Replacement of the Aortic Root in Patients with Marfan's Syndrome
Vincent L. Gott, M.D., Peter S. Greene, M.D., Diane E. Alejo, B.A., Duke E. Cameron, M.D., David C. Naftel, Ph.D., D. Craig Miller, M.D., A. Marc Gillinov, M.D., John C. Laschinger, M.D., and Reed E. Pyeritz, M.D., Ph.D.
Background Replacement of the aortic root with a prostheticgraft and valve in patients with Marfan's syndrome may preventpremature death from rupture of an aneurysm or aortic dissection.We reviewed the results of this surgical procedure at 10 experiencedsurgical centers.
Methods A total of 675 patients with Marfan's syndrome underwentreplacement of the aortic root. Survival and morbidity-freesurvival curves were calculated, and risk factors were determinedfrom a multivariable regression analysis.
Results The 30-day mortality rate was 1.5 percent among the455 patients who underwent elective repair, 2.6 percent amongthe 117 patients who underwent urgent repair (within 7 daysafter a surgical consultation), and 11.7 percent among the 103patients who underwent emergency repair (within 24 hours aftera surgical consultation). Of the 675 patients, 202 (30 percent)had aortic dissection involving the ascending aorta. Forty-sixpercent of the 158 adult patients with aortic dissection anda documented aortic diameter had an aneurysm with a diameterof 6.5 cm or less. There were 114 late deaths (more than 30days after surgery); dissection or rupture of the residual aorta(22 patients) and arrhythmia (21 patients) were the principalcauses of late death. The risk of death was greatest withinthe first 60 days after surgery, then rapidly decreased to aconstant level by the end of the first year.
Conclusions Elective aortic-root replacement has a low operativemortality. In contrast, emergency repair, usually for acuteaortic dissection, is associated with a much higher early mortality.Because nearly half the adult patients with aortic dissectionhad an aortic-root diameter of 6.5 cm or less at the time ofoperation, it may be prudent to undertake prophylactic repairof aortic aneurysms in patients with Marfan's syndrome whenthe diameter of the aorta is well below that size.
In 1896, A.B. Marfan described a five-year-old girl with longthin legs that he characterized as "spider-like."1 Over thenext 50 years, many other features of Marfan's syndrome weredescribed, including dislocated lenses in 1914, autosomal dominantinheritance in 1931, and aneurysm of the ascending aorta byTaussig and associates in 1943.2 In a landmark publication in1955,3 McKusick provided the comprehensive picture of Marfan'ssyndrome.
Before the era of open-heart surgery, the majority of patientswith Marfan's syndrome died prematurely of rupture of the aorta,often by the third decade of life.4 Even after open-heart surgerybecame established, it was usually reserved for patients withacute aortic dissection or rupture, and results were poor. In1968, Bentall and De Bono described a composite graft–valveprocedure in which a prosthetic valve was sewn onto the lowerend of a polytetrafluoroethylene (Teflon) tubular graft, whichin turn was anastomosed to the aortic annulus; the coronaryarteries were then anastomosed to the side of the graft.5 Thisrepair completely removes the aortic segment most prone to dissectionand rupture.
Nearly 30 years of experience has accumulated since the introductionof this procedure. Therefore, we evaluated the results fromseveral centers with expertise in the performance of aorticsurgery for patients with Marfan's syndrome.
Methods
Patients
A total of 675 patients with Marfan's syndrome underwent aortic-rootreplacement at 10 surgical centers (7 in North America and 3in Europe) between October 1968 and March 1996. To be eligiblefor entry into the study, patients had to have undergone aortic-rootreplacement and to have been given a diagnosis of classic Marfan'ssyndrome; patients with a forme fruste of the syndrome or relatedconnective-tissue disorders were not included. Nine surgicalcenters submitted information on all patients with Marfan'ssyndrome who had had aortic-root replacement performed by anysurgeon at the centers; one center submitted information onlyon patients operated on by one surgeon since February 1991,but these patients constituted 95 percent of the patients withMarfan's syndrome who had undergone aortic-root replacementat that institution since 1991.
Follow-up ended on April 1, 1997, and current follow-up datawere available for 611 of the 675 patients (91 percent). Themean length of follow-up was 6.7 years (range, 0 to 23). Forthe 64 patients whose whereabouts were unknown at the end offollow-up, the mean length of follow-up was 4.2 years, and dataon these patients were censored at the time of the last follow-upvisit. Thirty-two of the patients who could not be located hadreturned home to another country after surgery.
A total of 604 patients underwent composite-graft replacementaccording to the method of Bentall and De Bono or a modificationof that technique. Twenty-one patients (13 adults and 8 children)underwent placement of a homograft aortic root as the primaryprocedure, usually to avoid the use of anticoagulation mandatedby mechanical prostheses. Fifty patients underwent a valve-sparingprocedure; 15 of these patients had aortic dissection (12 acuteand 3 chronic) at the time of surgery. Of the 604 patients whounderwent a composite-graft procedure, 209 had side-to-sideanastomoses of the coronary ostia to the Dacron graft, 149 haddirect anastomoses of the coronary artery to the graft, and34 had a prosthetic interposition graft placed between the coronaryostia and the graft. In the remaining 212 patients, the operativenote did not indicate the type of coronary anastomosis used.Further description of these coronary anastomotic techniquesmay be found in the surgical literature.6
Statistical Analysis
Survival and event-free survival curves were calculated accordingto the Kaplan–Meier method. Risk factors for mortalitywere determined by a parametric multivariable analysis of thepostoperative risk. Clinically important rates are presented,with 95 percent confidence intervals. Computations were madewith the use of SAS statistical software that included a programto estimate risks that varied according to time.7
Results
Preoperative Characteristics of the Patients
Of the 675 patients with Marfan's syndrome, 473 (70 percent)were male. The mean age of the patients was 34 years (range,4 to 73). Two hundred two (30 percent) had a dissection involvingthe ascending aorta; 99 of the dissections were acute (operationperformed within 14 days after the event), whereas 103 werechronic (operation performed more than 14 days after the event).The diameter of the ascending aorta was documented in 524 adultpatients; the mean diameter in this group was 6.8 cm (range,3 to 15). Of these 524 patients, 158 had an aortic dissectionwith a mean diameter of 7.2 cm. In 73 of these patients withdissection (46 percent), the aortic diameter was 6.5 cm or less.Figure 1 shows the aortic-root diameters in the presence andabsence of dissection in the 524 adult patients. Five patientshad previously undergone aortic-valve surgery, 33 had previouslyundergone surgery of the ascending aorta, and 37 had previouslyundergone both types of surgery.
Figure 1. Diameter of the Aneurysm in 524 Adult Patients with Marfan's Syndrome, According to the Presence of Aortic Dissection.
The diameter of the aneurysm was documented for 524 patients.
