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Ann Thorac Surg 2002;73:438-443
© 2002 The Society of Thoracic Surgeons

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Original article: cardiovascular

Aortic root replacement in 271 Marfan patients: a 24-year experience¹

^a Division of Cardiac Surgery, the Institute of Genetic Medicine and the Howard Hughes Medical Institute, The Johns Hopkins Medical Institutions, Baltimore, Maryland, USA

Accepted for publication September 19, 2001.

* Address reprint requests to Dr Gott, 618 Blalock Building, The Johns Hopkins Hospital, 600 N. Wolfe Street, Baltimore, MD 21287, USA
e-mail: vgott{at}csurg.jhmi.jhu.edu

	Abstract

Top Footnotes Abstract Introduction Patients and methods Results Comment Acknowledgments References

 
Background. The introduction of composite graft repair of aortic root aneurysm by Hugh Bentall in 1968 promised Marfan patients the choice for a normal life expectancy. We performed our first Bentall composite graft procedure in 1976 and herein report our 24-year experience with 271 Marfan patients.

Methods. Between September 1976 and August 2000, 232 Marfan patients had a composite graft replacement of the aortic root, 15 patients received a homograft, and 24 had a valve-sparing procedure.

Results. Two hundred thirty-five Marfan patients underwent elective aortic root replacement with no 30-day mortality. Two early deaths occurred among 36 patients who underwent urgent or emergent operation. Eighty-three percent of patients in this series are currently alive. The actuarial freedom from thromboembolism, endocarditis, and reoperation on the residual aorta 20 years postoperatively was 93%, 90%, and 74%. Twenty-four patients have undergone valve-sparing procedures with encouraging results.

Conclusions. Elective aortic root replacement for Marfan patients can be performed with low operative risk. Elective repair before the aortic root reaches 6 cm in diameter is recommended to minimize risk of dissection and rupture.

	Introduction

Top Footnotes Abstract Introduction Patients and methods Results Comment Acknowledgments References

 
In 1896, Antoine Marfan described a 5-year-old girl with extremely long limbs that he characterized as arachnid or "spider like" [1]. Nearly 50 years passed before full elucidation of the Marfan syndrome as we know it today. After Marfan described the arachnodactyly in his article, a tendency for lens dislocation was described in 1914 and dominant inheritance of the trait in 1931. In 1943, Helen Taussig and associates published an article [2] describing two teenage girls at the Johns Hopkins Hospital with the foregoing three traits; tragically, both girls had died of ruptured ascending aortic aneurysms. In 1955, Victor McKusick at the Johns Hopkins Medical Institutions refined the description of the Marfan syndrome with a landmark article [3] and, about the same time, established a Marfan clinic at our hospital.

Before the era of open heart surgery, the majority of patients with Marfan syndrome died prematurely of aortic rupture, often by the third decade of life [4]. Even after open heart surgery became established, surgical management was reserved for Marfan patients with acute aortic dissection or rupture; as expected, operative results were poor. The senior author of this paper performed aortic root operations on five Marfan patients between 1965 and 1976. Four of these patients required emergent operation for acute dissection of the ascending aorta; the fifth patient underwent elective operation, but had end-stage left ventricular failure from aortic insufficiency. Three of these patients died in the early postoperative period resulting in a 60% mortality rate. Fortunately, this dismal outlook for Marfan patients with aortic root aneurysm changed dramatically with the new composite graft operation developed by Hugh Bentall in London and reported in 1968 [5].

In September 1976, we performed our first Bentall composite graft operation. Our 24-year experience with aortic root replacement in 271 Marfan patients is described herein. Although nearly 90% of these patients had a composite graft repair, 56% of the patients (18 of 32) operated on during the past 24 months have had a valve-sparing procedure.

	Patients and methods

Top Footnotes Abstract Introduction Patients and methods Results Comment Acknowledgments References

 
Patient selection
Two hundred seventy-one patients underwent aortic root replacement between September 1976 and August 2000. Two hundred thirty-two had a composite graft repair, 15 had aortic root replacement with a homograft, and 24 of the patients had a valve-sparing procedure. All patient data were entered into a computerized patient registry; late follow-up data were obtained from clinical records and direct contact with patients and their primary physicians. Late follow-up was achieved in 92% of the 271 patients.

