PREOPERATIVE DIAGNOSIS:
POSTOPERATIVE DIAGNOSIS:
Tetralogy of Fallot.
Tetralogy of Fallot.
PROCEDURE: Repair of tetralogy of Fallot:
1. Trans-atrial ventriculoseptal defect patch closure.
2. Trans-pulmonary artery repair of pulmonary valve.
3. Trans-artery resection of right ventricular outflow tract muscle
bundle.
4. Annulus and valve sparing repairs with augmentation of the main
pulmonary artery/right ventricle ventriculectomy with pericardial
patch.
5. Patent ductus arteriosus ligation and division.
6. Patent foramen ovale kept open.
PRIMARY SURGEON:
ASSISTANT:
V. Mohan Reddy, M.D.
Dharmapuram Kumar, M.D., Katherine, Medical Student
INDICATIONS FOR PROCEDURE: The patient is a 1-month-old male, who was
diagnosed two days after birth with tetralogy of Fallot. He has been
followed with Dr. Reitz and is scheduled to undergo a tetralogy of
Fallot repair on 08/22/2006 with Dr. Reddy. He presented to the Lucile
Packard Children's Hospital for preoperative evaluation. 2-D echo
evaluation showed severe tetralogy of Fallot with right aortic arch
with a large mal-aligned VSD with aortic override.
There was severe infundibular muscle obstruction with a gradient of
__________ mmHg. There was mild hypoplasia of the annulus measuring 7
mmHg. The pulmonary valve looked bicuspid and stenotic. The MPA was
mildly hypoplastic. The branch pulmonary arteries were confluent and
were fair sized. There was a PDA, which was small to moderate in size.
There was a PFO.
So in brief, the patient is a 1-month-old male, who was diagnosed with
tetralogy of Fallot, right aortic arch with patent ductus arteriosus
and the left SVC to the coronary sinus. All of the risks and benefits
of the procedure had been discussed in full with the mother and the
father and the baby was scheduled for the operation on 08/22/2006.
PROCEDURE IN DETAIL: The baby was anesthetized with general anesthesia
and after inducing general anesthesia and securing adequate lines of
monitoring, the patient was placed in the supine position and was
appropriately prepared and draped. A midline sternotomy was performed
and a sternal retractor was placed. The thymus was dissected and was
removed completely, leaving a small remnant of the top. There was a
small bridging communicating vein in view of the left SVC going to a
dilated coronary sinus. An adequate pericardial patch was harvested,
taking care not to injure the phrenic nerve on both of the sides. The
patch was prepared and was treated in glutaraldehyde for 20 minutes.
The external cardiac anatomy was inspected. Externally, it appeared to
be a severe tetralogy of Fallot. The aorta was normal in size. The
MPA appeared mildly hyperplastic. There was a moderate sized PDA. The
aortic arch was right-sided. There was a left SVC present, which
drained into a left coronary sinus. The pulmonary veins were draining
normally. The right atrium was normal in size, but the right ventricle
showed evidence of mild enlargement with hypertrophy. The coronary
artery anatomy appeared normal. Preparations were made for
cardiopulmonary bypass. The aorta and the main pulmonary artery were
dissected gently, very carefully and the PDA was looped with 5-0
Prolene and the PDA was resected. It was looped with 5-0 Prolene for
transfixation to be done after establishing cardiopulmonary bypass.
Pursestring sutures were placed on the aorta and both arteries, the SVC
and the IVC. The heparin was administered. A #8 aortic cannula was
placed in the aorta and both of the cavae were cannulated with #12
angled venous cannulae. Cardiopulmonary bypass was established and
cooling started to achieve a core temperature of 28 degrees Centigrade.
After establishing cardiopulmonary bypass, the PDA was immediately
ligated with 5-0 Prolene transfixation suture. The PDA was ligated and
divided in between two transfixation sutures of 5-0 Prolene and the
aortic end was clipped with a medium size Hemoclip. Both of the cavae
were looped in preparation for snugging. The main pulmonary artery was
resected and the branch of the pulmonary artery was mobilized. The
main pulmonary artery appeared mildly hypoplastic along with the mildly
hypoplastic annulus, but the branch pulmonary arteries appeared
confluent and were adequate in size. A #10 vent was placed into the
left ventricle from the right superior pulmonary vein across the LA.
After achieving the desired hypothermia, topical cooling of the heart
was performed and at the aortic root a pursestring was placed for
placing the cardioplegia needle. The aorta was crossclamped at a
temperature of 28 degrees Centigrade and cold crystalloid cardioplegia
was given. Both of the cavae were snugged and the right atrium was
opened and the cardioplegia was let out. After achieving cardiac
asystole, the main pulmonary artery was opened longitudinally in
between two stay sutures, which were placed, one above the annulus and
one below the bifurcation of the main pulmonary artery. The opening of
the main pulmonary artery was longitudinal extending from just above
the annulus to the level of the bifurcation. The pulmonary valve was
inspected and was found to be bicuspid. The leaflets of the pulmonary
valve appeared fairly thin without mitral thickening. They showed
evidence of fusion of both the commissures. A commissurotomy was
performed carefully by opening the commissural __________ and both of
the commissures onto the annulus and after this, the valve and annulus
were inspected and were found to be adequate from the pulmonary artery
end. Following this, the right ventricle outflow was opened by an
appropriately placed ventriculotomy in the region of the right
ventricle below the level of the annulus of the pulmonary valve. In
other words, the annulus was spared by not cutting across the annulus.
