1. Patients and Methods
Patients and methods
This study conducted on 50 children in preschool age
with TOF were considered candidates for total repair and,
were operated at El-Hussein University Hospital, Egypt
Children Hospital and National Heart Institute.
Inclusion criteria:
All patients were diagnosed as a TOF with favorable
anatomy for total correction, age above 6 months up to 6
years and/or weight over 8.5 Kg at presentation were
included for total correction weather primarily or after
initial palliation as a staged procedure.
Exclusion criteria:
Hypoplastic pulmonary arteries.
Neonates and small infants (less than 8.5 Kg).
Major coronary anomaly.
Major associated extra cardiac anomalies.
Patients with previous shunt operations.
Small left ventricle.
All children were subjected to the following:
A-Pre-operative assessment
1. History taking with emphasis on age, sex, date of
appearance of cyanosis, squatting and cyanotic spells
and its frequency and durations if present.
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2. Patients and Methods
2. Clinical examination including body weight, general
and local examinations.
3. Laboratory work up including Hemoglobin, coagulation
profile, CBC, liver and renal functions.
4. Chest X ray to assess the cardiac size, shape and
pulmonary vasculature.
5. Electrocardiography.
6. Echocardiography describing detailed cardiac anatomy
with special emphasis on degree and level of RVOTO,
diameters of the main pulmonary artery and its
branches, size and position of the VSD, degree of aortic
overriding, Mc-Goon ratio, cardiac dimensions, LV
index and presence of other abnormalities.
The assessment of adequacy of the size of branch
pulmonary arteries is based on the preoperative
echocardiographic and angiographic data namely;
McGoon's ratio correction was planned.
7. Cardiac catheterization if indicated to verify
echocardiography findings, saturations, pressures and
describing coronary anatomy.
8. Multi-slices CT and MRI.
B. Intra-operative assessment and operative technique:
Under general anesthesia, median sternotomy was the
standard incision. Dissection of the thymus gland with
removal of one or both lobes for better exposure if needed.
The pericardium is opened to the Rt. of the midline and
remains un-harvested for preparation of patch for repair.
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3. Patients and Methods
Examination of the general cardiac anatomy including
MPA, RPA, LPA, pulmonary annulus, RVOT, PDA,
PLSVC, examining the anatomy of the coronary arteries
and the size of the LV and RV, (Fig. 38).
Fig. 38: Showing the heart with deeply cyanotic
myocardium after opening the pericardium and
small main pulmonary artery (intra operative).
The aorta is dissected from the main and right
pulmonary arteries, which were examined as regard their
diameters. Prior to cannulation, full dose of Heparin (300
unit/Kg) was administrated; cardiopulmonary bypass was
instituted through aortic and bi-caval cannulation.
The aorta is cannulated high with a soft, flexible
cannula, whereas both cavae were cannulated directly with
right-angled metal-tipped cannulae.
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4. Patients and Methods
In patients with PLSVC (3 cases), dissection was done
around the PLSVC with tape passed around it , a third
venous cannula was ready for cannulation if venous return
was compromised so that, the third cannula could be passed
through the coronary sinus or direct cannulation of the Lt.
SVC then snaring it.
PDA is looked for routinely and ligated if present
prior to bypass, CPB was commenced with systemic
cooling to moderate levels of hypothermia (28C).
The aorta is cross-clamped and oxygenated blood
cardioplegia obtained from CPB machine is administered
into the aortic root which is repeated every 30 min, in
conjunction with topical ice saline slush.
Both vena cavae were snared, Rt. atrium was opened
longitudinally between SVC and IVC, an intra-cardiac vent
was inserted through already present foramen ovale or
through a small incision made into the intact inter-atrial
septum at site of foramen ovale.
As the Rt. atriotomy and the tricuspid valve were the
standard access to the VSD and RVOT, the intra-cardiac
anatomy was then inspected carefully, location and
extension of the VSD, its boundaries, the TV apparatus and
the RVOT.
Obstructing parietal muscle bundles were dissected
off the ventricular infundibular fold and transected 4 to 5
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5. Patients and Methods
mm away from the VSD and aortic annulus (toward the
right ventricular free wall). A wedge of muscle was
removed from this location, particularly in older patients
who may possess a large amount of hypertrophy of these
obstructing muscle bands then dissection was carried
upwards towards the pulmonary annulus and pulmonary
valve.
After excision of obstructing muscles, assessment of
the RVOTO is made by probing the PV with graded Hegar's
dilators, We were concerned to pass a Hegar dilator size
through the RVOT 2 mm larger than the predicted annular
size, as we considered this would ensure no significant
fixed anatomical obstruction in the outflow tract post-
operatively.