Operative technique
Two hundred thirty-two patients underwent aortic root replacement with a composite graft as described by Bentall [5]. Our current operative technique has evolved from the original Bentall procedure and has been described in detail in several publications [6, 7]. Coronary artery buttons are anastomosed to the Dacron graft and the residual aneurysmal wall is loosely tacked over the composite graft. In the 24 patients who had a valve-sparing procedure, a technique modified from Sarsan and Yacoub [8] and David and Feirdel [9] was used.

Standard hypothermic (28° to 30°C) cardiopulmonary bypass was used. Myocardial protection was obtained by simple antegrade administration of crystalloid potassium cardioplegia solution and continuous topical saline (4°C) at 100 mL/min. Since 1995, several surgeons have used cold retrograde blood perfusion of the heart.

Statistical methods
Survival and event-free curves were calculated using the Kaplan-Meier method and are presented. Univariate analysis was performed using the Mantel-Cox method and the multivariate analysis by the Cox proportional hazards method. Statistical computations were made using BMDP Statistical Software Package (Los Angeles, CA).

	Results

Top Footnotes Abstract Introduction Patients and methods Results Comment Acknowledgments References

 
Preoperative patient characteristics
One hundred ninety-two of the 271 patients were men (71%) and 79 were women (29%). Mean patient age was 33 years (range, 1.5 to 73.3 years). Forty-eight of the 271 patients (18%) had dissection of the ascending aorta (Table 1); 21 were acute dissections and 27 were chronic (more than 14 days old). Mean diameter of the ascending aorta in 229 adult patients in which this measurement was noted in the hospital chart was 6.8 cm (range, 4.0 to 13 cm). The mean diameter of the 43 ascending aortas with dissection was 7.6 cm. Fourteen patients had dissection in an aorta 6.5 cm or smaller.

View larger version (13K): [in this window] [in a new window]   Fig 1. Actuarial survival of 271 Marfan patients after aortic root replacement. Survival was 89% at 5 years, 81% at 10 years, 76% at 15 years, and 67% at 20 years. Patients at risk are depicted at yearly intervals.

View larger version (13K): [in this window] [in a new window]   Fig 2. Actuarial freedom from thromboembolism. Freedom from thromboembolism was 96% at 5 years, and remained at 93% from 10 to 20 years. Patients at risk are depicted at yearly intervals.

Three patients developed late coronary anastomotic dehiscence; 2 were successfully repaired at 1 month and 2.2 years postoperatively. In 1 patient, there was sudden death 7 years postoperatively. Interestingly, all three of these patients with late coronary dehiscence were operated on before 1984. Since 1990 we have used the coronary button technique in the composite graft patients, and therefore do not anticipate further late coronary dehiscences.

Eight of 269 patients discharged from the hospital (3.0%) died of late dissection or rupture of the residual aorta (2 of these patients underwent attempted emergent repair for acute dissection). Twenty-six of the 269 patients (9.7%) discharged from the hospital had late aortic procedures for progressive disease or dissection; 18 of these patients (69.2%) were alive at last follow-up. Figure 3 presents actuarial freedom from reoperation on the residual aorta. Freedom from reoperation on the residual aorta was 96% at 5 years, 89% at 10 years, 83% at 15 years, and 74% at 20 years.

View larger version (13K): [in this window] [in a new window]   Fig 3. Actuarial freedom from reoperation on the residual aorta. Freedom from reoperation was 96% at 5 years, 89% at 10 years, 83% at 15 years, and 74% at 20 years. Patients at risk are depicted at yearly intervals.

View larger version (22K): [in this window] [in a new window]   Fig 4. Postoperative echocardiography results in 24 patients having the valve-sparing operation. (*These two patients received a prosthetic valve at this time in their postoperative course. This patient is in New York Heart Association functional class II. All four children with late 2+ aortic insufficiency are New York Heart Association functional class I or II.)

	Comment

Top Footnotes Abstract Introduction Patients and methods Results Comment Acknowledgments References

In our series of 271 patients, 48 came to the operating room with aortic dissection. Fourteen of the 43 (32.6%) adults with a preoperative diagnosis of dissection had an aortic diameter of 6.5 cm or less. Given this incidence of dissection in the Marfan patient when the aorta reaches 5.5 cm, it is reasonable to recommend prophylactic aortic replacement even earlier. It is also generally accepted when there is family history of dissection; operation should be carried out when the aortic root reaches 5.0 cm.

