(Circulation. 1995;91:728-733.)
© 1995 American Heart Association, Inc.
Articles |
Marfan Syndrome
Long-term Survival and Complications After Aortic Aneurysm Repair
From the Department of Internal Medicine, University of Texas Medical School at Houston (R.F., D.M.); Department of Biomathematics, University of Texas, M.D. Anderson Cancer Center, Houston, Tex (D.J.); and Department of Surgery, Baylor College of Medicine, Houston, Tex (E.S.C., J.C.).
Correspondence to Dianna M. Milewicz, MD, PhD, University of Texas Health Science Center at Houston, 6431 Fannin, MSB 1.614, Houston, TX 77036.
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Abstract |
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Background Development of surgical therapy for aortic aneurysms and dissections has led to treatment of the life-threatening cardiovascular complications associated with Marfan syndrome. The present study determines the effect of surgical therapy on the life expectancy of patients with Marfan syndrome and the clinical course of these patients after aortic aneurysm repair.
Methods and Results Medical records were reviewed on 192 patients with Marfan syndrome who underwent aortic aneurysm repair during the past 26 years; 103 patients were interviewed, and complete preoperative and postoperative medical information was obtained. Survival curves were generated, and data were analyzed. The median cumulative probability of survival was 61 years, significantly increased compared with the median survival of 47 years for patients with Marfan syndrome determined 30 years ago (P<.0006). The majority of patients (53%) had second surgeries to repair subsequent aneurysms or dissections at other sites, the vast majority of which involved the aorta. The most common pattern of aneurysm repair was proximal ascending aortic aneurysm repair, followed by descending thoracic aneurysm surgery. The following variables predicted patients requiring second vascular surgeries: presence of acute or chronic dissection at the time of the first surgery, hypertension after the first surgery, and a history of smoking.
Conclusions The life expectancy of patients with Marfan syndrome undergoing surgical repair of aortic aneurysms has improved and is consistent with increased survival. After initial repair of an ascending aortic aneurysm, a significant number of patients have subsequent surgeries at other sites throughout the aorta, indicating Marfan syndrome is a disease involving the entire aorta. Patients who had a dissection at the time of the first aortic surgery were more likely to require subsequent aortic surgery than were patients who underwent prophylactic composite graft repair of an aortic aneurysm.
Key Words: Marfan syndrome • aneurysms • morbidity • mortality • surgery
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Introduction |
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TopAbstractIntroductionMethods Results Discussion References |
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Marfan syndrome is an autosomal dominant disorder of connective tissue characterized by abnormalities involving the skeletal, ocular, and cardiovascular systems.1 2 The cardiovascular features typically include progressive dilatation of the proximal aorta leading to aortic dissection or rupture. In addition, the dilatation of the aortic ring can cause aortic valve incompetence and regurgitation. Abnormalities of the mitral valve often lead to mitral valve regurgitation or prolapse. The cardiovascular complications of the syndrome lead to a reduced life expectancy for affected individuals if left untreated.3
The advent of cardiovascular surgery has led to treatment of the life-threatening complications associated with Marfan syndrome. In 1968, the composite valve graft technique of replacing the aortic valve and the tubular and sinus segments of the ascending aorta was introduced to repair proximal aortic aneurysms, with coronary artery ostial reattachment made to openings in the graft.4 Initially, the procedure involved wrapping the aneurysmal wall around the site of repair, but this technique was abandoned in the late 1970s after it became apparent that there was an increase in complications associated with the wrap. A technique was introduced to reattach the coronary ostia with Dacron grafts, thereby reducing the tension on the arteries. We and others5 6 7 8 have shown that composite graft repair of Marfan aneurysms of the ascending aorta can be performed with low hospital mortality rates and good 5- and 10-year survival rates.