In patients with smaller pulmonary annulus, or in
those with pulmonary valve annulus less than 2 mm beyond
the predicted Z-value of zero, or it doesn't accept the
predicted Hegar's size, we opened the main pulmonary
artery longitudinally between two fine stay sutures (5/0
poly propylene) and the PV is inspected and dealt with
according to its nature.
If the pulmonary valve cusps were normal and either
bi-cuspid or tri-cuspid, full commissurotomies were
performed, and the valve was probed again for the expected
Hegar's dilator, but in case of cartilaginous cusps or
tethering of cusp tissue we excised the PV completely to
have no residual obstructions.
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6. Patients and Methods
Fig. 39: Limited incision is made longitudinally in the
RVOT (intra operative).
Fig. 40: Probing of the RVOT and pulmonary valve with
Hegar's dilator after relief of the RVOTO (intra
operative).
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7. Patients and Methods
Residual narrowing could either be due to a
restrictive PV annulus or hypoplasia of the RVOT.
If the PV annulus was narrow but the infundibular
obstruction had been successfully relieved, the valve
annulus was incised through the most anterior commissure
without ventriculotomy or by limiting the ventriculotomy
to 3 to 5mm below the annulus, just enough to place a
patch to effectively enlarge the annulus.
In cases where the outflow tract was still obstructed
(due to hypo-plasia rather than hypertrophy of the outflow
tract), we felt that a right ventriculotomy was necessary
and should be extended at least a few millimeters beyond
the length of the infundibular septum. Further division and
excision of muscle bands was undertaken through the
pulmonary valve before probing again the adequacy of the
RVOT with Hegar's dilators, (Fig.40).
The left and right pulmonary arteries were also
assessed and if necessary the incision in the MPA was
extended into either pulmonary artery to augment an origin
stenosis if needed.
After ensuring that we had a wide RVOT, we closed
the VSD with patch material of either Poly-Tetra-Fluoro-
Ethylene (PTFE or Teflon) or stretch-knitted poly-ethylene
terephthalate (Dacron) or Hemasheild, after trimming to
the appropriate shape.
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8. Patients and Methods
Interrupted pledgetted braided non-absorbable sutures
were used to sew the patch in place, this technique for VSD
repair was used in all patients, (Fig.41).
Fig. 41: Showing the VSD after placement of 4/0
interrupted sutures supported on Teflon
pledgets. And the aortic valve on the left side
while testing the valve competence.
In cases with combined approaches, the pulmonary
artery and RVOT were reconstructed using un-treated
autologous pericardium fixed in place by poly-propylene
5/0 suture on a relaxed heart, (Fig. 42).
The heart is then re-warmed and de-aired through
foramen ovale before its closure with 5/0 poly-propylene,
the aorta is de-clamped, de-airing of the heart is continued
through an aortic root vent. The RA is closed with 5/0
poly-propylene, both caval snares are taken off, and
rewarming completed.
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9. Patients and Methods
Fig. 42: After completion of repair of the VSD, the
pulmonary artery is repaired with trans-
annular pericardial patch to augment the RVOT
and the PA (intra operative).
After stabilization of hemodynamics, patients were
weaned from CPB, the venous cannulae were removed,
heparin was neutralized with protamine, and then the
arterial cannula was removed. Two retrosternal drainage
tubes were inserted, as well as two temporary pace-maker
wires which are attached to the RV wall and further two
wires to the RA wall if dual chamber pacing was required.
Direct pressure measurements were used to rule out
residual pressure gradients between RV and PA, and also
the ratio between the RV and LV (RV/LV). The
pericardium is left open and routine closure of the sternum
in layers was accomplished. Patient was then transferred to
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10. Patients and Methods
ICU ventilated and on calculated doses of inotropes and
vasodilators.
C. Post-operative assessment
1. Full ICU monitoring for pulse, invasive blood pressure,
core and peripheral temperatures, central venous
pressures, urine output, oxygen saturations, Arterial
Blood Gases (ABG) and the chest tube drainage.
2. Mechanical ventilation was maintained till the patient
became candidate for extubation.
3. ICU medications and need for inotropic support.
4. Fluid and electrolyte balance.
5. ECG for arrhythmias or any type of conduction
abnormalities.
6. CXR daily in the ICU and immediately before discharge
from the hospital.
7. Early post-operative echocardiography before discharge
from hospital.
8. Three months post operative echocardiographic follow
up.
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