JTCS
HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS
QUICK SEARCH:   [advanced]


     

This Article
Right arrow Abstract Freely available
Right arrow Full Text (PDF)
Right arrow Alert me when this article is cited
Right arrow Alert me if a correction is posted
Right arrow Citation Map
Services
Right arrow Email this article to a friend
Right arrow Similar articles in this journal
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Add to Personal Folders
Right arrow Download to citation manager
Right arrow Author home page(s):
Osamu Tagusari
Hitoshi Ogino
Junjiro Kobayashi
Ko Bando
Kenji Minatoya
Kazuo Niwaya
Yutaka Okita
Motomi Ando
Soichiro Kitamura
Right arrow Permission Requests
Citing Articles
Right arrow Citing Articles via HighWire
Right arrow Citing Articles via Google Scholar
Google Scholar
Right arrow Articles by Tagusari, O.
Right arrow Articles by Kitamura, S.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by Tagusari, O.
Right arrow Articles by Kitamura, S.
Related Collections
Right arrow Great vessels

J Thorac Cardiovasc Surg 2004;127:1373-1380
© 2004 The American Association for Thoracic Surgery

Surgery for acquired cardiovascular disease

Should the transverse aortic arch be replaced simultaneously with aortic root replacement for annuloaortic ectasia in Marfan syndrome?

Osamu Tagusari, MDa,*, Hitoshi Ogino, MDa, Junjiro Kobayashi, MDa, Ko Bando, MDa, Kenji Minatoya, MDa, Hiroaki Sasaki, MDa, Kazuo Niwaya, MDa, Yutaka Okita, MDb, Motomi Ando, MDc, Toshikatu Yagihara, MDa, Soichiro Kitamura, MDa

a Department of Cardiovascular Surgery, National Cardiovascular Center, Osaka, Japan
b Department of Cardiovascular Surgery, National Cardiovascular Center, Kobe University, Osaka, Japan
c Fujita Health University, Aichi, Japan

Read at the Twenty-eighth Annual Meeting of The Western Thoracic Surgical Association, Big Sky, Mont, June 19-22, 2002.

Received for publication July 11, 2002; revisions received December 25, 2003; accepted for publication January 13, 2004.

* Address for reprints: Osamu Tagusari, MD, Department of Cardiovascular Surgery, National Cardiovascular Center, 5-7-1 Fujishirodai, Suita, Osaka 565-8565, Japan
otagusar{at}hsp.ncvc.go.jp


   Abstract
Top
 Abstract
Patients and methods
 Results
 Discussion
 References
 
OBJECTIVE: The purpose of this study was to determine, on the basis of the late fate of the intact aortic arch with abnormal tissue after aortic root replacement, whether the intact aortic arch should be replaced prophylactically at the time of aortic root replacement for annuloaortic ectasia in Marfan syndrome.

METHODS: A retrospective review was performed in 85 patients with Marfan syndrome who underwent aortic root replacement for annuloaortic ectasia with or without aortic dissection (mean age 37 years, range 19-61 years). These 85 patients were divided into four groups according to the postoperative condition of the residual aorta. In group I (n = 47), the patients underwent aortic root replacement for annuloaortic ectasia with or without localized dissection in the ascending aorta. In these patients the residual aorta, including the aortic arch, was therefore intact. In group II (n = 10), the aortic arch was intact, although the descending thoracic aorta was dissected because of the preoperative type B dissection. In groups III and IV, the patients had type A dissection involving the transverse arch associated with annuloaortic ectasia. In group III (n = 13), residual dissection existed in the descending thoracic aorta after concomitant total arch replacement. In group IV (n = 15), the aortic arch and the descending thoracic aorta were dissected.

RESULTS: There were 5 early deaths (3 in group I, 1 in group II, and 1 in group III). Subsequent operations were required in 10, 5, 6, and 7 cases in groups I, II, III, and IV, respectively. Regarding the aortic arch, only 2 of 53 survivors of the initial hospitalization with an intact aortic arch (groups I and II) underwent subsequent total arch replacement for the onset of dissection in the aortic arch, and 4 of 14 survivors of the initial hospitalization with a residual dissecting arch (group III) needed subsequent total arch replacement. Actuarial freedom from arch repair among patients with an intact aortic arch (91% at 15 years) was significantly higher than that among patients with a residual dissecting arch (49% at 15 years, P = .0078).

CONCLUSIONS: The incidence of new dissection in the residual intact arch after aortic root replacement was extremely low. Therefore prophylactic replacement of the intact arch does not appear to be necessary at aortic root replacement for annuloaortic ectasia in Marfan syndrome.

