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tall and Debono in 1968 [2]_ The first total repair of AAE
Introduction was described in the same article. The procedure was also
performed by Bircks and Schulte about the same time [23].
Annulo-aortic ectasia (AAE) is an anatomical entity con- In the early 70s nine patients with AAE were operated on
sisting of the aneurysm in the ascending aorta and insuffi- in our institution with supracoronary technique. However,
ciency of the aortic valve. The term was introduced by Ben- the primary results were not satisfactory and late results
w i t h f o r m a t i o n o f r e - a n e u r y s m s w e r e e v e n w o r s e . In 1975 Patients
w e c h a n g e d the o p e r a t i o n t e c h n i q u e to a m o d i f i c a t i o n o f 30
B e n t a l l ' s o p e r a t i o n . T h e p r o c e d u r e , w i t h r e s e c t i o n o f the ] = Marfan patients
a n e u r y s m a l t i s s u e and w i t h o u t a n y w r a p p i n g , w a s later
• = non-Marfan patients
c a l l e d the o p e n t e c h n i q u e . In this r e p o r t w e p r e s e n t o u r en-
tire e x p e r i e n c e o f e l e c t i v e and e m e r g e n c y s u r g e r y for A A E
20
in 100 c o n s e c u t i v e p a t i e n t s d u r i n g a 1 9 - y e a r p e r i o d f r o m
1975 to 1994.
10
Patients and methods
Conventional cardiopulmonary bypass was used. In the first four pa- The mean follow-up time until December 1994 was 97 months
tients the myocardium was protected by selective coronary artery (range: 10 months-19 years)_ Each patient underwent a routine con-
perfusion. In all other 96 operations the measures for myocardial pro- trol angiography 6 months after surgery. Additionally, another con-
tection were as follows: systemic moderate hypothermia (28 °C), trol angiography was undertaken in 53 patients with a mean follow-
crystalloid cardioplegic solution (modified St. Thomas II) infused up time of 56 months (range: 36-142 months). All patients have
antegradely, topical cooling with ice-cold saline and cooling device undergone annual check-ups by our cardiologists.
(TCD) 1. The aortic valve was excised and the aneurysmal tissue re-
sected. Coronary ostia with a collar and the proximal parts of the cor-
onary arteries were dissected free from the surrounding tissue. The
composite graft was made simultaneously by sewing a Bj6rk-Shiley Results
tilting disc valve (standard from 1975 to 1978, convex-concave to
March 1983 and monostrut since then) inside a woven Dacron tube E a r l y results
graft (standard woven in the first four cases, Cooley low porosity in
15 cases and Cooley very soft low porosity in the remaining cases).
The composite graft was sewn to the aortic annulus with single 2-0 In the e l e c t i v e g r o u p o n e p a t i e n t d i e d d u r i n g the o p e r a t i o n
Ticron sutures. For implantation of the coronary arteries the tissue (1.3%). H e h a d b e e n o p e r a t e d on 6 y e a r s b e f o r e for the
collar around the coronary ostia was sutured, with continuous 4-0
A A E u s i n g the s u p r a c o r o n a r y t e c h n i q u e . T h e d i s e a s e d
Prolene sutures, to the holes made by a knife in the tube prosthesis.
The distal anastomosis was made with continuous 4-0 Prolene su- z o n e b e t w e e n the v a l v e p r o s t h e s i s and the t u b e p r o s t h e s i s
h a d w i d e n e d to a h u g e r e - a n e u r y s m . In the e m e r g e n c y
group two patients, both having acute dissection, died dur-
1 Code Laboratories, Lakewood, Colo., USA i n g s u r g e r y ( 1 6 . 6 % ) . R e s t e r n o t o m y for e x c e s s i v e p o s t -
Fig. 2 Total repair of annulo- patient died 3 years postoperatively due to a brain abscess
aortic ectasia accomplished us- probably caused by prosthetic endocarditis. In one case
ing the open technique
with chronic dissection in addition to AAE, the dissection
progressed to aneurysm in the thoracic aorta and the pa-
tient died due to rupture of the aneurysm. Three of the 13
deaths were suicides. Five patients succumbed to acute
myocardial infarction. One patient died due to esophageal
carcinoma. The actuarial survival curve for the 78 patients
without Marfan syndrome and 22 with the syndrome are
shown in Fig. 3.
In the control angiograms carried out 6 months after sur-
gery no pseudo-aneurysms at the coronary ostia, distal
anastomosis or proximal anastomosis could be seen, and
none were detected in later angiograms either. Of 53 cases,
a slight-to-moderate dilatation in one or both coronary or-
igins was observed in 15 (Figs. 4a, and 4b) of whom
Patients (N) 9 were patients with classic Marfan syndrome.