Although the perioperative complications and causes of death of patients with Marfan syndrome after aortic aneurysm repair have been studied,6 7 8 9 there are no investigations of the subsequent clinical course and complications in these patients. In the present study, we obtained medical information on 192 patients with Marfan syndrome who underwent graft repair at the Methodist Hospital during the past 24 years. We have generated life survival curves of this patient population and compared these with survival curves of affected individuals who died before the availability of surgical aortic aneurysm repair. This report details the clinical course of these patients after aortic aneurysm repair, describes the pattern of subsequent aneurysm formation, and identifies the clinical variables that place patients at risk for further vascular problems. In addition, the evolution of Marfan phenotype with advancing age is assessed. The results of the present study provide important information for the subsequent care and surveillance of this patient population.
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Methods |
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Study Population
Two hundred and twenty-eight patients with Marfan syndrome underwent aortic aneurysm surgery performed by two of the authors (E.S.C. and J.C.) between 1966 and 1992. This population included 41 patients reported by Crawford,5 110 patients reported by Svensson and colleagues6 who underwent surgery between July 1, 1966, and June 20, 1988, and 76 patients operated on between June 21, 1988, and October 1, 1992. Thirty-six of these patients were excluded from the study because they did not meet the established diagnostic criteria for Marfan syndrome.10 Patients with one major criterion for Marfan syndrome (proximal aortic aneurysm or aortic dissection) required a positive family history and involvement of one other organ system to meet the established criteria. Patients without a family history of Marfan syndrome required involvement of two other systems for inclusion in the study. The study included 143 living patients (61 women and 82 men) and 49 deceased patients (17 women and 32 men).
Medical records from Methodist Hospital were reviewed for all patients. Detailed preoperative and postoperative medical information was obtained by telephone interviews of 103 of the 143 living patients (48 woman and 55 men). The remaining 40 patients either declined to be interviewed or lived in a foreign country. Medical records were obtained to confirm any reports of additional cardiovascular complications not previously documented in the patient's hospital records. The cause of death was obtained from the medical records of all deceased patients. Information concerning dissection of the aorta was obtained from preoperative and postoperative aortic imaging studies or surgical pathology reports.
Surgical Technique
Surgical techniques used to repair aortic
aneurysms before June
1988 were detailed in a previous report.6 All patients
undergoing surgical treatment subsequent to 1988 for aortic valvular
insufficiency or aortic root aneurysm underwent composite valve graft
replacement. Valve graft repair has been accomplished recently without
wrapping the graft with the aortic wall but rather by mobilizing
buttons of aortic tissue around the coronary arteries for direct
nontension anastomosis to the aortic graft.11 Patients
with massive aneurysm and/or repeat operation, in whom mobilization of
the coronary arteries was not feasible, had reestablishment of
circulation obtained with a transversely placed smaller Dacron tube
graft.12 All patients with acute type I aortic dissection
were treated in conjunction with profound hypothermia and circulatory
arrest. The criterion for surgical repair of distal aortic dissection
and/or aneurysmal dilatation was an overall diameter of 5.5 cm as
determined by computed tomography or magnetic resonance imaging
scanning. Abdominal aortic aneurysms of 5 cm were considered
significant and subjected to elective aneurysm replacement.
Postoperative Management
Routine imaging of the aorta,
computed tomography, or magnetic
resonance imaging was carried out 6 months after surgery and was
recommended annually thereafter for all patients. Patients who had
distal aortic surgery underwent annual evaluation of the aortic root,
aortic valve, and mitral valve by transthoracic echocardiography with
color flow Doppler. All patients with prosthetic valves were maintained
on a regimen of oral warfarin sodium. Patients with satisfactory
tolerance were maintained on ß-adrenergic–blocking agents.
Statistical Analysis
The data from medical records and
interviews were entered into a
file and processed using SPSS FOR WINDOWS (SPSS). Life
tables were constructed using the last known live age and the age at
death. Life tables were constructed for three separated groups: the
study population, deceased affected relatives of the study population,
and patients reported by Murdoch et al,3 the original life
expectancy study of patients with Marfan syndrome done before the
advent of surgical aortic aneurysm repair. The life tables were
analyzed using SURVIVAL from SPSS FOR
WINDOWS, Release 5,13 using the Kaplan-Meier
method14 ; group comparisons were performed using the Lee
and Desu generalization15 of the Wilcoxon (Gehan)
statistic.16
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Results |
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TopAbstractIntroductionMethods Results Discussion References |
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Survival Analysis
The life tables generated on patients with Marfan syndrome undergoing aortic aneurysm repair were used to construct the cumulative probability of survival curves (Figure
). Data on all 192
patients with Marfan syndrome were incorporated into the life tables,
including patients who died during surgery. The median expected
survival time for patients was 61 years, with no significant difference
between men and women.