Since Dr Antonine Marfan first described a case of Marfan syndrome in 1896,1 there have been reports of numerous investigations on this inheritable disorder of connective tissue, which often evolves to cause progressive dilatation of the aortic root and ascending aorta.2 Recent advances in molecular biology and genetics have enabled us to elucidate the precise mechanism of this abnormal arterial elasticity,3,4 and it is now known that Marfan syndrome is associated with mutations in the fibrillin 1 gene on chromosome 15q21.1. Fibrillin is an important component of connective tissue microfibrils, which form a meshwork of elastin in the aorta. Depletion of fibrillin may alter the pattern of elastin deposition so that the aorta is less able to withstand recurrent wall stress, resulting in progressive aortic dilatation, dissection, or rupture. Prophylactic surgery thus is crucial to improvement of the overall prognosis for patients with Marfan syndrome. The current recommendation is to operate when the size of the aortic root or ascending aorta is 5.0 to 5.5 cm. In patients with additional risk factors, such as progressive dilatation of an aneurysm or a family history of dissection, rupture, or aortic regurgitation, surgery for annuloaortic ectasia should be considered for a smaller size of the aortic root.5-8 However, the patient with Marfan syndrome still has the potential risk of dissection or rupture in the residual intact aorta after aortic root replacement for isolated annuloaortic ectasia. Therefore emergency total arch replacement through median resternotomy will be required if acute dissection occurs in the residual aortic arch after aortic root replacement. It is generally accepted that elective concomitant total arch replacement for an intact arch has a lower risk than does emergency total arch replacement through median resternotomy.

The purpose of this study was to determine whether not the intact aortic arch should be replaced prophylactically and aggressively at the time of aortic root replacement for annuloaortic ectasia in Marfan syndrome. To accomplish this, we studied the late fate of the intact residual arch after aortic root replacement in a series of 85 patients.


   Patients and methods
Top
 Abstract
 Patients and methods
Results
 Discussion
 References
 
Between October 1979 and May 2002, a total of 117 patients with Marfan syndrome underwent surgery for cardiovascular disease at the National Cardiovascular Center, Osaka, Japan. The diagnosis of Marfan syndrome was based on internationally established diagnostic criteria.9 Among the 117 patients with Marfan syndrome, 85 underwent aortic root replacement for annuloaortic ectasia with or without aortic dissection at the initial operation. These patients were divided into four groups according to the postoperative condition of the residual aorta (Figure 1).



View larger version (43K):
[in this window]
[in a new window]
  		Figure 1. Eighty-five patients who underwent aortic root replacement for annuloaortic ectasia at initial operation were divided into four groups according to postoperative condition of residual aorta. Group I, all remaining aortas intact (n = 47); group II, aortic arch intact, although type B dissection remained (n = 10); group III, dissection remained in descending thoracic aorta after concomitant total arch replacement (n = 13); group IV, residual dissection including aortic arch persisted after aortic root replacement alone (n = 15).

 
In group I (n = 47), the patients underwent aortic root replacement for annuloaortic ectasia with or without localized dissection in the ascending aorta. In these patients the remaining aorta including the aortic arch was therefore intact. In group II (n = 10), the aortic arch was intact, although the descending thoracic aorta was dissected because of preoperative Stanford type B dissection. In the groups III and IV, the patients had Stanford type A dissection involving the transverse arch associated with annuloaortic ectasia. In group III (n = 13), persistent dissection existed in the descending thoracic aorta after concomitant total arch replacement. In group IV (n = 15), the residual aortic arch and descending thoracic aorta were dissected. The distribution of aortic disease in the groups is shown in Table 1.


View this table:
[in this window]
[in a new window]
  		TABLE 1. Distribution of aortic disease

 
This study was approved by the institutional review board of the National Cardiovascular Center, Osaka, Japan.

Follow-up
Data were prospectively collected from the database of the National Cardiovascular Center. Follow-up was performed in all cases up to May 31, 2002. The mean follow-up time was 110 ± 78 months (maximum 287 months).

Statistical analysis
The continuous data in this study are expressed as mean value ± SD, and categoric variables are expressed as percentage. Comparisons of the characteristics were performed with the {chi}2 test for nominal variables and the Student t test in the two groups for continuous and ordinal variables. Actuarial survival and freedom from reoperation and subsequent arch repair were estimated by the Kaplan-Meier product-limit method. Survival curves were compared between the groups with the log-rank test. Survivals presented in this report include in-hospital (death within 30 postoperative days or before hospital discharge). Reoperations that appeared with a nonbiased time course were expressed as linearized rates.


   Results
Top
 Abstract
 Patients and methods
 Results
Discussion
 References
 
Surgical procedure
Surgical procedures in each group are given in Table 2. Sixty-eight patients (80.0%) underwent composite graft repair. The prosthetic valves used in this study included 56 mechanical and 12 bioprosthetic valves. Seventeen patients (20.0%) underwent valve-sparing procedures (13 reimplantation, 4 remodeling).5,10 From 1990 onward, in all the patients with a dissected arch, excluding 4 who were in a state of shock, concomitant total arch replacement was performed. At total arch replacement, cervical vessels were reconstructed with a branched graft to exclude as much diseased aortic tissue as possible (Figure 1). This technique averted the problem of bleeding from the suture line in dissected aortic tissue.


