Download principles of surgery in complex congenital cyanotic heart disease

PRINCIPLES OF SURGERY IN
COMPLEX CONGENITAL CYANOTIC
HEART DISEASE
Dr Vinod
• Normal human circulation needs two ventricles
and two unobstructed circulations.
• Most cyanotic children coming for surgical
intervention have two ventricles still a good
number will have only one functional ventricle
(Single Ventricle)
• A few of them have only one circulation
connected to the heart(Single Circulation) while
the other circulation is supplied through the duct
(duct dependent circulation)
• The type of repair depends on the availability of
ventricles and connections of both circulations to
the heart.
When there are two balanced ventricles and
normally connected two circulations a
biventricular repair is possible as in Tetralogy of
Fallot, TGA and TAPVC which can give the baby a
nearly normal heart for the rest of life.
• When there is a functional single ventricle
with both circulations normally connected the
pulmonary circulation is disconnected from
the ventricle in stages first the SVC and later
the IVC so that the single ventricle needs to
support only the systemic circulation offering
the child the maximum possible palliation for
a longer time. This is the Fontan principle
Tricuspid Atresia
• Definite surgery is Fontan
• Ideal candidates for a Fontan-type operation
are those who have normal LV function and
low pulmonary resistance
• Staged palliative procedure before FONTAN
are aimed at producing ideal candidates for a
future Fontan procedure.
1. Normal LV function results from prevention
of excessive volume or pressure loading of
the LV by
a. Preventing excessive volume load by using a
relatively small systemic-to-pulmonary shunt
(e.g., 3.5 mm for neonates)
b. Avoiding ventricular hypertrophy ( e.g, by
relieving outflow obstruction).
2. Low pulmonary resistance may result from
a. Providing adequate PBF that promotes the
growth of PA branches with resulting increase in
the cross-sectional area of the vascular bed
b. Preventing distortion of the central pulmonary
arteries. A shunt operation is preferably done on
the right PA, which can be incorporated into the
Fontan operation.
c. Protecting the pulmonary vascular bed from
overflow or pressure overload by PA band when
PBF is increased
• Stage I.
Blalock-Taussig shunt
Damus-Kaye-Stansel operation
PA banding is indicated for infants with too
much PBF
1. Blalock-Taussig shunt.
TA with decreased PBF need the BT shunt
• This procedure results in the volume load on
the LV because the LV supplies blood to both
the systemic and pulmonary circulations.
• The shunt should be relatively small (e.g., 3.5
mm) and should not be left alone too long.
2. Damus-Kaye-Stansel and shunt operation
(TA+TGA+RESTRICTIVE VSD)
• The main PA is transected and the distal PA is
sewn over. The proximal PA is connected end to
side to the ascending aorta.
• A systemic-to-PA shunt is created to supply
blood to the lungs.
• This procedure also results in volume overload
to the LV and a stage II operation should be
performed as early as possible.
3. Pulmonary artery banding.
• PA banding protects the pulmonary
vasculature from developing pulmonary
hypertension, and it may be performed at
any age with a mortality rate of less than 5%.
•
Stage II.
1. Bidirectional Glenn operation (BDG).
An end-to-side SVC-to-RPA shunt ( bidirectional superior cavopulmonary shunt) is
performed by 2.5 to 3 months of age . By this time the PVR is sufficiently low to
allow venous pressure to be the driving force for the pulmonary circulation. For
this procedure to be successful, the PVR has to be relatively low because the SVC
blood flows passively into the pulmonary arteries. Any previous systemic-to-PA
shunt is taken down at the time of the procedure. This procedure increases
oxygen saturation which averages 85% without adding volume work to the LV
2. The hemi-Fontan operation.
An incision is made along the most superior part
of the right atrial appendage and is extended
into the SVC. A connection is made between this
opening and the lower margin of the central
portion of the PA. An intra-atrial baffle is placed
to direct blood to the pulmonary arteries. The
Blalock-Taussig shunt is taken down and the
native pulmonary valve is oversewn.
Stage III
• A modified Fontan operation is the definitive
procedure for patients with tricuspid atresia.
• Directing the entire systemic venous blood to
the pulmonary arteries without an
intervening pumping chamber.
• The Fontan operation is usually completed
when the child is around 2 years of age.
The following are risk factors for the Fontan operation:
The presence of two or more of these risk factors
constitutes a high-risk situation.