Operative Results
The 30-day mortality rates are shown in Table 1 according tothe urgency of the operation. The 30-day mortality rate amongthe 455 patients who underwent elective repair was 1.5 percent(7 patients). The rate was 2.6 percent among the 117 patientswho underwent urgent repair — that is, within seven daysafter a surgical consultation. The rate was 11.7 percent amongthe 103 patients who underwent emergency repair — thatis, within 24 hours after a surgical consultation.
Table 1. Results of Aortic-Root Replacement in 675 Patients with Marfan's Syndrome, According to the Urgency of the Procedure.
Eighty-two of the 103 patients who underwent emergency surgeryhad aortic dissection; 73 of the dissections were acute. Theremaining 21 patients underwent emergency surgery because oftwo or more of the following factors: chest pain, an aneurysmwhose diameter exceeded 7.0 cm, or New York Heart Associationclass III or IV congestive heart failure. The 117 patients whounderwent urgent repair had one or more of the following factors:aortic dissection, chest pain, an aneurysm that was larger than7.0 cm, or New York Heart Association class III or IV congestiveheart failure.
Overall, 22 of the 675 patients (3.3 percent) died within 30days after surgery. When analyzed according to the type of operation,30-day mortality among the 604 patients who underwent aortic-rootreplacement with a composite graft was 3.5 percent (21 patients;95 percent confidence interval, 2.2 to 5.4 percent). Among the21 patients who underwent aortic-root replacement with a homograft,the 30-day mortality rate was 4.8 percent (1 patient; 95 percentconfidence interval, 0.2 to 26 percent). Among the 50 patientswho underwent valve-sparing repair, the 30-day mortality ratewas 0 percent.
Twelve of the 22 patients who died within 30 days after surgeryhad acute or chronic dissection of the ascending aorta. Theprincipal cause of death was low cardiac output postoperativelyin seven patients and arrhythmia in six patients. The remainingnine patients died as a result of preoperative aortic rupture(four patients), mediastinitis (one patient), myocardial infarction(one patient), and unknown causes (three patients).
There were 202 patients with ascending aortic dissection atthe time of surgery. The 30-day mortality rate was 9.1 percent(95 percent confidence interval, 4.5 to 17 percent) among the99 patients with acute dissection and 1.9 percent (95 percentconfidence interval, 0.3 to 7.5 percent) among the 103 patientswith chronic dissection. A total of 185 patients with dissectionof the ascending aorta received a composite graft, 15 underwenta valve-sparing procedure, and 2 received a homograft aorticroot.
Eighty-one of the patients also underwent a mitral-valve procedure.The overall 30-day mortality rate in this group was 7.4 percent(95 percent confidence interval, 3.0 to 16 percent). Thirty-fivepatients underwent concomitant mitral repair with an annuloplastyring, none of whom died within the first 30 days after surgery.Forty-six patients required concomitant mitral-valve replacement,six of whom died within 30 days postoperatively (13.0 percent;95 percent confidence interval, 5.4 to 27 percent).
Late Results
A parametric survival analysis showed an initially high riskof death for approximately the first 60 days postoperativelythat was followed by a low-risk late phase in which the riskwas constant for the duration of the study. The survival ratewas 93 percent at 1 year, 91 percent at 2 years, 84 percentat 5 years, 75 percent at 10 years, and 59 percent at 20 years.Figure 2 shows the Kaplan–Meier survival estimates stratifiedaccording to the urgency of the procedure.
Figure 2. Kaplan–Meier Survival Analysis of 675 Patients with Marfan's Syndrome, According to the Urgency of the Procedure.
I bars are 95 percent confidence intervals.
Dissection or rupture of the residual aorta (or both) and arrhythmiawere the leading causes of late death (Table 2). Of the 22 patientswho died of dissection or rupture of the residual aorta, 8 (36percent) had a DeBakey type I dissection (dissection arisingin the ascending aorta and extending into the descending aorta)at presentation.
Table 2. Causes of Death More Than 30 Days after Aortic-Root Replacement.
The results of the parametric analysis of the risk of deathassociated with various factors are shown in Table 3. Previousascending-aorta surgery (with or without concomitant aortic-valvereplacement), urgent repair, and emergency repair were all associatedwith an increased risk of death within the first 60 days aftersurgery. The presence of New York Heart Association class IVcongestive heart failure preoperatively was the only importantpredictor of the risk of death during the late postoperativephase. The presence of dissection preoperatively was highlycorrelated with the likelihood of urgent or emergency repair.In the final multivariable equation, age and a history of mitral-valvesurgery were not predictive of survival.
Table 3. Multivariable Parametric Hazard Analysis of Risk Factors for Death, According to the Time after Surgery.
Major late complications are presented in Table 4. Thromboembolismwas the most common late complication after aortic-root replacement.Of the 27 patients who had thromboembolism, 25 had receiveda composite graft, 1 had received a homograft aortic root, and1 had undergone a valve-sparing procedure. One patient had aprosthetic-valve thrombosis 10 years postoperatively but recoveredafter repeated composite-graft placement. Twelve of the 25 patientswith cerebral emboli had a complete recovery. The embolism occurredwithin 35 days after surgery in 9 of 25 patients and between3 and 13 years postoperatively in 16 patients. The actuariallikelihood of the absence of thromboembolism among the 593 patientswho received a composite graft and who survived the immediateperioperative period was 97 percent at 5 years, 94 percent at10 years, and 90 percent at 20 years. The linearized rate ofthromboembolism among these patients was 0.62 event per 100patient-years (25 events in 4041 patient-years of follow-up).
Table 4. Complications Occurring More Than 30 Days after Aortic-Root Replacement.
Endocarditis developed in 24 patients (Table 4). Fourteen ofthese patients were treated successfully: seven with antibioticsand four by replacement of the composite graft with a cryopreservedhomograft. Three of the four patients whose endocarditis wastreated by replacement of the composite graft did not survive.The overall likelihood of the absence of endocarditis was 97percent at 5 years, 95 percent at 10 years, and 84 percent at20 years.
Twenty-two of the 653 patients in our study who were dischargedfrom the hospital (3.4 percent) died of late dissection or ruptureof the residual aorta. Sixty-three of the 653 patients who weredischarged from the hospital (9.6 percent) had late aortic surgeryfor progressive disease, and 46 were still alive at the timeof the last follow-up.
None of the 50 patients who underwent a valve-sparing proceduredied in the early postoperative period. The first four valve-sparingoperations were performed in Zurich 19 to 29 years ago by Senningand are included in this report. At the time of this writing,one of these patients has moderate aortic insufficiency 20 yearsafter operation, one died of distal aortic rupture 7 years postoperatively,one died of unknown causes 21 years postoperatively, and onewas lost to follow-up 12 years after surgery. The remaining46 patients have been followed for 18 months to 9 years postoperatively.Two of these patients have died (one of amyotrophic lateralsclerosis at 1.8 years and one of pneumonia at 3.7 years). Asof this writing, 15 have no aortic insufficiency, 21 have mild-to-moderatepostoperative aortic insufficiency, and 1 patient with severeinsufficiency required a second operation. The presence or absenceof postoperative aortic insufficiency was not recorded in sevenpatients.