View this table:
[in this window]
[in a new window]
  		TABLE 2. Types of operations

 
Operative morbidity and mortality
There were 5 early deaths (5.9%). The operative mortalities were 6.4% (3/47) in group I, 10.0% (1/10) in group II, 0% in group III, and 6.7% (1/15) in group IV. Causes of death were low cardiac output (n = 1), ventricular tachycardia (n = 1), and sudden cardiac death (n = 1) in group I; low cardiac output (n = 1) in group II; and low cardiac output (n = 1) in group IV. Postoperative complications included bleeding (n = 3), intraoperative balloon pump insertion (n=2), percutaneous extracorporeal membrane oxygenation support (n = 2), stroke (n = 1), respiratory dysfunction (n = 3), liver dysfunction (n = 4), and renal failure (n = 1) in group I; bleeding (n = 1), intraoperative balloon pump insertion (n = 1), left ventricular assist device insertion (n = 1), mediastinitis (n = 1), and liver dysfunction (n = 1) in group II; bleeding (n = 1), respiratory failure (n = 1), intraoperative balloon pump insertion (n = 1), and percutaneous extracorporeal membrane oxygenation support (n = 1) in group III; and bleeding (n = 1), renal failure (n = 1), and respiratory failure (n = 1) in group IV.

Reoperation
Thirty-seven (46.3%) of 80 patients who survived initial hospitalization required 67 subsequent operations for the remaining part of the aorta, valve regurgitation, or late complications such as pseudoaneurysm formation or prosthetic valve dysfunction after the initial operation. Focusing on reoperations for the residual aorta beyond the location of earlier repairs, 28 patients required 47 subsequent operations (Figures 2 and 3). The actuarial freedoms from reoperation for all patients were calculated to be 80.3% ± 4.8% at 5 years, 59.1% ± 6.9% at 10 years, and 48.0% ± 8.1% at 15 years. The linearized rate of reoperation was 6.0 ± 11.4 events per 100 patient-years in 80 patients who survived initial hospitalization.



View larger version (37K):
[in this window]
[in a new window]
  		Figure 2. Outcomes of patients in each group. Numbers in parentheses represent in-hospital deaths. TAR, Total arch replacement; DTAR, descending thoracic aortic replacement; AAR, abdominal aortic replacement; TAAR, thoracoabdominal aortic replacement; LD, late death. Asterisk indicates replacement of entire aorta; dagger indicates femerofemeral bypass for malperfusion because of acute type B dissection; double dagger indicates external iliac artery–superior mesenteric artery bypass for malperfusion because of acute type B dissection.

 


View larger version (26K):
[in this window]
[in a new window]
  		Figure 3. Actuarial freedom from reoperation in each group.

 
In group I, 10 of 44 patients who survived initial hospitalization underwent the second-stage operation. The actuarial freedoms from reoperation in the 44 initial survivors were 89.5% ± 5.7% at 5 years, 78.5% ± 7.4% at 10 years, and 64.3% ± 11.0% at 15 years. The linearized rate of reoperation was 2.7 ± 5.9 events per 100 patient-years. The reasons for the second-stage operation were onset of type B dissection in 7 patients, onset of aortic arch dissection in 2 patients, and abdominal aortic aneurysm in 1 patient. The time until the onset of type B dissection ranged from 16 months to 14 years after aortic root replacement. The actuarial freedoms from operation for the onset of type B dissection were 89.4% ± 5.1% at 5 years, 81.2% ± 7.2% at 10 years, and 73.9% ± 9.6% at 15 years. In 2 patients who received total arch replacement, the times after aortic root replacement until the onset of aortic arch dissection were 5 and 14 years. The actuarial freedoms from aortic arch repair were 97.0% ± 3.0% at 5 years, 97.0% ± 3.0% at 10 years, 89.5% ± 7.7% at 15 years, and 89.5% ± 7.7% at 20 years.

In group II, 5 of 9 patients who survived initial hospitalization required 8 subsequent operations for residual type B dissection. However, there was no onset of arch dissection or retrograde dissection toward the residual intact aortic arch. The actuarial freedom from reoperation among the 9 patients who survived initial hospitalization was 55.6% ± 16.6% at 5 years. The linearized rate of reoperation was 10.4 ± 10.6 events per 100 patient-years.

In group III, 6 of 13 patients who survived initial hospitalization required 10 subsequent operations for residual dissection in the descending thoracic aorta. The actuarial freedoms from reoperation were 47.6% ± 16.8% at 5 years and 35.7% ± 16.3 % at 10 years. The linearized rate of reoperation was 35.7 ± 16.3 events per 100 patient-years.

In group IV, 7 of 14 patients who survived initial hospitalization required 14 subsequent operations for dissection in the residual aorta including the aortic arch. The actuarial freedoms from reoperation were 92.3% ± 7.4% at 5 years and 36.9% ± 16.3% at 10 years. The linearized rate of reoperation was 10.4 ± 19.5 events per 100 patient-years. Among the 7 patients who underwent subsequent operations, 4 underwent total arch replacement. The actuarial freedoms from subsequent arch repair were 88.9% ± 10.5% at 5 years, 64.8% ± 16.5% at 10 years, and 48.6% ± 18.7% at 15 years.