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The survival of the study population was compared with that of affected relatives who did not undergo aortic aneurysm repair because they died before diagnosis or the availability of surgical aneurysm repair. The median expected survival time for the 45 affected relatives was 30.4 years. Comparison of survival between patients who underwent surgical aortic aneurysm repair and the surgically untreated affected relatives showed that the prolonged survival by aortic aneurysm repair was highly significant (P<.00001).
Survival of the study population was compared with that of the population with Marfan syndrome used by Murdoch et al3 to generate the original life survival curves for individuals with Marfan syndrome before the advent of surgical aneurysm repair. The median expected survival for the Murdoch population was 46.6 years. A comparison of the survival between our study population undergoing aortic aneurysm repair and the Murdoch population showed a highly significant prolongation of survival in the patients with Marfan syndrome undergoing cardiovascular surgery (P<.0006).
Cardiovascular Complications After Aortic Aneurysm Repair
The
ascending aorta was the site of the first operation in 83.8%
of the patients (161 of 192). Thirteen of these patients had the
transverse aortic arch repaired with the same procedure. The descending
aorta was the site of the initial operation in the remaining 31
patients: descending thoracic aorta in 8, thoracoabdominal aorta in 18,
and abdominal aorta in 5. The mean age at first surgery was 32.4
years.
Data collected from subsequent operations, imaging studies, and autopsies indicated that the majority of patients had subsequent aneurysms or dissections involving other sites of their vascular system (135 of 192 patients, or 70.3%). The vast majority of these events involved other regions of the aorta (95%), but a small number involved the arteries directly arising from the aorta, including the iliac, carotid, renal, subclavian, and innominate arteries (5%).
The majority
of patients had second surgeries to repair subsequent
aneurysms or dissections at second sites (101 of 192, or 52.6%). Of
the 161 patients who had initial repair of the ascending aorta, 81
patients had second vascular surgeries elsewhere (Table 1). In
addition, 29 of these 81 patients had third
vascular procedures at other sites, 9 patients had fourth surgeries,
and 2 patients had fifth surgeries. Including all subsequent vascular
procedures, 73 of the 161 patients who initially had ascending aortic
aneurysm repair had portions of their descending thoracic aortas
repaired. Thirty patients had their transverse aortic arch repaired
with their initial repair or in subsequent procedures.
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Of the 31 patients that had initial surgery at sites other than the ascending aorta, 20 had second vascular procedures at other sites, 6 patients had third surgeries, and 3 had fourth surgeries. Ten had ascending aortic aneurysm repair. In addition, 2 patients died of dissection of the ascending aorta before surgical repair, and 5 patients have ascending aortic aneurysms that have not been repaired. Ten patients had subsequent aneurysm repair involving other regions of the descending aorta. Six patients had only isolated thoracoabdominal aneurysm repair.
In addition to subsequent surgeries at new sites, 48 patients had repeat operations at sites of previous repair. Thirty-eight patients had second operations of the ascending aorta, and 10 had repeat operations involving regions of their descending aorta. Taking into account all surgeries on the vascular system, the 192 patients had a total of 170 operations involving their vascular system, 165 of which involved the aorta.
Five clinical variables were assessed to determine
if clinical markers
could be useful in predicting the need for subsequent vascular surgery
at other sites, excluding repeat operations at the original site of
surgery (Table 2). The variables studied included the
presence of dissection (either chronic or acute) at the time of the
first operation, hypertension or the use of ß-adrenergic blockers
subsequent to the first operation, smoking, and sex. In addition, the
patient's age at the time of the first surgery and the length of time
between the first and second surgeries were compared in these
groups.
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The presence of dissection, either acute or chronic, at the time of the first operation was a significant predictor of subsequent aneurysm repeat operation. The majority of patients with dissection present at the time of the first surgery underwent subsequent surgeries at other sites. The age at first surgery and the time between surgeries did not differ significantly between these groups. Information on the presence or absence of dissection of the aorta at the time of the initial surgery was not available for 19 patients.