In Senning's early valve-sparing procedures, the left coronaryaortic sinus and coronary ostium were left intact. The remainderof the aortic root was replaced with a prosthetic graft, andthe right coronary artery was reimplanted into the graft. Sarsamand Yacoub modified this procedure in 1979 by resecting theentire aneurysmal portion of the aortic root and replacing itwith a prosthetic graft into which the coronary arteries areimplanted.8 Two recent variations of the valve-sparing operationhave been described by David and Feindel9 and David.10 In thecurrent study, Senning's original valve-sparing procedure wasused in 4 patients, the modified procedure of Sarsam and Yacoub8was used in 2 patients, and the modified procedure of Davidand Feindel9 and David10 was used in 44 patients.
Discussion
The introduction of the composite graft–valve procedureby Bentall and De Bono in 1968 changed the bleak outlook forpatients with Marfan's syndrome and aneurysm of the ascendingaorta. Our survey of 10 major surgical centers worldwide indicatesthat elective repair of such aneurysms in patients with Marfan'ssyndrome is associated with a low mortality rate (<2.0 percent).On the other hand, the 30-day mortality rate associated withemergency repair was eight times as high.
Kouchoukos and Dougenis have recommended that patients withMarfan's syndrome undergo elective replacement of the ascendingaorta when the diameter of the aorta exceeds 5.0 to 5.5 cm.11Similarly, Coady et al. monitored 230 patients with thoracicaneurysms from 1985 to 199612 and found that the odds of ruptureor acute dissection are 8.8 times as high among patients withaneurysms of 6.0 to 6.9 cm as among patients with aneurysmsof 4.0 to 4.9 cm. They suggest that 5.5 cm is an acceptablediameter for prophylactic resection of ascending aortic aneurysms.Almost half the adult patients with aortic dissection in ourstudy (46 percent) had an aortic diameter of 6.5 cm or less;it is the policy of the centers included in the study to repairthese aneurysms prophylactically when the diameter reaches 5.5to 6.0 cm, regardless of a patient's symptoms.
Often, an adult patient who has an aneurysm with a diameterof 6.0 cm has minimal aortic regurgitation or none at all andis asymptomatic, and aneurysms of this size are frequently notapparent on routine chest films. Unfortunately, every year manypatients with Marfan's syndrome and previously unrecognizedaneurysms require emergency surgery for acute dissection orsustain fatal rupture before surgery can be performed. Not infrequentlysuch patients had skeletal and ocular changes diagnostic ofMarfan's syndrome that were not recognized by the patients'physicians. There is also a widespread misconception that theoperative risk is high in patients with Marfan's syndrome, andso patients who have sizable aneurysms are not referred forsurgical repair. These circumstances are unfortunate becauselong-term results after elective repair of the aortic root inpatients with Marfan's syndrome are good. The majority resumeactive lifestyles and have no complications associated withthe aortic prosthesis. However, prosthetic endocarditis remainsa potentially serious late problem. Since it is commonly associatedwith the performance of dental work in the absence of prophylacticantibiotics, we recommend that our patients with mechanicalprostheses receive parenteral rather than oral prophylacticantibiotics before and after dental procedures. Though composite-graftendocarditis can sometimes be cured with antibiotics, seriousinfections ordinarily require replacement of the prosthesiswith a homograft aortic root. Our results indicate that repeatedcomposite-graft placement is usually an inadequate treatmentfor endocarditis.
After aortic-root replacement, the residual aorta must be monitoredby serial computed tomography or magnetic resonance imaging.Unfortunately, 22 of our 653 patients who were discharged fromthe hospital (3.4 percent) died of late dissection or ruptureof the residual aorta. In many of these patients, routine serialmonitoring of the aorta had not been carried out.
The role of valve-sparing procedures in patients with Marfan'ssyndrome remains unclear. These procedures, introduced by Senning29 years ago and popularized by Yacoub and David in recent years,are widely used for other types of patients with ascending aorticaneurysms, but used with caution in patients with Marfan's syndrome.Several surgeons who participated in this study believe thatMarfan's syndrome is a contraindication for valve-sparing surgery.There has been concern about the possibility of dilatation ofthe aortic annulus after the valve-sparing procedure. Recenthistologic studies by Fleischer et al. revealed a high degreeof structural deterioration of aortic leaflets excised frompatients with Marfan's syndrome at the time of aortic-root replacement.13Using an immunofluorescence staining technique, these investigatorsdemonstrated fragmentation and scarcity of fibrillin, a majorstructural protein in the aortic leaflet, in the excised aorticleaflets. Their findings support a cautious approach to theuse of valve-sparing procedures in patients with Marfan's syndrome.The procedure of choice among most surgeons in this study isthe composite graft–valve procedure of Bentall and DeBono, but several believe there is a role for valve-sparingprocedures among children and young women, the latter becauseof the risk of anticoagulation during pregnancy. Nevertheless,an unexpected finding in our study was the outstanding long-termresults in four patients who had undergone a valve-sparing procedureat the University of Zurich more than 19 years earlier. Also,recently Yacoub et al.14 described excellent long-term results(September 1979 through April 1997) with the valve-sparing procedurein 68 patients with skeletal manifestations of Marfan's syndrome.
Our survey of surgical repair of the aorta demonstrates thatthe outlook for patients with Marfan's syndrome has improveddramatically in the past 25 years. Notwithstanding major recentadvances in the understanding of the cause of the syndrome,15aggressive, preventive medical and surgical management willremain the mainstay of treatment in the near term. Much is stillto be learned about the pathogenesis of the condition —for instance, how it is that the mutation of one allele of thefibrillin-1 gene (often so small as to affect only 1 nucleotideout of 10,000 in the coding sequence) in a specific patientadversely affects the extracellular matrix in diverse organsand tissues.15,16 Knowledge of the mutation in a patient canallow the identification of relatives at risk for cardiovascularproblems before the diagnosis could be established on the basisof clinical criteria.17 Similarly, molecular testing may identifya relative who does not have the mutation and is therefore atlow risk for aortic problems; with this information that personcan avoid the expense and inconvenience of repeated evaluation.18
Recently, it has been shown that targeting the fibrillin-1 genein the mouse stimulates the vascular phenotype of Marfan's syndrome.19One goal of such research is to create a model of the syndromein a larger animal. The use of somatic gene therapy to corrector ameliorate the fibrillin-1 defect, even in a localized fashion,seems years away, but progress is being made.20 Until more effectivemethods are developed, patients and their families should understandthat cardiovascular complications of Marfan's syndrome can bemanaged effectively in most cases by moderate restriction ofphysical activity, ß-adrenergic blockade, routineimaging of the aorta, and prophylactic replacement of the aorticroot before the diameter exceeds 5.5 to 6.0 cm.12,16,21
Supported in part by the Dana and Albert "Cubby" Broccoli Centerfor Aortic Diseases at Johns Hopkins Hospital, by a grant (HL35877)from the National Institutes of Health, and by the NationalMarfan Foundation.