Reoperation in patients with an intact aorta versus patients with a residual dissecting aorta
As mentioned previously, the residual aorta was intact after the operation in group I. On the other hand, patients in groups II, III, and IV had residual aortic dissection after the operation. Eighteen of 36 patients who survived initial hospitalization with aortic dissection required 32 subsequent operations. The actuarial freedoms from reoperation among these patients were 68.8% ± 8.3% at 5 years, 32.7% ± 10.6% at 10 years, and 24.6% ± 10.7% at 15 years (Figure 4). The linearized rate of reoperation was 10.2 ± 14.7 events per 100 patient-years among the 36 patients who survived initial hospitalization. There were significant differences in the actuarial freedom from reoperation (P = .0002) and linearized rate of reoperation (P = .0028) between the patients with and without aortic dissection.



View larger version (29K):
[in this window]
[in a new window]
  		Figure 4. Actuarial freedom from reoperation in patients with and without residual dissection, showing significant difference in actuarial freedom from reoperation (P = .0002) between groups.

 
Reoperation in patients with concomitant total arch replacement versus patients with aortic root replacement alone
Between patients with concomitant total arch replacement (group III) and patients with aortic root replacement alone (group IV), there were no significant differences in the actuarial freedom from reoperation (P = .177) and the linearized rate of reoperation (P = .900).

Reoperation for aortic arch in patients with arch dissection versus patients without arch dissection
As mentioned previously, the residual aortic arch was intact in groups I and II. Only 2 patients among the 53 group I and II patients who survived initial hospitalization underwent subsequent total arch replacement for the onset of aortic arch dissection. The actuarial freedoms from operation for the aortic arch were 97.4% ± 2.5% at 10 years and 90.5% ± 7.1% at 20 years (Figure 5). There was a significant difference (P = .0078) in the actuarial freedom from operation for the aortic arch between patients with a residual intact arch (groups I and II) and patients with arch dissection (group IV).



View larger version (27K):
[in this window]
[in a new window]
  		Figure 5. Actuarial freedom from reoperation for aortic arch in the patients with and without arch dissection, showing significant difference between groups (P = .0078).

 
Late survival and late outcome
There were 14 late deaths (Figure 2). In group I, 7 late deaths were due to intracerebral bleeding (n = 1), septicemia (n = 1), liver cancer (n = 1), renal failure (n = 1), tuberculosis (n = 1), and unknown causes (n = 2). In group II, 1 patient died at the second-stage operation of descending thoracic aorta replacement for type B dissection. In group III, 2 late deaths occurred as a result of bowel ischemia subsequent to malperfusion from residual dissection and an unknown cause. In group IV there were 4 late deaths. Two patients died of rupture of the residual dissection. One patient died at the second-stage operation for type B dissection. One patient died at the fourth-stage operation for graft replacement of the entire aorta. Overall actuarial survivals were 85.4% ± 3.9% at 5 years, 80.0% ± 4.7% at 10 years, 73.6% ± 6.2% at 15 years, and 60.6% ± 10.3% at 20 years. Actuarial survivals of individual groups are shown in Figure 6.



View larger version (23K):
[in this window]
[in a new window]
  		Figure 6. Actuarial survival in each group.

 
Late survival in patients with residual aortic dissection versus patients without residual aortic dissection
All the patients in groups II, III, and IV had residual aortic dissection after the initial operation. Among these patients, the actuarial survivals were 83.4% ± 6.3% at 5 years, 79.6% ± 7.0% at 10 years, 71.6% ± 9.9% at 15 years, and 57.3% ± 15.0% at 20 years (Figure 7). On the other hand, actuarial survivals in group I were 86.9% ± 5.0% at 5 years, 80.2% ± 6.5% at 10 years, 74.5% ± 8.2% at 15 years, and 67.0% ± 10.2% at 20 years (Figure 7). There was no significant difference between group I and the other groups combined (P = .7657).



View larger version (23K):
[in this window]
[in a new window]
  		Figure 7. Actuarial survival in patients with and without residual dissection, showing no significant difference (P = .7657) between groups.

 

   Discussion
Top
 Abstract
 Patients and methods
 Results
 Discussion
References
 
Aortic root dilatation, with subsequent aortic valve regurgitation, aortic dissection, or rupture, is a common and morbid cardiovascular abnormality in Marfan syndrome. The introduction of aortic root replacement with a composite graft by Bentall and De Bono11 in 1968 significantly improved the surgical results in such cases. In recent years, elective surgery has been performed with an operative risk below 5% to provide a full recovery and a normal life-style.5-7,12 In our series of operations on patients with Marfan syndrome, the mortality among patients with aortic root replacement between 1990 and 2002 was 4.2% (2/48), which was almost half (8.1%) that in earlier experiences between 1977 and 1989. Moreover, the life expectancy of patients with Marfan syndrome has significantly improved (actuarial survivals were 80% at 10 years and 61% at 20 years).

