the earliest to minimize the incidence of late hypertension  Resection/extended resection and end to end anastomosis / subclavian flap for neonates  Patch aortoplasty with PTFE for children > 2 years of age  For children with 2-16 year age group, interposition graft placement  Balloon dilation is the initial procedure of choice for recoarctation, if unsuccessful, patch aortoplasty or interposition graft  Complex coarctation of the aorta single stage repair