We are indebted to Mrs. Vera Kuda for her assistance with thepreparation of the manuscript, and to the following for datacollection at the 10 surgical centers: Dr. Duan Mingke at HannoverUniversity Hospital, Ms. Mireille Fontanel at HôpitalLa Pitié, Ms. Susan Murphy and Dr. Dimitrios Dougenisat Jewish Hospital, Ms. Barbara Dobbs and Dr. Jorge Salazarat Johns Hopkins Hospital, Ms. Autumn Hall and Ms. Kathy Clementat Methodist Hospital, Dr. M. Arison Ergin at Mt. Sinai Hospital(Dr. Ergin was also the primary surgeon for many of the patientsat Mt. Sinai and assisted with the preparation of the manuscript),Ms. Kathy Moore and Ms. Katrina Higbee at Stanford UniversityMedical Center, Ms. Dena Houchin and Ms. Luceli Cuasay at TexasHeart Institute, Ms. Susan Armstrong at Toronto Hospital, andDr. Mariette Schonbeck at Zurich University Hospital.
Source Information
From the Division of Cardiac Surgery, Johns Hopkins Hospital, Baltimore (V.L.G., P.S.G., D.E.A., D.E.C., A.M.G.); the Department of Surgery, University of Alabama, Birmingham (D.C.N.); the Department of Cardiothoracic Surgery, Stanford University Medical Center, Stanford, Calif. (D.C.M.); the Division of Cardiac Surgery, St. Joseph Hospital, Baltimore (J.C.L.); and the Department of Human Genetics, Allegheny University of the Health Sciences, Pittsburgh (R.E.P.). Other authors were Hans G. Borst, M.D., Division of Cardiothoracic Surgery, Hannover University Hospital, Hannover, Germany; Christian E.A. Cabrol, M.D., Department of Cardiothoracic Surgery, Hôpital La Pitié, Paris; Denton A. Cooley, M.D., Department of Surgery, Texas Heart Institute, Houston; Joseph S. Coselli, M.D., Department of Surgery, Methodist Hospital, Houston; Tirone E. David, M.D., Division of Cardiovascular Surgery, Toronto Hospital, Toronto; Randall B. Griepp, M.D., Department of Cardiothoracic Surgery, Mt. Sinai Hospital, New York; Nicholas T. Kouchoukos, M.D., Division of Cardiothoracic Surgery, Jewish Hospital, St. Louis; and Marko I. Turina, M.D., Department of Cardiovascular Surgery, University Hospital, Zurich, Switzerland.
Address reprint requests to Dr. Gott at the Division of Cardiac Surgery, 618 Blalock Bldg., Johns Hopkins Hospital, Baltimore, MD 21287-4618, or at vgott{at}csurg.jhmi.jhu.edu.
References
Marfan AB. Un cas de déformation congénitale des quatre membres, plus prononcée aux extrémités, caractérisée par l'allongement des os avec un certain degré d'amincissement. Bull Soc Hop Paris 1896;13:220-6.
Baer RW, Taussig HB, Oppenheimer EH. Congenital aneurysmal dilatation of aorta associated with arachnodactyly. Bull Johns Hopkins Hosp 1943;72:309-31.
McKusick VA. Cardiovascular aspects of Marfan's syndrome: heritable disorder of connective tissue. Circulation 1955;11:321-342. [Medline]
Murdoch JL, Walker BA, Halpern BL, Kuzma JW, McKusick VA. Life expectancy and causes of death in the Marfan syndrome. N Engl J Med 1972;286:804-808.
Bentall HH, De Bono A. A technique for complete replacement of the ascending aorta. Thorax 1968;23:338-339. [Free Full Text]
Gott VL, Cameron DE, Pyeritz RE, Reitz BA. The Marfan syndrome. Chest Surg Clin N Am 1992;2:425-437.
Blackstone EH, Naftel DC, Turner ME Jr. The decomposition of time-varying hazard into phases, each incorporating a separate stream of concomitant information. J Am Stat Assoc 1986;81:615-24.
Sarsam MAI, Yacoub M. Remodeling of the aortic valve anulus. J Thorac Cardiovasc Surg 1993;105:435-438. [Abstract]
David TE, Feindel CM. An aortic valve-sparing operation for patients with aortic incompetence and aneurysm of the ascending aorta. J Thorac Cardiovasc Surg 1992;103:617-622. [Abstract]
David TE. An anatomic and physiologic approach to acquired heart disease: 8th Annual Meeting of the European Cardio-thoracic Association, The Hague, Netherlands, September 25-28, 1994. Eur J Cardiothorac Surg 1995;9:175-180. [CrossRef][Medline]
Kouchoukos NT, Dougenis D. Surgery of the thoracic aorta. N Engl J Med 1997;336:1876-1888. [Free Full Text]
Coady MA, Rizzo JA, Hammond GL, et al. What is the appropriate size criterion for resection of thoracic aortic aneurysms? J Thorac Cardiovasc Surg 1997;113:476-491. [Free Full Text]
Fleischer KJ, Nousari HC, Anhalt GJ, Stone CD, Laschinger JC. Immunohistochemical abnormalities of fibrillin in cardiovascular tissues in Marfan's syndrome. Ann Thorac Surg 1997;63:1012-1017. [Free Full Text]
Yacoub MH, Gehle P, Chandrasekaran V, Birks EJ, Child A, Radley-Smith R. Late results of a valve-preserving operation in patients with aneurysms in the ascending aorta and root. J Thorac Cardiovasc Surg 1998;115:1080-1090. [Free Full Text]
Dietz HC, Pyeritz RE. Mutations in the human gene for fibrillin-1 (FBN1) in the Marfan syndrome and related disorders. Hum Mol Genet 1995;4:1799-1809. [Abstract]
Pyeritz RE. Marfan syndrome and other disorders of fibrillin. In: Rimoin DL, Connor JM, Pyeritz RE, eds. Emery and Rimoin's principles and practice of medical genetics. 3rd ed. Vol. 1. New York: Churchill Livingstone, 1997:1027-66.
De Paepe A, Devereux RB, Dietz HC, Hennekam RC, Pyeritz RE. Revised diagnostic criteria for the Marfan syndrome. Am J Med Genet 1996;62:417-426. [CrossRef][Medline]
Pereira L, Levran O, Ramirez F, et al. A molecular approach to the stratification of cardiovascular risk in families with Marfan's syndrome. N Engl J Med 1994;331:148-153. [Free Full Text]
Pereira L, Andrikopoulous K, Tian J, et al. Targetting of the gene encoding fibrillin-1 recapitulates the vascular aspect of Marfan syndrome. Nat Genet 1997;17:218-222. [CrossRef][Medline]
Montgomery RA, Dietz HC. Inhibition of fibrillin 1 expression using U1 snRNA as a vehicle for the presentation of antisense targeting sequence. Hum Mol Genet 1997;6:519-525. [Free Full Text]
Shores J, Berger KR, Murphy EA, Pyeritz RE. Progression of aortic dilatation and the benefit of long-term ß-adrenergic blockade in Marfan's syndrome. N Engl J Med 1994;330:1335-1341. [Free Full Text]
Botta, L., Russo, V., La Palombara, C., Rosati, M., Di Bartolomeo, R., Fattori, R.