1.High PVR (>2 U/m2) or high mean PA pressure (>18
mm Hg)
2.Distorted PAs secondary to previous shunt
operations
3.Poor systolic or diastolic ventricular function with LV
end-diastolic pressure greater than 12 mm Hg or an
ejection fraction less than 60%
4.AV valve regurgitation
1 Following bidirectional Glenn procedure.
• An intra-atrial tubular pathway is created from
the orifice of the IVC to the orifice of the SVC
termed cavocaval baffle or a “lateral tunnel”
• The cardiac end of the SVC is anastomosed to
the undersurface of the RPA to complete the
operation
• An extracardiac conduit may be used to
complete the Fontan operation.
use fenestration (4 to 6 mm) in the baffle in high
risk patients.
• Advantages of fenestration
decompression of the systemic venous
circulation and augmentation of cardiac output
in the early postoperative period.
• Disadvantages include
systemic arterial desaturation with possible
systemic embolization from the systemic veins
and the later need to close the fenestration
2. Following the hemi-Fontan operation.
The intra-atrial patch that was used to direct
SVC blood to the PAs is excised and a lateral
atrial tunnel is constructed directing flow
from the IVC to the previously created
amalgamation of the SVC with the RPA
Hypoplastic left heart syndrome
Underdevelopment of the left ventricle aorta complex,
resulting in critical aortic valve stenosis or aortic valve
atresia with an intact ventricular septum is the most
recognized form of HLHS
Staged approach
The staged approach ultimately leads a singleventricle in-series circulation
stage 1 :
 reconstruction of the aortic arch into the right
ventricular outflow,
 separation of branch pulmonary arteries from
the right ventricle
 creation of a restrictive source of pulmonary
blood flow from a systemic artery or directly from
the single ventricle
Stage 2 :
unloads the single ventricle by replacing the
systemic-to-pulmonary shunt with a superior
cavopulmonary anastomosis .
• The staged pathway is completed by
modifications of a Fontan connection from the
inferior vena cava (IVC) to the pulmonary arteries
Stage 1:Norwood procedure
• 1) The main PA is divided, the distal stump is closed
with a patch, and the ductus arteriosus is ligated.
• 2) A right-side Gore-Tex shunt is created (with a 4- to
5-mm tube) to provide PBF while preventing CHF and
pulmonary hypertension. An RV-to-PA shunt, using
PTFE graft, was used (Sano modification). The
advantage includes a higher aortic diastolic pressure
thus a higher coronary artery perfusing pressure and
a lower PA mean pressure.
• 3) The atrial septum is excised to allow
adequate interatrial mixing.
• 4) Using an aortic or PA allograft, one
connects the proximal PA and the hypoplastic
ascending aorta and aortic arch.
Stage 2
Bidirectional Glenn procedure and the hemi-Fontan
procedure.
• 1) Cavopulmonary shunt ( the bidirectional Glenn
operation) is an end-to-side anastomosis of the SVC to the
right PA performed at 3 to 6 months of age in an effort to
reduce the volume overload to the systemic RV.
•
2) The hemi-Fontan operation. This procedure includes
augmentation of the central PA without dividing the SVC,
while excluding IVC blood from the pulmonary arteries
• A modified Fontan operation is performed at 12 to 18 months of
age.
Five important hemodynamic and anatomic features considered
essential to successful Fontan operation are
1 unrestrictive interatrial communication
2 competence of the tricuspid valve
3 unobstructed PA–to–descending aorta anastomosis (with pressure
gradient less than 25 mm Hg)
4 undistorted PAs and low PVR
5 preservation of RV function.
Significant TR appears to be an important predictor of poor
outcome of the Fontan operation.
TGA WITH VSD AND SEVERE PS
REV procedure (réparation à l'étage
ventriculaire)
First reported by Lecompte, performed for patients
with D-TGA with VSD and severe PS.
1 Infundibular resection to enlarge the VSD,
2 Intraventricular baffle to direct LV output to the aorta
3 Aortic transection in order to perform the Lecompte
maneuver (by which the right pulmonary artery [RPA]
is brought anterior to the ascending aorta)
4 Direct RV-to-PA reconstruction by using an anterior
patch . This may require fewer reoperations than the
Rastelli procedure
Nikaidoh procedure
(D-TGA+VSD+PS)
The aortic root is mobilized and translocated to the
pulmonary position.
The repair consists of the following:
1. Harvesting the aortic root from the RV with attached
coronary arteries in the original procedure
2. Relieving the LVOT obstruction by dividing the outlet
septum and excising the pulmonary valve
3. Reconstructing the LVOT with posteriorly
translocated aortic root and the VSD patch
4. Reconstructing the RVOT with a pericardial patch or a
homograft
Damus-Kaye-Stansel operation
(D-TGA+VSD+SUBAORTIC STENOSIS)
.
• The subaortic stenosis is bypassed by
connecting the proximal PA trunk to the
ascending aorta.