After making the ventriculotomy, the infundibular muscle obstruction
was examined and the muscle bundles were excised to open up the right
ventricle outflow tract. The excision of the muscle bundle was done to
the level of the annulus, but sparing the annulus and the pulmonary
valve. A #8 Hegar dilator was placed through the ventriculotomy across
the annulus into the main pulmonary artery and it was sized and found
to be adequate. Hence, a decision was made to do an annulus-sparing
repair by not cutting across the annulus and achieving the repair from
both the pulmonary artery and the right ventriculotomy sites.
Following this, the right atrium was inspected for evaluation of the
VSD. The VSD was found to be large and mal-aligned with significant
aortic override. The VSD was repaired with a pericardial patch that
was already treated with glutaraldehyde. The patch was appropriately
repaired to match the size of the VSD and the closure was done with 6-0
Prolene continuous suture. The suturing was started at the 3 o'clock
position and continued superiorly towards the aortic valve, taking care
not to injure the chordae of the tricuspid valve. At the superior end,
the sutures were brought out through the septal leaflet of the
tricuspid valve at the aortic mound. Inferiorly, the suturing of the
VSD patch was continued posteroinferiorly in a continuous fashion away
from VSD margin, taking care not to injure the __________. At the
posterior inferior angle of the VSD, the suture was brought out the
septal leaflet of the tricuspid valve into the right atrium. On the
site of the septal leaflet of the tricuspid valve, the VSD was closed
with a series of interrupted mattress sutures of 6-0 Prolene that were
taken through a small pledget of pericardium. In other words, this
margin was repaired with four interrupted mattress sutures of 6-0
Prolene. All of the sutures were tied down to the septal leaflet of
the tricuspid valve. A continuous suture of 6-0 Prolene was tied at
the superior end and the inferior end onto the nearest mattress suture
of 6-0 Prolene to complete the VSD closure. Following this, cold slush
cardioplegia was repeated from the aortic root, taking care that the
aortic root was adequately de-aired by making a small arteriotomy at
the root of the aorta. After repeating the cardioplegia, the VSD
closure was completed on the side of the septal leaflet of the
tricuspid valve. Following completion of the VSD closure, the atrial
septum was inspected and was found to have a patent foramen ovale with
the valve. This PFO was kept open. Following this, preparations were
made to release the aortic crossclamp by stopping the left atrial vent
and de-airing the aortic root adequately. The aortic crossclamp was
released. After releasing the right aortic crossclamp, the heart
started to regain its rhythm gradually. Rhythm was slowly established.
On beating heart, the main pulmonary artery and the right ventricular
infundibulum were augmented with a pericardial patch as follows: On
the main pulmonary artery, the pericardial patch augmentation was
performed with an oval-shaped pericardial patch that was sutured with
6-0 Prolene continuous suture all around and with interrupted sutures
of 7-0 Prolene towards the annulus. After this, the RV ventriculotomy
was augmented with another pericardial patch that was sutured to the
ventriculotomy incision with 6-0 Prolene continuous suture all around,
but with interrupted sutures of 6-0 Prolene towards the annulus. This
technique of placing interrupted sutures at the level of the annulus
for augmenting both the MP and the ventriculotomy was mainly to prevent
a pursestring effect. After augmentation of the main pulmonary artery
and the right ventriculotomy, the right atrium was closed with 6-0
Prolene continuous suture. The caval snuggers were released and rewarming was slowly completed. At this juncture, the left atrial vent
was removed and the left atrial line was placed into the left atrium.
At the end of the re-warming, preparations were made to wean the heart
off CPB. Intraoperative TEE was done for evaluation of the repair.
Moderate inotropic support was started with 5 mg of dopamine and 0.5 of
milrinone and an infusion of calcium. This was done through the
jugular vein. The heart was weaned off cardiopulmonary bypass with
moderate inotropic support and with stable hemodynamics. The rhythm
appeared to be in sinus rhythm and the myocardium showed good
contraction. Intraoperatively showed a good repair with an intact VSD
patch without any significant residual defects in the patch. There was
no significant gradient in the RV outflow and the pulmonary valve
showed good coaptation and without a significant gradient. There was
no evidence of tricuspid regurgitation. The PFO was shunting left to
right. The right ventricle and the left ventricle showed good
contraction and no residual valve motion abnormality. There was
evidence of good blood flow into the patched pulmonary arteries. After
ascertaining an adequate repair by an intraoperative TEE, Protamine was
administered and the patient subsequently underwent decannulation. The
hemodynamics remained stable with good saturations and with acceptable
left atrial pressures. Two atrial pacing wires and two ventricular
pacing wires were placed. The left chest was opened widely and a #15
Blake was placed to drain fluid out. Decannulation was performed and
hemostasis was checked at all of the cardiotomy sites including the
ventriculotomy sites. After ascertaining adequate hemostasis, a #20
chest tube was placed in the mediastinum from the right side. The
sternum was closed with steel wires and the subcutaneous tissue and the
skin were closed in the usual fashion.
The baby was transferred to the CVICU with stable hemodynamics and
saturations and with acceptable LV pressures and without significant
mediastinal bleeding.