The presence of hypertension between the first and second surgery also predicted the need for vascular surgery at another site. Last, patients who smoked at any time during their life were more likely to undergo second aortic surgeries. The other clinical variables studied—ß-adrenergic receptor antagonist use between the first and second surgeries and sex—did not differ significantly between the individuals requiring repeat operation and those who did not. The age at first surgery and the time between first and second surgeries did not differ significantly with any of these variables.
The cause of
death was known for 46 of the 49 deceased patients.
Eighteen died of complications related to surgery, and the causes of
these deaths were detailed in part by Svensson et al.6
Cardiovascular causes accounted for 25 of the 31 late deaths (Table
3). Aneurysm rupture, perioperative death during
subsequent aneurysm operation, and congestive heart failure accounted
for most of the negative outcomes (19 of 31, or 61%).
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Twenty-one of the 192 patients with Marfan syndrome (10.9%) had their mitral valve repaired sometime during their lives. Other cardiovascular complications reported by the patients included endocarditis in 10 patients, congestive heart failure in 8 patients, and cerebrovascular accidents in 13 patients.
Other Complications Related to the Marfan Syndrome
The
percentage of patients demonstrating known features of Marfan
syndrome in our study population was compared with results from
previous published studies to determine if the extended life span
increased the expression of these manifestations (Table 4). The
prevalence of musculoskeletal features of the
disorder (pectus deformity and scoliosis/kyphoscoliosis), dural
ectasia, retinal detachment, and the incidence of spontaneous
pneumothorax did not differ significantly from previous studies. In
contrast, striae and inguinal hernia were more prevalent in our study
population than in previously reported populations with Marfan
syndrome. Ocular problems also differed from the published rate, with
myopia being more common and lens dislocation less common. Sixty
percent of the patients had a family history of Marfan syndrome.
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A complete review of systems was obtained and medical records were reviewed to identify other potential pleiotropic manifestations of Marfan syndrome related to aging. Twenty-two percent of patients had been diagnosed with arthritis by a physician, with the mean age of 33 years at diagnosis. Twenty-three percent of patients had varicose veins not associated with pregnancy (19 of 47 women and 14 of 51 men). Fourteen percent of patients had experienced a ruptured or herniated disc. Ten percent of the women had prolapse of the uterus, and 10% of women had prolapse of the bladder. Twenty-eight patients (15%) had gallstones. Twenty-two of the patients (11%) had a deviated nasal septum.
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Discussion |
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TopAbstractIntroductionMethods Results Discussion References |
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In the present study, we document the survival outcome and subsequent clinical course of patients with Marfan syndrome after aortic aneurysm repair. The primary life-threatening complication of Marfan syndrome is rupture of an aortic aneurysm. In a study done in the early 1970s, before surgical therapy had a beneficial impact on survival, Murdoch and colleagues3 showed a decreased life expectancy for patients with Marfan syndrome. Cardiovascular complications were the cause of death in >90% of the cases, with aortic rupture accounting for 80% of the deaths. The development of surgical therapies to repair aortic aneurysms would be predicted to prolong the life expectancy of patients with Marfan syndrome by preventing sudden death due to aortic rupture. Previous studies have demonstrated that surgical repair of aortic aneurysms in patients with Marfan syndrome can be done with minimal early and late mortality.5 6 7 Svensson et al6 showed a 30-day survival rate of 94% and 5- and 10-year survival rates after surgery of 75% and 56%, respectively. The life survival analysis presented here is the first to show that surgical therapy of aortic aneurysms prolongs the life expectancy of patients with Marfan syndrome an additional 30 years compared with relatives who died without surgical intervention. Compared with the population with Marfan syndrome studied by Murdoch et al, life expectancy is prolonged by 14 years.
The survival curve of the Murdoch population differs significantly from the survival curve of the unoperated affected relatives despite the fact that these groups should both reflect Marfan populations who did not undergo surgical aneurysm repair. One explanation is that Marfan population in the present study is more severely affected than the clinic population studied by Murdoch et al. An alternative explanation is that the affected relative survival curve is skewed toward death because more mildly affected individuals in these families are not included in these data.