However, the abnormal aortic tissue in Marfan syndrome requires multiple surgical reconstructions, and the quality of life of patients with Marfan syndrome is significantly restricted by repeated operations. Of all 85 patients in this study, 28 (32.9%) required a total number of 47 subsequent staged operations. This was more common among patients with residual aortic dissection after the initial operation. In this study, the actuarial freedom from reoperation among patients with residual dissection (group II, III, IV) was significantly lower than that among patients without residual dissection (group I). The linearized rate of reoperation was also significantly higher. Unfortunately, this trend of reoperation was immutable even in patients who received concomitant total arch replacement for associated type A dissection. Among patients with aortic dissection in the aortic arch, there was no significant difference in the actuarial freedom from reoperation (P = .177) and the linearized rate of reoperation (P = .900) between patients with concomitant total arch replacement (group III) and patients with aortic root replacement alone (group IV). Indeed, more than 60% of patients with Marfan syndrome require a reoperation within 10 years, irrespective of whether the total aortic arch is replaced or not. These results showed that concomitant total arch replacement was therapeutic but not curative, because multiple reentries in the fragile dissected septum in Marfan syndrome disturbed the thrombosed closure of the residual false channel.13-15 On the basis of these results, controversy still continues regarding whether concomitant total arch replacement is necessary for a dissecting aortic arch.16,17 Concerning the second-stage operation, however, it is generally accepted that replacement of the descending thoracic aorta through left thoracotomy is preferable to total arch replacement through median resternotomy. Additionally, regarding the staged operation for entire aorta replacement, concomitant total arch replacement is more advantageous than aortic root replacement alone. Among our patients, 4 patients in group III had a complete aortic reconstruction with a total of 12 operations, whereas 3 patients in group IV had complete aortic reconstruction with a total of 12 operations (Figure 2). We therefore recommend total arch replacement for type A dissection involving the aortic arch simultaneously with aortic root replacement for annuloaortic ectasia as long as the patient's condition permits this.

Another point highlighted by this study is the late fate of the residual intact arch after aortic root replacement with abnormal tissue, that is, the potential risk of dissection or rupture in the future. This is a key to answering the primary question of whether the intact aortic arch should be replaced prophylactically and aggressively at the time of aortic root replacement for annuloaortic ectasia.

In general, the cause of aortic dissection is the process of injury and repair of the aortic media by the turbulence of blood flow.18 The initial intimal tear of a dissection is most frequently situated within the first few centimeters of the ascending aorta.19 In Marfan syndrome, aortic dissection sometimes occurs in a normal-sized ascending aorta, although the aortic root and sinus of Valsalva are enlarged.8 On the basis of our results, we propose the possible mechanism of a tear in ascending aorta among patients with Marfan syndrome as follows. Turbulence in the ascending aorta is exacerbated by both the velocity of ejected blood and the interface between the ejected blood and the relatively stagnant blood. However, in a normal aortic root, the position of the leaflets helps to reduce turbulence by masking the dilated sinuses and producing a uniform diameter above the ventriculoarterial junction when blood is ejected through the valve orifice.20-24 In contrast, in a gourdlike aortic root, which is typical of annuloaortic ectasia in Marfan syndrome, this mechanism is not effective. The high-velocity flow of ejected blood reaches the ascending aortic wall with medial degeneration, and turbulence occurs at the junction between the ascending aorta and the dilated sinus of Valsalva because the leaflets cannot mask the dilated sinus of Valsalva (Figure 8).25 Another factor in dissection is the different tensile strength of the aortic wall itself, which depends on the content of elastic fibers and collagen. The contents of elastic fibers and collagen differ between the ascending aorta and the sinus of Valsalva26-30 and also between the inner and outer layers of the aortic wall itself. This leads to the "breaking point" of the internal layer in the ascending aorta during aortic dilatation.21 Therefore these two major factors will be eliminated by composite graft replacement of the dilated sinus of Valsalva and the proximal ascending aorta. This hypothesis is supported by the long-term results of patients with an intact arch in our study. The incidence of new dissection in the residual intact arch after aortic root replacement was extremely low; only 2 of the 53 patients who survived initial hospitalization with an intact arch underwent subsequent total arch replacement for the onset of arch dissection, whereas 4 of the 14 patients who survived initial hospitalization with dissection in the aortic arch underwent subsequent total arch replacement. These results show that prophylactic replacement of the intact aortic arch is not necessary at the time of aortic root replacement for annuloaortic ectasia, because aortic root replacement itself plays a prophylactic role in aortic dissection.



View larger version (20K):
[in this window]
[in a new window]
  		Figure 8. Right, Normal aortic root. Position of leaflets helps to reduce turbulence by masking dilated sinus to produce uniform diameter above ventriculoarterial junction when blood is ejected through valve orifice. Left, Typical annuloaortic ectasia in Marfan syndrome. High-velocity flow of ejected blood reaches ascending aortic wall with medial degeneration, and turbulence occurs at junction between ascending aorta and dilated sinus of Valsalva (SoV) because leaflets cannot mask dilated sinus. Arrows indicate blood flow. Sinus of Valsalva has less elastic fiber than collagen; ascending aorta has more elastic fiber than collagen.