(2009). Stent graft repair of descending aortic dissection in patients with Marfan syndrome: An effective alternative to open reoperation?. J. Thorac. Cardiovasc. Surg.
138: 1108-1114
[Abstract][Full Text]
David, T. E., Armstrong, S., Maganti, M., Colman, J., Bradley, T. J.
(2009). Long-term results of aortic valve-sparing operations in patients with Marfan syndrome.. J. Thorac. Cardiovasc. Surg.
138: 859-864
[Abstract][Full Text]
Shirley, L. E. D., Sponseller, P. D.
(2009). Marfan Syndrome. J Am Acad Orthop Surg
17: 572-581
[Abstract][Full Text]
Vargas, H A, Hoey, E T D, Gopalan, D, Agrawal, S K B, Screaton, N J, Gulati, G S
(2009). Congenital and acquired conditions of the aortic root: multidetector computed tomography features. Postgrad. Med. J.
85: 383-391
[Abstract][Full Text]
Parish, L. M., Gorman, J. H. III, Kahn, S., Plappert, T., St. John-Sutton, M. G., Bavaria, J. E., Gorman, R. C.
(2009). Aortic size in acute type A dissection: implications for preventive ascending aortic replacement. Eur. J. Cardiothorac. Surg.
35: 941-946
[Abstract][Full Text]
Everitt, M. D., Pinto, N., Hawkins, J. A., Mitchell, M. B., Kouretas, P. C., Yetman, A. T.
(2009). Cardiovascular surgery in children with Marfan syndrome or Loeys-Dietz syndrome.. J. Thorac. Cardiovasc. Surg.
137: 1327-1333
[Abstract][Full Text]
Volguina, I. V., Miller, D. C., LeMaire, S. A., Palmero, L. C., Wang, X. L., Connolly, H. M., Sundt, T. M. III, Bavaria, J. E., Dietz, H. C., Milewicz, D. M., Coselli, J. S., Aortic Valve Operative Outcomes in Marfan Patients,
(2009). Valve-sparing and valve-replacing techniques for aortic root replacement in patients with Marfan syndrome: Analysis of early outcome.. J. Thorac. Cardiovasc. Surg.
137: 1124-1132
[Abstract][Full Text]
Volguina, I. V., Miller, D. C., LeMaire, S. A., Palmero, L. C., Wang, X. L., Connolly, H. M., Sundt, T. M. III, Bavaria, J. E., Dietz, H. C., Milewicz, D. M., Coselli, J. S., Aortic Valve Operative Outcomes in Marfan Patients,
(2009). Valve-sparing and valve-replacing techniques for aortic root replacement in patients with Marfan syndrome: analysis of early outcome.. J. Thorac. Cardiovasc. Surg.
137: 641-649
[Abstract][Full Text]
Dong, Z. H., Fu, W. G., Wang, Y. Q., Guo, D. Q., Xu, X., Ji, Y., Chen, B., Jiang, J. H., Yang, J., Shi, Z. Y., Zhu, T., Shi, Y.
(2009). Retrograde Type A Aortic Dissection After Endovascular Stent Graft Placement for Treatment of Type B Dissection. Circulation
119: 735-741
[Abstract][Full Text]
Pyeritz, R. E
(2009). Marfan syndrome: 30 years of research equals 30 years of additional life expectancy. Heart
95: 173-175
[Full Text]
Nienaber, C. A., Akin, I., Erbel, R., Haverich, A.
(2009). CHAPTER 31 Diseases of the Aorta and Trauma to the Aorta and the Heart. ESC Textbook of Cardiovascular Medicine
2: med-9780199566990-chapter-med-9780199566990-chapter
[Abstract][Full Text]
Marcheix, B., Rousseau, H., Bongard, V., Heijmen, R. H., Nienaber, C. A., Ehrlich, M., Amabile, P., Beregi, J.-P., Fattori, R.
(2008). Stent Grafting of Dissected Descending Aorta in Patients With Marfan's Syndrome: Mid-Term Results. J Am Coll Cardiol Intv
1: 673-680
[Abstract][Full Text]
Skaggs, D. L., Bushman, G., Grunander, T., Wong, P. C., Sankar, W. N., Tolo, V. T.
(2008). Shortening of Growing-Rod Spinal Instrumentation Reverses Cardiac Failure in Child with Marfan Syndrome and Scoliosis. A Case Report. JBJS
90: 2745-2750
[Full Text]
Tsunekawa, T., Ogino, H., Matsuda, H., Minatoya, K., Sasaki, H., Kobayashi, J., Yagihara, T., Kitamura, S.
(2008). Composite Valve Graft Replacement of the Aortic Root: Twenty-Seven Years of Experience at One Japanese Center. Ann. Thorac. Surg.
86: 1510-1517
[Abstract][Full Text]
Aalberts, J. J.J., Waterbolk, T. W., van Tintelen, J. P., Hillege, H. L., Boonstra, P. W., van den Berg, M. P.
(2008). Prophylactic aortic root surgery in patients with Marfan syndrome: 10 years' experience with a protocol based on body surface area. Eur. J. Cardiothorac. Surg.
34: 589-594
[Abstract][Full Text]
Patel, H. J., Deeb, G. M.
(2008). Ascending and Arch Aorta: Pathology, Natural History, and Treatment. Circulation
118: 188-195
[Full Text]
Pyeritz, R. E.
(2008). A Small Molecule for a Large Disease. NEJM
358: 2829-2831
[Full Text]
Patel, N. D., Weiss, E. S., Alejo, D. E., Nwakanma, L. U., Williams, J. A., Dietz, H. C., Spevak, P. J., Gott, V. L., Vricella, L. A., Cameron, D. E.
(2008). Aortic Root Operations for Marfan Syndrome: A Comparison of the Bentall and Valve-Sparing Procedures. Ann. Thorac. Surg.
85: 2003-2011
[Abstract][Full Text]
Faivre, L, Collod-Beroud, G, Child, A, Callewaert, B, Loeys, B L, Binquet, C, Gautier, E, Arbustini, E, Mayer, K, Arslan-Kirchner, M, Stheneur, C, Kiotsekoglou, A, Comeglio, P, Marziliano, N, Halliday, D, Beroud, C, Bonithon-Kopp, C, Claustres, M, Plauchu, H, Robinson, P N, Ades, L, De Backer, J, Coucke, P, Francke, U, De Paepe, A, Boileau, C, Jondeau, G
(2008). Contribution of molecular analyses in diagnosing Marfan syndrome and type I fibrillinopathies: an international study of 1009 probands. J. Med. Genet.