Patients with Marfan syndrome are surviving the initial aortic injury through surgical intervention and living to an older age. The present study shows that the majority of the patients have subsequent vascular complications at other sites, the vast number of which involve the aorta. Although the literature contains case reports of aneurysms at other locations than the proximal aorta in patients with Marfan syndrome,17 18 19 20 21 our study shows that vascular complications can occur anywhere in the aorta or arteries stemming from the aorta (innominate, subclavian, renal, iliac). This complication of Marfan syndrome most likely reflects the longer life expectancy resulting in the expression of more complicated and extensive vascular disease.
The pattern of surgical repair in this patient population may be a useful predictor of subsequent aneurysm formation in patients with Marfan syndrome. The majority of patients with Marfan syndrome have ascending aortic aneurysm formation as their initial vascular event with subsequent vascular problems involving primarily the descending thoracic aorta. The small group of patients with Marfan syndrome who presented with descending aortic disease subsequently had primarily ascending aortic complications.
Fifteen percent of patients had initial surgery involving portions of the descending aorta. Although the literature contains case reports of patients with Marfan syndrome presenting with aneurysms at other locations than the proximal aorta,17 18 21 the data we collected suggest that presenting with descending aortic dissections or aneurysms are not unusual for patients with Marfan syndrome. Our data also show that aneurysms and dissections can involve many of the arteries stemming from the aorta, including the innominate, subclavian, renal, and iliac. Unusual locations for aneurysms previously reported in patients with Marfan syndrome, including the internal carotid artery,22 berry aneurysms,23 and pulmonary artery aneurysms,24 were not observed in this patient population. There were no vascular events involving peripheral arteries.
The most significant predictor of subsequent vascular complications is aortic dissection, either acute or chronic, present at the time of the initial aneurysm repair. There were insufficient data to correlate the need for second operations with the location or extent of the initial dissection. Currently, it is recommended that patients with Marfan syndrome undergo prophylactic aortic grafting when the aortic diameter exceeds 5.5 cm because the probability of aortic dissection or rupture increases with greater diameters.25 Our data suggest that prophylactic surgery not only decreases the risk of sudden death from aortic rupture but also may decrease subsequent vascular complications. However, the individuals who have aortic dissection before the corrective surgery may be more prone to subsequent dissection due to more severe underlying pathology. Further studies are needed to discriminate between these possibilities.
In a follow-up study of 527 patients with and without Marfan syndrome who underwent aortic repair for dissections, DeBakey et al26 found that the incidence of subsequent aneurysms was dependent on the presence of uncontrolled hypertension. Second aneurysms developed in 46% of patients with uncontrolled hypertension compared with 17% of the patients with controlled hypertension. Despite the fact that the majority of patients in the present study had controlled hypertension, they were still at a significantly higher risk for subsequent vascular problems requiring surgery.
A history of smoking does predict that a patient with Marfan syndrome will require a second vascular operation. Smoking is a known risk factor for the development of aneurysms. Auerbach and Garfinkel27 studied 1412 aortas at autopsy from men and found that aneurysms were eight times more frequent in individuals who smoked one to two packs of cigarettes per day than in nonsmokers. Patients with Marfan syndrome increase their risk for requiring a second aortic procedure if they smoke, therefore implying that smoking poses additional damage to the integrity of the aorta.
The defective gene causing Marfan syndrome is the FBN1 gene encoding for fibrillin, a large glycoprotein.28 29 30 31 Fibrillin is found in microfibrils, a fiber system closely associated with elastin in the aorta.32 33 The elastic fiber system is found throughout the aorta but is less prominent in the abdominal aorta than in the thoracic aorta.34 Despite this fact, aortic pathology in these patients extends throughout the aorta.
The frequency of many features of Marfan syndrome did not differ from previous reports. The lower frequency of lens dislocation most likely reflects underreporting of this complication by the patients. The majority of patients had myopia, probably a more accurate reflection of this complication. The incidence of striae and inguinal hernias is higher than previously reported for patients with Marfan syndrome and may represent further expression of these complications as the population ages.