 
The major limitation of our study was that the time scale of this study ranged through 22 years. During this period mortality and morbidity in aortic surgery were clearly improved by refinements in surgical technique and perioperative management. Additionally, a large proportion of the patients required ongoing treatment and follow-up. Another limitation of this study was that the numbers of patients in each group were too small to ascertain any statistically significant difference between groups for operative mortality or the necessity for reintervention.

In conclusion, the incidence of new dissection in the residual intact arch after aortic root replacement is extremely low, because aortic root replacement may remove the factors provoking dissection. According to our results, prophylactic replacement of the intact arch in Marfan syndrome is not necessary during aortic root replacement for annuloaortic ectasia.

Discussion
Dr Nicholas Kouchoukos (St Louis, Mo). In their study, Tagusari and colleagues have focused on the question of whether the aortic arch should be replaced at the time of replacement of the dilated aortic root in patients with Marfan syndrome. Among the 57 patients with an intact arch at the time of aortic root replacement, only 2 required subsequent arch replacement for new-onset arch dissection. The actuarial freedom of reoperation in this group was 90% at 10 years. These observations are in agreement with the results of other large series of patients with Marfan syndrome and an intact arch who have undergone only aortic root replacement. The aggregate experience strongly suggests that replacement of the arch is not indicated in this setting.

What is less clear is whether the aortic arch should be replaced at the time of aortic root replacement in patients with acute or chronic type A dissection. Albeit the number of patients with dissection in this series was small, 29 patients, no difference in survival or in freedom from any operation was observed at 10 years between the 15 patients undergoing only aortic root replacement and the 13 patients who had aortic root and simultaneous arch replacement. However, the rate of reoperation on the aortic arch was significantly higher among the patients with dissected arch who underwent only aortic root replacement than among the patients with an intact arch. This suggests that replacement of the dissected arch may be advantageous.

Only 10 of the 46 patients with dissection in this series had acute dissection. Tagusari and colleagues did not examine outcomes in this subgroup, presumably because of small numbers. However, the decision to replace the aortic arch in the presence of acute dissection may be associated with higher risk than if the dissection is chronic.

I have several questions for Dr Tagusari. First, on the basis of your findings, what is your current strategy for management of patients undergoing aortic root replacement who have a type A dissection?

Dr Tagusari. We perform aortic root replacement with composite graft and total arch replacement.

Dr Kouchoukos. Do you manage the patients with acute dissection in this setting any differently than you manage the patients with chronic dissection?

Dr Tagusari. In general, the patient with Marfan syndrome who has an aortic dissection is young. Accordingly, we should perform total arch replacement simultaneously to save further operation.

Dr Kouchoukos. Do you recommend complete aortic arch replacement in the setting of acute dissection?

Dr Tagusari. Yes.

Dr Kouchoukos. A valve-sparing procedure was performed in 17 of the 86 patients in the series. How much of the ascending aorta was replaced in these patients? Do you believe, from your experience to date, that this is a durable procedure in patients with Marfan syndrome?

Dr Tagusari. For the patient with Marfan syndrome, I prefer a composite graft replacement to valve-sparing operation, especially a remodeling procedure, because in our histologic findings the aortic valve showed concentric layering of collagen fibers mixed with glycosaminoglycans. This means severe degeneration of the leaflet itself. Actually, 4 of the 17 patients who underwent valve-sparing operations (2 of 13 reimplantations and 2 of 4 remodelings) needed aortic valve replacement.