100
Discussion
0 1 2 3 4 5 6 7 8 9 10
used; the aneurysmal tissue was nearly totally removed and
the origins of the coronary arteries were dissected free from
Follow-up (years)
the surrounding tissue. The advantages of this open method
Fig. 3 Actuarial survival of the Marfan and non-Marfan patients are obvious: no tension in the coronary anastomoses, no
following the open surgical procedure for annulo-aortic ectasia later pseudo-aneurysms. The procedure can be carried out
in both elective and emergency cases with acute dissection
superimposed on the aneurysm. The open method can also
be applied in cases without any upward dislocation of the
operative bleeding was performed in five patients (5.0%). coronary origins, even though the opposite is claimed by
Four patients needed permanent pacemakers because of others [18].
total atrio-ventricular (AV) block caused by surgery. In all cases we used the same type of non-porous tube
prosthesis with good results; the incidence of resternoto-
mies for excessive postoperative bleeding is comparable
Late results with those in other series [14, 20]. The only disadvantage
of the tube prosthesis is its hard texture, which makes it
In the follow-up period of up to 19 years there were 13 late difficult to handle.
deaths altogether, four of which were related to surgery. During the last 10 years many centers have switched
One patient died 3 months after surgery due to anoxic brain from the original Bentall procedure and its modifications
damage caused iatrogenically during an emergency rester- to the open method [9, 13, 19]. We have used the same
notomy in the intensive care unit. Another patient suc- method since 1975 and can present early and late results
cumbed 3 months postoperatively due to multiple emboli which are at least comparable with those achieved using
caused by neglected anticoagulant therapy. An autopsy other procedures, Our primary mortality in the elective
showed emboli and infarctions in the heart, left kidney and group is very low and in the emergency group within the
brain, but also pseudo-aneurysm surrounding the tube range reported by others [3, 6, 13, 14, 19, 20]. In our ex-
prosthesis and compressing the left coronary artery. One perience the most important single factor contributing to
the mortality of urgent cases is diagnostic delay. At least most of the patients having the dilation in coronary arter-
in our hands, transthoracic echocardiography combined ies had classic Marfan syndrome with accentuated connec-
with the transesophageal echo is the fastest way to diag- tive tissue changes in the aortic wall. More important, no
nose acute cases. leakages or pseudo-aneurysms could be seen in coronary
In the group with late mortality there were only four anastomoses.
deaths which were related to surgery. Two points concern- Despite the improved results in the surgery of AAE, there
ing late mortality should be emphasized. One patient died are still two issues which should be emphasized: one is the
due to complication of a remaining chronic dissection. timing of the surgery and the other is the diagnosis of the
However, the incidence of complications developing in the disease. The recommendations concerning the critical
aortic arch and thoracic aorta has been higher in other re- upper limit of the aneurysm in the ascending aorta, with or
ports [12, 13]. To us it is an indication for closer follow- without insufficiency in the aortic valve, have changed with
up of the remaining thoracic aorta in the patients operated time. For instance, Gott and his group operated on Marfan
on for AAE. We are also concerned about the number of patients with AAE, even prophylactically, if the aneurysm
suicides in our late mortality. Two of them were in the ira- was more than 60 m m wide [11]_ However, today their
mediative postoperative phase and the third 3 years after recommendation for the limit is 55 m m or even only 50 m m
surgery. That provoked us to start a study on the quality of if the patient has a strong family history [10]. The others
life in these patients, who were often actually operated on do not want to give any strict limit in absolute width but
prophylactically without any preoperative symptoms. recommend surgery if the aneurysm is twice the diameter
The need for permanent pacemakers in our series has of the adjacent normal aorta [17]. Roman and her group
not been reported by others. In the open method, where have emphasized the significance of the pattern of aortic
there is no wrapping, sufficient tissue in the aortic annu- root dilation, at least in the Marfan patients [21]. Our policy
lus must be sutured to the rim of the valve prosthesis to is to operate, even without aortic valve insufficiency, if the
avoid paraprosthetic leakage. Some of these deep stitches aortic aneurysm exceeds 60 mm.
could damage the conductive tissue. Since we have started The diagnosis of AAE is relatively easy in cases with
using tissue glue to reinforce aortic valve sutured and cor- classic Marfan syndrome because of the typical habitus of
onary artery anastomoses, the stitches have been more the patients and the well-known heredity of the disease [1].
superficial and we have not seen any more total blocks. Most of us today are familiar with the cardiovascular man-
The dilatations at the coronary origins seen in the fol- ifestations of Marfan syndrome and its fatal complications,
low-up angiograms have not been reported by others. On such as acute dissection or rupture of the aneurysm. On the
the other hand, there are only a few reports with angio- other hand, to identify the patients with AAE but without
graphic follow-up [ 18]. The reason for the dilatation could Marfan syndrome is much more difficult. However, the he-
be diseased connective tissue in the walls of the coronary redity of AAE, even without classic Marfan syndrome,
arteries. In the patients operated on with the supracoronary seems probable [22]. That is why the relatives, at least the
technique, we have seen how the narrow rim between valve children and siblings of the patients operated on for AAE,
prosthesis and tube prosthesis progressively dilates to an routinely undergo clinical examination and echocardiog-
aneurysm. These findings are supported by the fact that raphy in our institution.
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