A complete review of all medical problems may have revealed unidentified pleiotropic manifestations of Marfan syndrome, possibly related to aging of this population. These medical problems included the onset of arthritis at a young age, varicose veins, and prolapse of the uterus or bladder in women. Although not related to aging, 10% of patients had a deviated nasal septum, indicating that this is an unrecognized pleiotropic manifestation of Marfan syndrome. The number of individuals with gallstones is not unexpected because of the high prevalence of gallstones after major surgery.35
The surgeons caring for these patients receive referrals from all over
the country for complex aortic procedures, raising the possibility that
this population may reflect a subpopulation of patients with Marfan
syndrome with more severe vascular problems. Although we cannot exclude
this possibility, many clinical variables indicate that other
complications of Marfan syndrome (ie, pulmonary, skeletal,
neurological) do not differ from Marfan populations previously reported
(Table 4
). Patterns of aneurysm formation and predictors for
subsequent
vascular events should be valid even if this is a more severely
affected population.
In summary, the present study demonstrates an improved life span for patients with Marfan syndrome with the advent of surgical repair of aortic aneurysms and defines the clinical progression of Marfan syndrome as affected individuals grow older. The present study emphasizes that Marfan syndrome is not just a disease of the proximal aorta but rather affects the entire aorta, stressing the need to continue to image the aorta routinely in patients with Marfan syndrome throughout their lifetime.
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Acknowledgments |
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This work has been supported in part by a Pfizer Scholars Award to Dr Milewicz. We are indebted to Drs Heinrich Taegtmeyer, Jacqueline Hecht, Lesley Ades, and Hope Northrup for their critical comments on the study protocol and data interpretation; to Nancy Luzak and Phyllis Atcherson for assistance in the preparation of the manuscript; and to the patients with Marfan syndrome without whose cooperation the work could not have been done. We are also indebted to Dr J. Lamont Murdoch for supplying the original data from "Life Expectancy and Causes of Death in Marfan Syndrome" (N Engl J Med. 1972;286:804-808.).
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Footnotes |
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Guest editor for this article was J. David Bristow, MD, Oregon Health Sciences University, Portland.
Received September 13, 1994; accepted November 13, 1994.
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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]
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S. A. LeMaire, S. A. Carter, I. V. Volguina, A. T. Laux, D. M. Milewicz, G. W. Borsato, C. K. Cheung, J. Bozinovski, J. M. Markesino, W. K. Vaughn, et al. Spectrum of Aortic Operations in 300 Patients With Confirmed or Suspected Marfan Syndrome Ann. Thorac. Surg., June 1, 2006; 81(6): 2063 - 2078. [Abstract] [Full Text] [PDF]
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D. Baumgartner, C. Baumgartner, G. Matyas, B. Steinmann, J. Loffler-Ragg, E. Schermer, U. Schweigmann, I. Baldissera, B. Frischhut, J. Hess, et al. Diagnostic power of aortic elastic properties in young patients with Marfan syndrome J. Thorac. Cardiovasc. Surg., April 1, 2005; 129(4): 730 - 739. [Abstract] [Full Text] [PDF]
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D. M. Milewicz, H. C. Dietz, and D. C. Miller Treatment of Aortic Disease in Patients With Marfan Syndrome Circulation, March 22, 2005; 111(11): e150 - e157. [Full Text] [PDF]
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V. Anttila, M. Piaszczynski, B. Mora, I. Hagino, R. V. Lacro, D. Zurakowski, and R. A. Jonas Improved outcome with composite graft versus homograft root replacement for children with aortic root aneurysms Eur. J. Cardiothorac. Surg., March 1, 2005; 27(3): 420 - 424. [Abstract] [Full Text] [PDF]