   References
Top
 Abstract
 Patients and methods
 Results
 Discussion
 References
 

  1. Marfan AB. Un cas de déformation congénitale des quatre membres plus prononcé aux extrémités caractérisée par l'allongement des os avec un certain degré d'amincissement. Bull Mem Soc Med Hop Paris. 1896;13:220–226
  2. Pyeritz RE, McKusick VA. The Marfan syndrome: diagnosis and management. N Engl J Med. 1979;300:772–777[Medline]
  3. Dietz HC, Pyeritz RE. Mutation in the human gene of fibrillin-1 (FBN1) in the Marfan syndrome and related disorders. Hum Mol Genet. 1995;4:1799–1809[Abstract]
  4. Loeys B, Nuytinck L, Delvaux I, De Bie S, De Paepe A. Genotype and phenotype analysis of 171 patients referred for molecular study of the fibrillin-1 gene FBN1 because of suspected Marfan syndrome. Arch Intern Med. 2001;161:2447–2454[Medline]
  5. Birks EJ, Webb C, Child A, Radley-Smith R, Yacoub MH. Early and long-term results of a valve-sparing operation for Marfan syndrome. Circulation. 1999;100(19 Suppl):II29–35
  6. Baumgartner WA, Cameron DE, Redmond JM, Greene PS, Gott VL. Operative management of Marfan syndrome: the Johns Hopkins experience. Ann Thorac Surg. 1999;67:1859–1860[Abstract/Free Full Text]
  7. Alexiou C, Langley SM, Charlesworth P, Haw MP, Livesey SA, Monro JL. Aortic root replacement in patients with Marfan's syndrome: the Southampton experience. Ann Thorac Surg. 2001;72:1502–1508[Abstract/Free Full Text]
  8. Svensson LG, Khitin L. Aortic cross-sectional area/height ratio timing of aortic surgery in asymptomatic patients with Marfan syndrome. J Thorac Cardiovasc Surg. 2002;123:360–361[Free Full Text]
  9. Beighton P, De Paepe A, Danks D, Finidori G, Gedde-Dahl T, Goodman R, et al. International nosology of heritable disorders of connective tissue. Am J Med Genet. 1986;29:581–594
  10. David TE. Current practice in Marfan's aortic root surgery: reconstruction with aortic valve preservation or replacement? What to do with the mitral valve? J Card Surg. 1997;12:147–150[Medline]
  11. Bentall H, De Bono A. A technique for complete replacement of the ascending aorta. Thorax. 1968;23:338–339[Abstract/Free Full Text]
  12. Gott VL, Cameron DE, Alejo DE, Greene PS, Shake JG, Caparrelli DJ, Dietz HC. Aortic root replacement in 271 Marfan patients: a 24-year experience. Ann Thorac Surg. 2002;73:438–443[Abstract/Free Full Text]
  13. Ando M, Takamoto S, Okita Y, Morota T, Matsukawa R, Kitamura S. Elephant trunk procedure for surgical treatment of aortic dissection. Ann Thorac Surg. 1998;66:82–87[Abstract/Free Full Text]
  14. Okita Y, Tagusari O, Minatoya K, Ando M, Kitamura S, Nakajima N, et al. Is distal anastomosis only to the true channel in chronic type B aortic dissection justified? Ann Thorac Surg. 1999;68:1586–1591[Abstract/Free Full Text]
  15. Ando M, Okita Y, Tagusari O, Morota T, Kitamura S. Three-channeled aortic dissection in Marfan syndrome. Cardiovasc Surg. 2000;8:153–158[Medline]
  16. Crawford ES, Kirklin JW, Naftel DC, Svensson LG, Coselli JS, Safi HJ. Surgery for acute dissection of ascending aorta. Should the arch be included? J Thorac Cardiovasc Surg. 1992;104:46–59[Abstract]
  17. Yun KL, Glower DD, Mille DC, Fann JI, Mitchell RS, White WD, et al. Aortic dissection resulting from tear of transverse arch: is concomitant arch repair warranted? J Thorac Cardiovasc Surg. 1991;102:355–370[Abstract]
  18. Schlatmann TJ, Becker AE. Histologic changes in the normal aging aorta: implications for dissecting aortic aneurysm. Am J Cardiol. 1977;39:13–20[Medline]
  19. Hirst AE Jr, Johnson VJ Jr, Kime SW. Dissecting aneurysm of the aorta: a review of 505 cases. Medicine. 1958;37:217–279[Medline]
  20. Maier GW, Wechsler AS. Pathophysiology of aortic valve disease. Edmunds LH Jr. Cardiac surgery in the adult. 1st ed. New York: McGraw-Hill; 1997. p. 835–855
  21. Robicsek F, Thubrikar M. The mechanism and prevention of aortic dissection in Marfan syndrome. Hetzer R, Gehle P, Ennker J. Cardiovascular aspects of Marfan syndrome. 1st ed. Berlin, Germany: Springer; 1995. p. 61–70
  22. Thubrikar M. Geometry of the aortic valve. Thubrikar M. The aortic valve. 1st ed. Boca Raton (FL): CRC Press; 1990. p. 1–20
  23. Peacock JA. An in vitro study of the onset of turbulence in the sinus of Valsalva. Circ Res. 1990;67:448–460[Abstract/Free Full Text]
  24. Vellhouse BJ. Velocity and pressure distributions in the aortic valve. J Fluid Mech. 1969;37:587–600
  25. Grande-Allen KJ, Cochran RP, Reinhall PG, Kunzelman KS. Mechanisms of aortic valve incompetence: finite-element modeling of Marfan syndrome. J Thorac Cardiovasc Surg. 2001;122:946–954[Abstract/Free Full Text]
  26. Perejda AJ, Abraham PA, Carnes WH, Coulson WF, Uitto J. Marfan's syndrome: structural, biochemical, and mechanical studies of the aortic media. J Lab Clin Med. 1985;06:376–383
  27. Hirata K, Triposkiadis F, Sparks E, Bowen J, Wooley CF, Boudoulas H. The Marfan syndrome: abnormal aortic elastic properties. J Am Coll Cardiol. 1991;18:57–63[Medline]
  28. Jeremy RW, Huang H, Hwa J, McCarron H, Hughes CF, Richards JG. Relation between age, arterial distensibility, and aortic dilatation in the Marfan syndrome. Am J Cardiol. 1994;74:369–373[Medline]
  29. Sutton JP, Ho SY, Anderson RH. The forgotten interleaflet triangles: a review of the surgical anatomy of the aortic valve. Ann Thorac Surg. 1995;59:419–427[Abstract/Free Full Text]
  30. Sheppard M. Diseases of the aorta. Sheppard M, Davies MJ. Practical cardiovascular pathology. 1st ed. New York: Oxford University Press; 1998. p. 172–190