There have been important technical refinements in the Bentall composite graft procedure during the past 35 years that have further improved overall long-term results. One is Kouchoukos’ recommendation that the composite graft not be tightly wrapped with the aneurysm wall to contain bleeding [11]. This technique was frequently used in the original Bentall procedure because excessive postpump bleeding from the operative site was common and could be controlled with the aortic wrap. The "downside" of the blood-tight wrap was the possibility of a contained hematoma, which could impinge on the prosthetic valve; this complication occurred in one of our patients early in the series, but was corrected with an emergent operative procedure. Improved vascular grafts, operative technique, and management of coagulopathy have made postoperative bleeding less of a problem, therefore the aortic wrap has been eliminated. Another important technical refinement has been the coronary button technique, which nearly eliminates the possibility of coronary ostial dehiscence. It is important to note that the 3 patients in our series with late coronary dehiscence were operated on before 1984 (Table 6), when we first used this latter technique.

Late complications still occur after the Bentall procedure. Thromboembolism and endocarditis are the most common. Fifteen of our 269 hospital survivors developed thromboembolism. Virtually all were inadequately anticoagulated at the time of the thromboembolic event. One patient had thrombosis of a Björk-Shiley valve 10 years after operation; the valve was replaced and the patient has done well. Of 14 patients with postoperative emboli, 6 had emboli within 35 days after operation. Thromboembolism in these patients can be attributed to early inadequate anticoagulation. Noncompliance with coumadin has contributed to late thromboemboli in several patients. One individual had an embolus 8 years postoperatively, evidently after stopping his coumadin for several months. Fortunately, 9 of the 14 patients who sustained a thromboembolic episode made a complete recovery, whereas 5 were left with only mild incapacity.

Eleven patients developed late endocarditis; 5 patients with early prosthetic infection were successfully treated with antibiotics. We learned early in our experience that severe prosthetic infections are best managed by complete removal of all prosthetic material and replacement with a cryopreserved homograft root. Three patients were successfully repaired this way. Three of the 11 patients with endocarditis died, all without benefit of a homograft aortic root. Twenty-six of our 269 hospital survivors (9.7%) required subsequent operation for aortic dissection and impending rupture or progressive dilatation of the residual aorta; 18 of these patients are still alive. Eight patients died of unexpected aortic rupture demonstrating the necessity of lifelong monitoring of Marfan aneurysm patients with serial computed tomographic scans or magnetic resonance imaging.

As noted in Table 5, risk factors for long-term mortality as determined by multivariate analysis were preoperative New York Heart classification or the requirement for urgent operation. Interestingly, age, gender, preoperative dissection, and associated mitral valve surgery were not risk factors for mortality. Figure 1 presents actuarial survival of the 271 Marfan patients. It is gratifying that in this 24-year surgical experience, 83% of all operated patients are currently alive.

The most significant change in our surgical management of Marfan aneurysm of the aortic root since our last survey in 1996 [12] has been an increasing use of the valve-sparing procedure. In our 1996 article, we reported an overall surgical experience with 212 Marfan patients who underwent aortic root replacement. At the time of that report, we had only performed the valve-sparing procedure in 2 patients. In this current report, we include 24 patients who have had the valve-sparing procedure. In fact, 56% of the 32 Marfan patients undergoing aortic root repair in the past 24 months have had the valve-sparing procedure. This procedure, first introduced by Sarsan and Yacoub [8] and David and Feirdel [9] has obvious advantages, such as avoidance of lifetime anticoagulation and much lower rate of thromboembolism and endocarditis. These considerations make the valve-sparing procedure attractive, but caution is still warranted because of the potential for progressive annular dilatation and aortic valvular degeneration in patients with the Marfan syndrome. The late results for our 24 patients undergoing valve-sparing procedures are presented in Figure 4. It has been gratifying that 17 of the 24 patients have mild or no aortic regurgitation on follow-up echocardiography. Our results with this operation have been more encouraging in our adult patients than in the children. Although 6 of our 10 pediatric patients have only 0 to 1+ aortic insufficiency in late echocardiography follow-up, and the other 4 children have 2+ aortic insufficiency, several children have developed postoperative annular dilatation that is of some concern. In all of the pediatric patients, strips of Teflon felt (Bard Impra Inc, Tempe, AZ) were used to buttress the annular suture line. In our more recent pediatric patients, a circumferential heavy polypropylene suture has been placed around the proximal end of the Dacron graft, anchoring the three Dacron "tongues" to prevent dilatation. Our early results with this circumferential suture have been encouraging, but we remain selective in the application of the valve-sparing operation for both children and adults.