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T. Carrel, L. Beyeler, A. Schnyder, P. Zurmuhle, P. Berdat, J. Schmidli, and F. S. Eckstein Reoperations and late adverse outcome in Marfan patients following cardiovascular surgery Eur. J. Cardiothorac. Surg., May 1, 2004; 25(5): 671 - 675. [Abstract] [Full Text] [PDF]
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H.-Y. Yu, Y.-S. Chen, S.-C. Huang, S.-S. Wang, and F.-Y. Lin Late outcome of patients with aortic dissection: study of a national database Eur. J. Cardiothorac. Surg., May 1, 2004; 25(5): 683 - 690. [Abstract] [Full Text] [PDF]
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T. Kazui, K. Yamashita, H. Terada, N. Washiyama, T. Suzuki, K. Ohkura, and K. Suzuki Late reoperation for proximal aortic and arch complications after previous composite graft replacement in marfan patients Ann. Thorac. Surg., October 1, 2003; 76(4): 1203 - 1207. [Abstract] [Full Text] [PDF]
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C. A. Nienaber and K. A. Eagle Aortic Dissection: New Frontiers in Diagnosis and Management: Part II: Therapeutic Management and Follow-Up Circulation, August 12, 2003; 108(6): 772 - 778. [Full Text] [PDF]
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D. Pacini, F. Ranocchi, E. Angeli, F. Settepani, M. Pagliaro, S. Martin-Suarez, R. Di Bartolomeo, and A. Pierangeli Aortic root replacement with composite valve graft Ann. Thorac. Surg., July 1, 2003; 76(1): 90 - 98. [Abstract] [Full Text] [PDF]
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H. Nakajima, K. Bando, S. Kitamura, J. Kobayashi, K. Niwaya, and O. Tagusari Maze procedure in the Marfan syndrome J. Thorac. Cardiovasc. Surg., June 1, 2003; 125(6): 1539 - 1540. [Full Text] [PDF]
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J. C S Dean Management of Marfan syndrome Heart, July 1, 2002; 88(1): 97 - 103. [Full Text] [PDF]
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C. Alexiou, S. M. Langley, P. Charlesworth, M. P. Haw, S. A. Livesey, and J. L. Monro Aortic root replacement in patients with Marfan's syndrome: the Southampton experience Ann. Thorac. Surg., November 1, 2001; 72(5): 1502 - 1507. [Abstract] [Full Text] [PDF]
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A. Ingu, M. Ando, Y. Okita, N. Yamada, and S. Kitamura Redo operation for thoracoaortic aneurysm after entire aortic replacement Ann. Thorac. Surg., November 1, 2001; 72(5): 1766 - 1767. [Abstract] [Full Text] [PDF]
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C. Detter, H. Mair, H.-G. Klein, C. Georgescu, A. Welz, and B. Reichart Long-term prognosis of surgically-treated aortic aneurysms and dissections in patients with and without Marfan syndrome Eur. J. Cardiothorac. Surg., April 1, 1999; 13(4): 416 - 423. [Abstract] [Full Text] [PDF]
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C. L. Stowe, M. A. Baertlein, M. D. Wierman, M. Rucker, and G. Ebra Surgical management of ascending and aortic arch disease: refined techniques with improved results Ann. Thorac. Surg., August 1, 1998; 66(2): 388 - 395. [Abstract] [Full Text] [PDF]
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A. M. Gillinov, K. J. Zehr, J. M. Redmond, V. L. Gott, H. C. Deitz, B. A. Reitz, J. C. Laschinger, and D. E. Cameron Cardiac Operations in Children With Marfan's Syndrome: Indications and Results Ann. Thorac. Surg., October 1, 1997; 64(4): 1140 - 1144. [Abstract] [Full Text]
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D. M. Milewicz, K. Michael, N. Fisher, J. S. Coselli, T. Markello, and A. Biddinger Fibrillin-1 (FBN1) Mutations in Patients With Thoracic Aortic Aneurysms Circulation, December 1, 1996; 94(11): 2708 - 2711. [Abstract] [Full Text]
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L. H. Cohn, R. J. Rizzo, D. H. Adams, S. F. Aranki, G. S. Couper, N. Beckel, and J. J. Collins Jr Reduced Mortality and Morbidity for Ascending Aortic Aneurysm Resection Regardless of Cause Ann. Thorac. Surg., August 1, 1996; 62(2): 463 - 468. [Abstract] [Full Text]
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