This article has been cited by other articles:


Home page
 Interact CardioVasc Thorac SurgHome page
L. Heim, R. J. Poole, R. Warwick, and M. Poullis
The concept of aortic replacement based on computational fluid dynamic analysis: patient-directed aortic replacement
Interact CardioVasc Thorac Surg, May 1, 2013; 16(5): 583 - 588.
[Abstract] [Full Text] [PDF]

Home page
 Eur J Cardiothorac SurgHome page
F. S. Schoenhoff, A. Kadner, M. Czerny, S. Jungi, K. Meszaros, J. Schmidli, and T. Carrel
Should aortic arch replacement be performed during initial surgery for aortic root aneurysm in patients with Marfan syndrome?
Eur J Cardiothorac Surg, January 27, 2013; (2013) ezs705v1.
[Abstract] [Full Text] [PDF]

Home page
 J. Thorac. Cardiovasc. Surg.Home page
L. Sun, M. Li, J. Zhu, Y. Liu, Q. Chang, J. Zheng, and R. Qi
Surgery for patients with Marfan syndrome with type A dissection involving the aortic arch using total arch replacement combined with stented elephant trunk implantation: The acute versus the chronic
J. Thorac. Cardiovasc. Surg., September 1, 2011; 142(3): e85 - e91.
[Abstract] [Full Text] [PDF]

Home page
 Eur Heart JHome page
L. Mimoun, D. Detaint, D. Hamroun, F. Arnoult, G. Delorme, M. Gautier, O. Milleron, C. Meuleman, F. Raoux, C. Boileau, et al.
Dissection in Marfan syndrome: the importance of the descending aorta
Eur. Heart J., February 2, 2011; 32(4): 443 - 449.
[Abstract] [Full Text] [PDF]

Home page
 J. Thorac. Cardiovasc. Surg.Home page
L. Botta, V. Russo, C. La Palombara, M. Rosati, R. Di Bartolomeo, and R. Fattori
Stent graft repair of descending aortic dissection in patients with Marfan syndrome: An effective alternative to open reoperation?
J. Thorac. Cardiovasc. Surg., November 1, 2009; 138(5): 1108 - 1114.
[Abstract] [Full Text] [PDF]

Home page
 Ann. Thorac. Surg.Home page
E. Girdauskas, T. Kuntze, M. A. Borger, V. Falk, and F. W. Mohr
Distal Aortic Reinterventions After Root Surgery in Marfan Patients
Ann. Thorac. Surg., December 1, 2008; 86(6): 1815 - 1819.
[Abstract] [Full Text] [PDF]

Home page
 Ann. Thorac. Surg.Home page
L. Sun, R. Qi, Q. Chang, J. Zhu, Y. Liu, C. Yu, H. Zhang, B. Lv, J. Zheng, L. Tian, et al.
Surgery for Marfan Patients With Acute Type A Dissection Using a Stented Elephant Trunk Procedure
Ann. Thorac. Surg., December 1, 2008; 86(6): 1821 - 1825.
[Abstract] [Full Text] [PDF]

Home page
 Ann. Thorac. Surg.Home page
J. Bachet, F. Larrazet, B. Goudot, G. Dreyfus, T. Folliguet, F. Laborde, and D. Guilmet
When Should the Aortic Arch Be Replaced in Marfan Patients?
Ann. Thorac. Surg., February 1, 2007; 83(2): S774 - S779.
[Abstract] [Full Text] [PDF]

This Article
Right arrow Abstract Freely available
Right arrow Full Text (PDF)
Right arrow Alert me when this article is cited
Right arrow Alert me if a correction is posted
Right arrow Citation Map
Services
Right arrow Email this article to a friend
Right arrow Similar articles in this journal
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Add to Personal Folders
Right arrow Download to citation manager
Right arrow Author home page(s):
Osamu Tagusari
Hitoshi Ogino
Junjiro Kobayashi
Ko Bando
Kenji Minatoya
Kazuo Niwaya
Yutaka Okita
Motomi Ando
Soichiro Kitamura
Right arrow Permission Requests
Citing Articles
Right arrow Citing Articles via HighWire
Right arrow Citing Articles via Google Scholar
Google Scholar
Right arrow Articles by Tagusari, O.
Right arrow Articles by Kitamura, S.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by Tagusari, O.
Right arrow Articles by Kitamura, S.
Related Collections
Right arrow Great vessels

HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS
ANN THORAC SURG ASIAN CARDIOVASC THORAC ANN EUR J CARDIOTHORAC SURG
J THORAC CARDIOVASC SURG ICVTS ALL CTSNet JOURNALS