45: 384-390
[Abstract][Full Text]
Keane, M. G., Pyeritz, R. E.
(2008). Medical Management of Marfan Syndrome. Circulation
117: 2802-2813
[Full Text]
Fazel, S. S., Mallidi, H. R., Lee, R. S., Sheehan, M. P., Liang, D., Fleischman, D., Herfkens, R., Mitchell, R. S., Miller, D. C.
(2008). The aortopathy of bicuspid aortic valve disease has distinctive patterns and usually involves the transverse aortic arch.. J. Thorac. Cardiovasc. Surg.
135: 901-907.e2
[Abstract][Full Text]
David, T. E.
(2008). Aortic Valve Repair and Aortic Valve Sparing Operations. Card Surg Adult
3: 935-948
[Full Text]
Brinster, D. R., Rizzo, R. J., Bolman, R. M. III
(2008). Ascending Aortic Aneurysms. Card Surg Adult
3: 1223-1250
[Full Text]
Ahimastos, A. A., Aggarwal, A., D'Orsa, K. M., Formosa, M. F., White, A. J., Savarirayan, R., Dart, A. M., Kingwell, B. A.
(2007). Effect of Perindopril on Large Artery Stiffness and Aortic Root Diameter in Patients With Marfan Syndrome: A Randomized Controlled Trial. JAMA
298: 1539-1547
[Abstract][Full Text]
Pape, L. A., Tsai, T. T., Isselbacher, E. M., Oh, J. K., O'Gara, P. T., Evangelista, A., Fattori, R., Meinhardt, G., Trimarchi, S., Bossone, E., Suzuki, T., Cooper, J. V., Froehlich, J. B., Nienaber, C. A., Eagle, K. A., on behalf of the International Registry of Acute A,
(2007). Aortic Diameter >=5.5 cm Is Not a Good Predictor of Type A Aortic Dissection: Observations From the International Registry of Acute Aortic Dissection (IRAD). Circulation
116: 1120-1127
[Abstract][Full Text]
Redruello, H. J., Cianciulli, T. F., Rostello, E. F., Recalde, B., Lax, J. A., Picone, V. P., Belforte, S. M., Prezioso, H. A.
(2007). Monozygotic twins with Marfan's syndrome and ascending aortic aneurysm. Eur J Echocardiogr
8: 302-306
[Abstract][Full Text]
Ha, H. I., Seo, J. B., Lee, S. H., Kang, J.-W., Goo, H. W., Lim, T.-H., Shin, M. J.
(2007). Imaging of Marfan Syndrome: Multisystemic Manifestations. RadioGraphics
27: 989-1004
[Abstract][Full Text]
Etz, C. D., Homann, T. M., Rane, N., Bodian, C. A., Di Luozzo, G., Plestis, K. A., Spielvogel, D., Griepp, R. B.
(2007). Aortic root reconstruction with a bioprosthetic valved conduit: A consecutive series of 275 procedures. J. Thorac. Cardiovasc. Surg.
133: 1455-1463
[Abstract][Full Text]
von Kodolitsch, Y., Robinson, P. N
(2007). Marfan syndrome: an update of genetics, medical and surgical management. Heart
93: 755-760
[Full Text]
Matsumori, M., Tanaka, H., Kawanishi, Y., Onishi, T., Nakagiri, K., Yamashita, T., Okada, K., Okita, Y.
(2007). Comparison of distensibility of the aortic root and cusp motion after aortic root replacement with two reimplantation techniques: Valsalva graft versus tube graft. ICVTS
6: 177-181
[Abstract][Full Text]
Svensson, L. G., Blackstone, E. H., Feng, J., de Oliveira, D., Gillinov, A. M., Thamilarasan, M., Grimm, R. A., Griffin, B., Hammer, D., Williams, T., Gladish, D. H., Lytle, B. W.
(2007). Are Marfan Syndrome and Marfanoid Patients Distinguishable on Long-Term Follow-Up?. Ann. Thorac. Surg.
83: 1067-1074
[Abstract][Full Text]
Miller, D. C.
(2007). Valve-Sparing Aortic Root Replacement: Current State of the Art and Where Are We Headed?. Ann. Thorac. Surg.
83: S736-S739
[Full Text]
Kallenbach, K., Baraki, H., Khaladj, N., Kamiya, H., Hagl, C., Haverich, A., Karck, M.
(2007). Aortic Valve-Sparing Operation in Marfan Syndrome: What Do We Know After a Decade?. Ann. Thorac. Surg.
83: S764-S768
[Abstract][Full Text]
Settepani, F., Szeto, W. Y., Pacini, D., De Paulis, R., Chiariello, L., Di Bartolomeo, R., Gallotti, R., Bavaria, J. E.
(2007). Reimplantation Valve-Sparing Aortic Root Replacement in Marfan Syndrome Using the Valsalva Conduit: An Intercontinental Multicenter Study. Ann. Thorac. Surg.
83: S769-S773
[Abstract][Full Text]
Bachet, J., Larrazet, F., Goudot, B., Dreyfus, G., Folliguet, T., Laborde, F., Guilmet, D.
(2007). When Should the Aortic Arch Be Replaced in Marfan Patients?. Ann. Thorac. Surg.
83: S774-S779
[Abstract][Full Text]
Westaby, S., Bertoni, G. B.
(2007). Fifty Years of Thoracic Aortic Surgery: Lessons Learned and Future Directions. Ann. Thorac. Surg.
83: S832-S834
[Abstract][Full Text]
Chavanon, O., Rama, A., Leprince, P., Bonnet, N., Pavie, A., Jondeau, G., Gandjbakhch, I.
(2006). Valve-sparing operation in a young woman with Marfan syndrome: A word of caution.. J. Thorac. Cardiovasc. Surg.
132: 683-684
[Full Text]
Engelfriet, P M, Boersma, E, Tijssen, J G P, Bouma, B J, Mulder, B J M
(2006). Beyond the root: dilatation of the distal aorta in Marfan's syndrome. Heart
92: 1238-1243
[Abstract][Full Text]
Patel, N. D., Williams, J. A., Barreiro, C. J., Bethea, B. T., Fitton, T. P., Dietz, H. C., Lima, J. A.C., Spevak, P. J., Gott, V. L., Vricella, L. A., Cameron, D. E.
(2006). Valve-Sparing Aortic Root Replacement: Early Experience With the De Paulis Valsalva Graft in 51 Patients. Ann. Thorac. Surg.
82: 548-553
[Abstract][Full Text]
Lima, B., Hughes, G. C., Lemaire, A., Jaggers, J., Glower, D. D., Wolfe, W. G.