• The VSD is closed
• A conduit is placed between the RV and the
distal PA
Also done in patients with single ventricle and
TGA with an obstructive bulboventricular
foramen or DORV with subaortic stenosis
Single ventricle
• Most common form is
LV
• Great arteries are
transposed
• Aorta arising from
rudimentary RV
• PS in 50%
The surgical approach for single
ventricle
Initial surgical palliative procedures
Blalock-Taussig shunt is necessary for
cyanotic patients with PS or pulmonary
atresia . Shunt to the right PA is preferable
because any distortion of the RPA can be
incorporated later in the Fontan anastomosis.
In PGE1-dependent neonates, PDA is ligated
after placement of the shunt
• PA banding - infants with CHF and pulmonary
edema resulting from increased PBF
• The major risk factor is the presence or
development of an obstructed
bulboventricular foramen.
PA banding is performed only when the
bulboventricular foramen is normal or
unobstructed.
If the bulboventricular foramen is too small
1. The Damus-Kaye-Stansel operation is the preferred
procedure in the absence of pulmonary or subpulmonary
stenosis.
• PA-to-aorta anastomosis, by transection of the main PA
and anastomosis of the proximal PA to the ascending
aorta.
• This is combined with Blalock-Taussig shunt , single
ventricle to PA (Sano) shunt , or bidirectional Glenn shunt.
• A Fontan-type operation can be performed later .
2) Enlargement of the bulboventricular
foramen by a transaortic approach
• This procedure is performed especially when
PS is present.
• Second-stage surgical palliative procedures
a. A bidirectional Glenn operation is carried
out between 3 and 6 months before
proceeding with the Fontan operation.
b. Hemi-Fontan procedure
• After the second-stage surgical procedure,
the child needs to be followed up with
attention to the O2 saturation.
• Initially there is a remarkable improvement
in O2 saturation approximately 85% but a
gradual deterioration in O2 saturation may
occur in the months postoperatively related
to the development of pulmonary AV fistula
•
If the child's O2 saturation is 75% or less, one
may proceed with the Fontan procedure.
Cardiac catheterization is performed by 12
months after the second-stage operation.
Ideal candidates should have low mean PA
pressure (<16 to 18 mm Hg), low PVR (<2
units), and low end diastolic pressure less
than 12 mm Hg
Definitive procedures.
• The Fontan-type operation is performed at
18 to 24 months of age
• lateral tunnel Fontan procedure (also called
cavopulmonary connection) the procedure of
choice . Some centers make a 4- to 6-mm
fenestration in the baffle.
• extracardiac conduit modification of the
Fontan procedure.
DOUBLE OUTLET RIGHTVENTRICLE
• Palliative surgery
1.PA banding in multiple VSD
2.Atrial septostomy in Taussig Bing type
3.BT shunt in RVOT O
• Definitive surgery
Depends on type of DORV
Subaortic or Doubly Committed VSD.
• An intraventricular tunnel between the VSD
and the subaortic outflow tract is created by
a Dacron patch.
• RVOT may have to be augmented with an
outflow patch if the VSD-AO tunnel obstructs
the RV outflow tract
Fallot type
• 1.VSD-AO TUNNEL+Rastelli repair
• 2.REV procedure
• 3.Nikoidoh procedure
Taussig Bing type
• VSD-PA tunnel +ASO
1.BDG involves
A.SVC to RPA anastomosis
B.RV to PA
C.IVC to MPA
D.PA to aorta
• 2.Sano modification invoives
A.RV to PA
B.RV to Aorta
C.SVC to PA
D.IVC to PA
3.DKS procedure false statement
• A.PA to Aortic anastomosis
• B.done in TGA +VSD+PS
• C.done in TGA +VSD+subaortic stenosis
• D.RV-PA conduit present
• 4.REV procedure FALSE statement
• A.Done in TGA+VSD+PS
B.Direct RV – PA connection
• C.Lecompte maneuver performed
• D.RV –PA conduit is used
•
•
•
•
•
5.High risk factors for Fontan all except
A.mean PA pressure >18
B.LV end diastolic pressure >12
C.distorted PA
D.low PVR
• 6.Single ventricle with obstructed
bulboventricular foramen stage 1 palliation
include all except
• A.DKS
• B.PA Banding
• C.BT shunt
• D.widening of foramen
•
•
•
•
•
7.Procedure of choice in Taussig Bing anomaly
A.VSD to PA tunnel + ASO
B.Rastelli
C.REV
D,Nikoidoh
8.TGD+VSD+PS treatment includes all except
• A.Rastelli
• B.REV
• C.Nikoidoh
• D.DKS
• 9.DORV Fallot type treatment includes all
except
• A.BT shunt as palliative
• B.Rastelli
• C,REV
• D.ASO
10.Fontan advised in all except
• A.single ventricle of LV type
• B.HLHS
• C.TA
• D.DORV