[Voiceover:] This leader is provided to permit the projectionist to complete adjustment
of focus and sound level before the film
begins.]
[Narrator:] Elastic tissue abnormalities in the blood vessels of patients with Marfan's disease
predispose these patients to
the formation of aneurysms.
These aneurysms may be found in a variety of forms and locations, and dissecting
processes involving the ascending aorta
are not unusual.
This 30-year-old man was admitted to the hospital with a history of severe substernal pain
radiating through to the back and down into the lumbar region, one and a half years prior
to admission. This was followed by
transient numbness and weakness in the
right leg, but these symptoms subsided
spontaneously over the next several days.
Three weeks prior to admission, he again
experienced an episode of severe
substernal pain with radiation to the
back. Roentgenographic examination
of the chest demonstrated enlargement of his ascending aorta. Examination revealed
the patient to be six feet eight inches
in height with long, slender hands,
fingers, feet, and toes. In both legs there
was wide separation between the anterior
tibial tubercle and the inferior border
of the patella, characteristic
of Marfan's disease. No abnormalities were noted in either eye. The heart was
slightly enlarged to percussion, and an
early grade 2 decrescendo diastolic
murmur was heard at the base with
radiation toward the apex.
The blood pressure was 120 over 60 millimeters of mercury in both arms, and all peripheral
pulses were present and of a water
hammer characteristic.
Intravenous contrast visualization of the thoracic aorta demonstrated aneurysmal dilatation
of almost the entire ascending portion.
The transverse and descending portions
of the thoracic aorta did not appear
unusual.
There was no roentgenographic evidence
of a double density characteristic of a
dissecting process. Under general
endotracheal anesthesia,
a median sternotomy incision was made.
The sternum was split with a Lebsche
knife.
The pericardium was opened
longitudinally.
As the sternum was spread, the aneurysmal
process was seen to extend from the
aortic valve annulus to just proximal to
the innominate artery.
This drawing illustrates the extent of
aneurysmal involvement.
Cardiopulmonary bypass was instituted employing a disposable plastic oxygenator primed
with 5 percent dextrose in distilled water
under normothermic conditions.
The vena cavae were drained by gravity into the pump oxygenator, and a sump in the left ventricular apex
allowed for a left heart decompression. Oxygenated blood was
returned to the femoral artery through a
roller pump, and cannulae were used
to profuse the coronary arteries during the proximal anastomosis.
Clamps were placed across the aorta
between the aneurysm and the innominate artery.
The aorta was divided between the
clamps.
The distal cut end of the aorta was
freed up to the innominate artery and re-clamped.
A small septum representing the
distal portion of the dissecting process was excised.
A woven, crimped Dacron graft of the appropriate size was selected and
anastomosed to the distal cut end of the
aorta, employing an over-and-over
suture of 3-0 linear polyethylene, as
demonstrated in this drawing.
While the distal anastomosis was performed, the coronary arteries were perfused through
the aneurysm itself, from coronary sinus
blood oxygenated by intermittent
inflation of the lungs and by bronchial
artery returned to the left side of the heart.
Over-distension of the left heart
during the maneuver was prevented
by intermittent decompression through the sump in the left ventricular apex.
Upon completion of the distal anastomosis,
a vascular clamp was placed
across the graft,
and the integrity of the anastomosis was tested.
The left ventricular sump was used to evacuate the aneurysm, and the aneurysm was entered.
The double walls of the true and false lumen were seen with a tear in the inner wall, just above the aortic valve annulus.
The cusps of the aortic valve
were relatively normal. Aortic valvular
insufficiency resulted from prolapse of
the cusps into the ventricle, secondary
to prolapse of their attachments to the
inner wall.
This prolapse of the cusps in relation
to their normal position is demonstrated
in this illustration.
Coronary cannulae were inserted, and coronary perfusion was begun.
A major portion of the aneurysm was
excised, leaving part of the outer wall
for future support of the graft.
The graft was pulled down and cut to length.
The proximal anastomosis was begun
posteriorly in such a manner as to
pull the inner wall with its attachment
to the aortic valve cusps back into
normal position, during the performance
of the anastomosis.
This anastomosis was also performed with
an over-and-over suture of 3-0 linear
polyethylene, incorporating both the
inner and outer walls of the aorta,
obliterating the false lumen as the
prolapsed cusps were pulled back from
within the ventricle.
As the anastomosis neared completion, the right coronary cannula was removed,
but the left coronary cannula was left in
place as long as possible.
In this manner, excellent cardiac contractions were maintained.
Immediately prior to completion of the
proximal anastomosis, the tourniquet on
the superior vena cava was released and
the left heart decompression was stopped
temporarily to allow the heart to fill
with blood.
Prior to tying this suture, the clamp on the distal aorta was released briefly in order to allow the
graft to fill in a retrograde fashion,
evacuating air from the graft.
Finally, the proximal anastomosis was
completed, and the clamp was removed from
the distal aorta. The sump in the left
ventricular apex was used to evacuate
any air remaining in the ventricle,
following which it was removed.
The completed operation is demonstrated
in this drawing with a graft extending
from the aortic valve annulus to the
level of the innominate artery. The outer
wall of the aneurysm was cut to size.
The proximal anastomosis was wrapped with
surgical hemostatic gauze, and the
remaining portion of the outer wall of
the aneurysm was used to further support the graft.
The sternum was closed with
interrupted wire sutures.
The chest was closed in layers. The patient's course following operation was uneventful.
Retrograde contrast visualization of the
thoracic aorta was performed two weeks
following operation and demonstrated
satisfactory function of the graft
without evidence of aortic valvular
insufficiency. The patient was discharged
three days later with a blood pressure
of 120 over 75 millimeters of mercury in
both arms, and has remained asymptomatic.