(2006). Short-Term and Intermediate-Term Outcomes of Aortic Root Replacement with St. Jude Mechanical Conduits and Aortic Allografts. Ann. Thorac. Surg.
82: 579-585
[Abstract][Full Text]
Habashi, J.P., Judge, D.P., Holm, T.M., Cohn, R.D, Loeys, B., Cooper, T.K., Myers, L., Klein, E.C., Liu, G., Calvi, C., Podowski, M., Neptune, E.R., Halushka, M.K., Bedja, D., Gabrielson, K., Rifkin, D.B., Carta, L., Ramirez, F., Huso, D.L., Dietz, H.C., Bridgham, J.T., Carroll, S.M., Thornton, J.W.
(2006). Losartan in Marfan Syndrome--Beyond Blood Pressure Lowering: Losartan, an AT1 Antagonist, Prevents Aortic Aneurysm in a Mouse Model of Marfan Syndrome. Science 312: 117-121, 2006. J. Am. Soc. Nephrol.
17: 1759-1764
[Full Text]
Habashi, J. P., Judge, D. P., Holm, T. M., Cohn, R. D., Loeys, B. L., Cooper, T. K., Myers, L., Klein, E. C., Liu, G., Calvi, C., Podowski, M., Neptune, E. R., Halushka, M. K., Bedja, D., Gabrielson, K., Rifkin, D. B., Carta, L., Ramirez, F., Huso, D. L., Dietz, H. C.
(2006). Losartan, an AT1 antagonist, prevents aortic aneurysm in a mouse model of Marfan syndrome.. Science
312: 117-121
[Abstract][Full Text]
(2006). Characterization of the inflammatory and apoptotic cells in the aortas of patients with ascending thoracic aortic aneurysms and dissections.. J. Thorac. Cardiovasc. Surg.
131: 671-678
Davies, R. R., Gallo, A., Coady, M. A., Tellides, G., Botta, D. M., Burke, B., Coe, M. P., Kopf, G. S., Elefteriades, J. A.
(2006). Novel Measurement of Relative Aortic Size Predicts Rupture of Thoracic Aortic Aneurysms. Ann. Thorac. Surg.
81: 169-177
[Abstract][Full Text]
Neri, E., Barabesi, L., Buklas, D., Vricella, L. A., Benvenuti, A., Tucci, E., Sassi, C., Massetti, M.
(2005). Limited role of aortic size in the genesis of acute type A aortic dissection. Eur. J. Cardiothorac. Surg.
28: 857-863
[Abstract][Full Text]
Vricella, L. A., Williams, J. A., Ravekes, W. J., Holmes, K. W., Dietz, H. C., Gott, V. L., Cameron, D. E.
(2005). Early Experience With Valve-Sparing Aortic Root Replacement in Children. Ann. Thorac. Surg.
80: 1622-1627
[Abstract][Full Text]
Meijboom, L. J., Vos, F. E., Timmermans, J., Boers, G. H., Zwinderman, A. H., Mulder, B. J.M.
(2005). Pregnancy and aortic root growth in the Marfan syndrome: a prospective study. Eur Heart J
26: 914-920
[Abstract][Full Text]
Maron, B. J., Ackerman, M. J., Nishimura, R. A., Pyeritz, R. E., Towbin, J. A., Udelson, J. E.
(2005). Task Force 4: HCM and other cardiomyopathies, mitral valve prolapse, myocarditis, and Marfan syndrome. J Am Coll Cardiol
45: 1340-1345
[Full Text]
Kim, S. Y., Martin, N., Hsia, E. C., Pyeritz, R. E., Albert, D. A.
(2005). Management of Aortic Disease in Marfan Syndrome: A Decision Analysis. Arch Intern Med
165: 749-755
[Abstract][Full Text]
Milewicz, D. M., Dietz, H. C., Miller, D. C.
(2005). Treatment of Aortic Disease in Patients With Marfan Syndrome. Circulation
111: e150-e157
[Full Text]
Anttila, V., Piaszczynski, M., Mora, B., Hagino, I., Lacro, R. V., Zurakowski, D., Jonas, R. A.
(2005). Improved outcome with composite graft versus homograft root replacement for children with aortic root aneurysms. Eur. J. Cardiothorac. Surg.
27: 420-424
[Abstract][Full Text]
Zehr, K. J., Orszulak, T. A., Mullany, C. J., Matloobi, A., Daly, R. C., Dearani, J. A., Sundt, T. M. III, Puga, F. J., Danielson, G. K., Schaff, H. V.
(2004). Surgery for Aneurysms of the Aortic Root: A 30-Year Experience. Circulation
110: 1364-1371
[Abstract][Full Text]
Bethea, B. T., Fitton, T. P., Alejo, D. E., Barreiro, C. J., Cattaneo, S. M., Dietz, H. C., Spevak, P. J., Lima, J. A. C., Gott, V. L., Cameron, D. E.
(2004). Results of aortic valve-sparing operations: Experience with remodeling and reimplantation procedures in 65 patients. Ann. Thorac. Surg.
78: 767-772
[Abstract][Full Text]
Nollen, G. J., Groenink, M., Tijssen, J. G.P., van der Wall, E. E., Mulder, B. J.M.
(2004). Aortic stiffness and diameter predict progressive aortic dilatation in patients with Marfan syndrome. Eur Heart J
25: 1146-1152
[Abstract][Full Text]
Carrel, T., Beyeler, L., Schnyder, A., Zurmuhle, P., Berdat, P., Schmidli, J., Eckstein, F. S.
(2004). Reoperations and late adverse outcome in Marfan patients following cardiovascular surgery. Eur. J. Cardiothorac. Surg.
25: 671-675
[Abstract][Full Text]
Karck, M., Kallenbach, K., Hagl, C., Rhein, C., Leyh, R., Haverich, A.
(2004). Aortic root surgery in Marfan syndrome: Comparison of aortic valve-sparing reimplantation versus composite grafting. J. Thorac. Cardiovasc. Surg.
127: 391-398
[Abstract][Full Text]
Hopkins, R. A.
(2003). Aortic valve leaflet sparing and salvage surgery: evolution of techniques for aortic root reconstruction. Eur. J. Cardiothorac. Surg.
24: 886-897
[Abstract][Full Text]
Svensson, L. G., Kim, K.-H., Lytle, B. W., Cosgrove, D. M.
(2003). Relationship of aortic cross-sectional area to height ratio and the risk of aortic dissection in patients with bicuspid aortic valves. J. Thorac. Cardiovasc. Surg.
126: 892-893
[Full Text]
Roos-Hesselink, J W, Scholzel, B E, Heijdra, R J, Spitaels, S E C, Meijboom, F J, Boersma, E, Bogers, A J J C, Simoons, M L
(2003). Aortic valve and aortic arch pathology after coarctation repair. Heart
89: 1074-1077
[Abstract][Full Text]
Nienaber, C. A., Eagle, K. A.