Clearly, the outlook for Marfan patients undergoing elective surgical repair of ascending aorta aneurysms is now much better than 30 years ago. Accompanying the improved surgical results have been remarkable developments in our understanding of the genetics of the Marfan syndrome. It has been known for years that Marfan disease is transmitted as an autosomal dominant trait. It has also been known for some time that the connective tissue of Marfan patients is deficient in the extracellular matrix protein, fibrillin-1. This defect in fibrillin not only explains the weakness in the aortic wall but similar structural weaknesses in the suspensory ligament of the lenses, and in tendons and ligaments.

In 1990, a Finnish research team led by Katariina Kainulainen [13] demonstrated that the defective gene in Marfan syndrome is located on the long arm of chromosome 15 and in 1991, Dietz and associates [14] reported their discovery of the first mutation in the fibrillin-1 gene. Six years later, Pereira and associates [15] reported that they had successfully developed a mouse model of the Marfan syndrome; these Marfan mice would, in fact, develop aneurysms of the ascending aorta, which in turn, would dissect and rupture. The use of somatic gene therapy to correct or ameliorate the fibrillin-1 defect, even in a localized fashion, seems years away but progress is being made [16].

A century ago, Sir William Osler stated, "There is no disease more conducive to clinical humility than aneurysm of the aorta." Certainly for Marfan patients with aortic aneurysm, this was true before 1968 when Bentall and DeBono published their landmark article. But now, Marfan patients can undergo elective operation with an operative risk well below 5% and have a chance at a normal life expectancy. However, it is unfortunate that even today, aneurysms are frequently not recognized and timely operation is not carried out, primarily because a 6-cm aneurysm of the aortic root usually produces no symptoms and may not be visible on routine chest roentgenogram. Misdiagnosis of aortic root dissection by emergency room physicians continues to be one of the most litigious areas in medicine. With a corrective operation that carries an operative risk of less than 5%, it is essential that cardiovascular surgeons continue to educate their medical colleagues about this problem.

	Acknowledgments

Top Footnotes Abstract Introduction Patients and methods Results Comment Acknowledgments References

 
This study was supported in part by the Dana and Albert "Cubby" Broccoli Center for Aortic Diseases at the Johns Hopkins Medical Institutions, by National Institutes of Health Grant RO1-AR41135, by the Howard Hughes Medical Institute, and by the Smilow Family Foundation.

The authors thank Drs Michael Acker, Michael Borkon, Robert Brawley, Alfred Casale, John Conte, Timothy Gardner, John Laschinger, Mark Redmond, Bruce Reitz, and Scott Stuart for allowing us to include their patients in this report. We also wish to thank Dr Sarah Clauss, a pediatric cardiology fellow at our institution, for her help in evaluating the children undergoing a valve-sparing procedure. We also thank Jo Ann Harper for her assistance in preparing our manuscript for publication.

	Footnotes

Top Footnotes Abstract Introduction Patients and methods Results Comment Acknowledgments References

 
¹ This manuscript was adapted in part from the William W. L. Glenn Lecture presented by Dr Gott at the American Heart Association Meeting, New Orleans, LA, Nov 13, 2000.

	References

Top Footnotes Abstract Introduction Patients and methods Results Comment Acknowledgments References

 

1. Marfan A.B. 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 Hosp Paris 1896;13:220-226.
2. Baer R.W., Taussig H.B., Oppenheimer E.H. Congenital aneurysmal dilatation of aorta associated with arachnodactyly. Bull Johns Hopkins Hosp 1943;72:309-331.
3. McKusick V.A. Cardiovascular aspects of Marfan syndrome: heritable disorder of connective tissue. Circulation 1955;11:321-342.[Medline]
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