(2003). Aortic Dissection: New Frontiers in Diagnosis and Management: Part II: Therapeutic Management and Follow-Up. Circulation
108: 772-778
[Full Text]
Carrel, T., Berdat, P., Pavlovic, M., Sukhanov, S., Englberger, L., Pfammatter, J.-P.
(2003). Surgery of the dilated aortic root and ascending aorta in pediatric patients: techniques and results. Eur. J. Cardiothorac. Surg.
24: 249-254
[Abstract][Full Text]
Iung, B., Baron, G., Butchart, E. G., Delahaye, F., Gohlke-Barwolf, C., Levang, O. W., Tornos, P., Vanoverschelde, J.-L., Vermeer, F., Boersma, E., Ravaud, P., Vahanian, A.
(2003). A prospective survey of patients with valvular heart disease in Europe: The Euro Heart Survey on Valvular Heart Disease. Eur Heart J
24: 1231-1243
[Abstract][Full Text]
Miller, D. C.
(2003). Valve-sparing aortic root replacement in patients with the Marfan syndrome. J. Thorac. Cardiovasc. Surg.
125: 773-778
[Full Text]
de Oliveira, N. C., David, T. E., Ivanov, J., Armstrong, S., Eriksson, M. J., Rakowski, H., Webb, G.
(2003). Results of surgery for aortic root aneurysm in patients with Marfan syndrome. J. Thorac. Cardiovasc. Surg.
125: 789-796
[Abstract][Full Text]
Albes, J. M., Wahlers, T.
(2003). Valve-sparing root reduction plasty in aortic aneurysm: the "Jena" technique. Ann. Thorac. Surg.
75: 1031-1033
[Abstract][Full Text]
David, T. E.
(2003). Aortic Valve Repair and Aortic Valve-Sparing Operations. Card Surg Adult
2: 811-824
[Full Text]
Anderson, C. A., Rizzo, R. J., Cohn, L. H.
(2003). Ascending Aortic Aneurysms. Card Surg Adult
2: 1123-1148
[Full Text]
Kim, K.-H., Lee, C., Ahn, H.
(2002). Successful treatment in a patient with Takayasu's arteritis and Marfan syndrome. Ann. Thorac. Surg.
74: 908-910
[Abstract][Full Text]
Iung, B., Gohlke-Barwolf, C., Tornos, P., Tribouilloy, C., Hall, R., Butchart, E., Vahanian, A.
(2002). Recommendations on the management of the asymptomatic patient with valvular heart disease. Eur Heart J
23: 1253-1266
Massih, T. A., Vouhe, P. R., Mauriat, P., Mousseaux, E., Sidi, D., Bonnet, D.
(2002). Replacement of the ascending aorta in children: A series of fourteen patients. J. Thorac. Cardiovasc. Surg.
124: 411-413
[Full Text]
Dean, J. C S
(2002). Management of Marfan syndrome. Heart
88: 97-103
[Full Text]
Rose, C., Wessel, A.
(2002). Three-decade follow-up in pulmonary artery ectasia: risk assessment strategy. Ann. Thorac. Surg.
73: 973-975
[Abstract][Full Text]
Wityk, R. J., Zanferrari, C., Oppenheimer, S.
(2002). Neurovascular Complications of Marfan Syndrome: A Retrospective, Hospital-Based Study. Stroke
33: 680-684
[Abstract][Full Text]
Gott, V. L., Cameron, D. E., Alejo, D. E., Greene, P. S., Shake, J. G., Caparrelli, D. J., Dietz, H. C.
(2002). Aortic root replacement in 271 Marfan patients: a 24-year experience. Ann. Thorac. Surg.
73: 438-443
[Abstract][Full Text]
Svensson, L. G., Khitin, L.
(2002). Aortic cross-sectional area/height ratio timing of aortic surgery in asymptomatic patients with Marfan syndrome. J. Thorac. Cardiovasc. Surg.
123: 360-361
[Full Text]
Prifti, E., Bonacchi, M., Frati, G., Proietti, P., Giunti, G., Babatasi, G., Massetti, M., Sani, G.
(2002). Early and long-term outcome in patients undergoing aortic root replacement with composite graft according to the Bentall's technique. Eur. J. Cardiothorac. Surg.
21: 15-21
[Abstract][Full Text]
Loeys, B., Nuytinck, L., Delvaux, I., De Bie, S., De Paepe, A.
(2001). 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
161: 2447-2454
[Abstract][Full Text]
Alexiou, C., Langley, S. M., Charlesworth, P., Haw, M. P., Livesey, S. A., Monro, J. L.
(2001). Aortic root replacement in patients with Marfan's syndrome: the Southampton experience. Ann. Thorac. Surg.
72: 1502-1507
[Abstract][Full Text]
Erbel, R., Alfonso, F., Boileau, C., Dirsch, O., Eber, B., Haverich, A., Rakowski, H., Struyven, J., Radegran, K., Sechtem, U., Taylor, J., Zollikofer, Ch., Klein, W.W., Mulder, B., Providencia, L.A.
(2001). Diagnosis and management of aortic dissection: Task Force on Aortic Dissection, European Society of Cardiology. Eur Heart J
22: 1642-1681
Sundt, T. M. III, Mora, B. N., Moon, M. R., Bailey, M. S., Pasque, M. K., Gay, W. A. Jr
(2000). Options for repair of a bicuspid aortic valve and ascending aortic aneurysm. Ann. Thorac. Surg.
69: 1333-1337
[Abstract][Full Text]
Tambeur, L., David, T. E., Armstrong, S., Ivanov, J., Webb, G.
(2000). Results of surgery for aortic root aneurysm in patients with the Marfan syndrome. Eur. J. Cardiothorac. Surg.
17: 415-419
[Abstract][Full Text]
Schievink, W. I., Tamargo, R. J., Conway, J. E., Hutchins, G. M.
(1999). Marfan Syndrome and Intracranial Aneurysms • Response. Stroke
30
: 2759-2768
[Full Text]
Birks, E. J., Webb, C., Child, A., Radley-Smith, R., Yacoub, M. H.
(1999). Early and Long-Term Results of a Valve-Sparing Operation for Marfan Syndrome. Circulation
100
: II-29-II-35
[Abstract][Full Text]
Bubb, M. R., Treasure, T., Chow, T., Gallivan, S., Gott, V. L., Greene, P. S., Cameron, D. E.
(1999). Replacement of the Aortic Root in Marfan's Syndrome. NEJM
341: 1473-1474
[Full Text]
(1999). Aortic Surgery in Marfan's Syndrome. JWatch General
1999: 6-6
[Full Text]
Devereux, R. B., Roman, M. J.
(1999). Aortic Disease in Marfan's Syndrome. NEJM
340: 1358-1359
[Full Text]
Grebenc, M. L., Zech, E. R.
(2002). Case 42: Aortic Homograft Anastomotic Dehiscence and Pseudoaneurysm Formation. Radiology
222: 139-143
[Full Text]
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