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Ann Thorac Surg 1999;67:1859-1860
© 1999 The Society of Thoracic Surgeons

Operative management of Marfan syndrome: the Johns Hopkins experience

^a Division of Cardiac Surgery, The Johns Hopkins Hospital, Baltimore, Maryland, USA

Address reprint requests to Dr Baumgartner, Dept of Surgery, The Johns Hopkins Hospital, 600 N Wolfe St, Blalock 618, Baltimore, MD 21287-4618
e-mail: wbaumgar{at}csurg.jhmi.jhu.edu

Presented at the Aortic Surgery Symposium VI, April 30–May 1, 1998, New York, NY.

	Abstract

Top Abstract Introduction Material and methods Results Comments References

 
Background. Doctor Antoine Marfan described the first case of Marfan syndrome in 1896. It was over 50 years later that the development of aortic aneurysms and subsequent rupture was appreciated as the most life-threatening component of the syndrome.

Methods. Doctor Vincent Gott, at our institution, performed the first Bentall procedure for an aneurysm of the ascending aorta in 1976. Since that time, the aortic root has been replaced in 231 Marfan patients. Of this group, 218 patients had a composite graft repair, 11 had an aortic root replacement with a homograft, and 2 patients had valve sparing procedures. There were 168 males and 63 females. Of the total 231 patients, 150 were operated on by Dr Gott. The remaining 81 patients were operated on by 10 other Hopkins surgeons. The average diameter of the ascending aorta was 6.8 cm, with a range from 4.5 to 10. The average aortic diameter of 43 patients who had an ascending aortic dissection was 7.3 cm. Fourteen of these patients had dissection with an aortic diameter of 6.5 cm or less.

Results. Among the 198 patients who underwent elective repair, there was no 30-day mortality. Thirty-three patients underwent urgent repair with 2 deaths, yielding a 30-day mortality of 6.1%. The mortality for the entire group of patients was 0.9%. Complications associated with this series of patients included 8 with endocarditis, 7 with thromboembolism, and 4 late coronary dehiscences. Actuarial survival was 88% at 5 years, 81% at 10 years, and 75% at 20 years. Multivariate analysis revealed New York Heart Association classification, male gender and urgent surgery as independent risk factors for mortality.

Conclusion. Marfan patients with aortic aneurysms can undergo elective surgery with a low operative risk and excellent long-term survival with low morbidity. We feel that elective resection of an aneurysm in a Marfan patient should occur when it approaches a diameter of 5.5 cm. It is essential that a timely diagnosis be made in this group of young patients.

	Introduction

Top Abstract Introduction Material and methods Results Comments References

 
Doctor Antoine Marfan described the first patient with what is now called Marfan syndrome: the case history of a 5-year-old girl with typical findings which was published in the Bulletin of the Medical Society of Paris in 1896 [1]. It was over 50 years later that the development of aortic aneurysm and subsequent rupture was appreciated as the most life-threatening complication of this syndrome. Doctor Helen Taussig and associates published a paper in 1943 [2] describing two young girls with Marfan traits who died from a rupture of an aneurysm of the ascending aorta. Doctor Victor McKusick, Professor of Medicine and Genetics at The Johns Hopkins Hospital and University School of Medicine, further elaborated, elucidated and defined the clinical and hereditary aspects of the Marfan syndrome. In addition to establishing a Marfan clinic, he was instrumental in initiating basic genetic research, which continues today.

	Material and methods

Top Abstract Introduction Material and methods Results Comments References

 
Doctor Vincent Gott at The Johns Hopkins Hospital performed the first Bentall procedure for an aneurysm of the ascending aorta in 1976. A recent clinical review was conducted. The records of 231 Marfan patients undergoing aortic root surgery were analyzed. Two-hundred and eighteen patients underwent composite graft repair. Eleven patients had their aortic root replaced with a homograft, and 2 patients had valve sparing procedures. This cautious approach to the concept of sparing the aortic valve is the result of recent histologic studies demonstrating valvular structural deterioration similar to that observed in the aortic wall of the Marfan patient [3].

There were 168 males and 63 females, with an average age of 32.8 years, and a range from 4 to 73. The average diameter of the aortic root in 223 patients was 6.8 cm, ranging from 4.5 to 10. The average diameter of the ascending aorta in 43 patients with the diagnosis of dissection was 7.5 cm, but 14 patients had dissection with an aortic diameter of 6.5 cm.

	Results

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The operative results are depicted in Table 1. Among the 198 patients who had an elective repair, there were no deaths. There were 2 deaths among the 33 patients who underwent operation urgently.

Actuarial survival was 88% at 5 years, 81% at 10 years, and 75% at 20 years. Multivariate analysis revealed New York Heart Association classification, male gender and urgent surgery as independent risk factors for mortality. Of note, preoperative dissection, age and concomitant mitral valve surgery were not independent predictors of mortality.

	Comments

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Prior to the Bentall operation, surgical results in this group of patients were dismal [4]. In contrast, the 30-day mortality for aortic root replacement is < 5% in the majority of currently reported series. Late survival, as illustrated in this review as well as reported by others, is excellent. Complications have also been relatively infrequent. In particular, the risk of thromboembolism is low compared to other series of isolated aortic valve replacements with mechanical prostheses. This may, in part, be related to the composite valve graft design, in which all of the sutures and pledgets are outside the circulation following implantation.

There have been significant advances in discovering the genetic basis of the Marfan syndrome. It has been documented that the connective tissue of these patients contains defective fibrillin. The first report localizing the fibrillin gene to the long arm of chromosome 15 was by Dr Kainulainen and her Finnish associates [5]. One year later, Dr Hal Dietz and his associates at Johns Hopkins demonstrated the first mutations of the fibrillin gene [6]. It is anticipated that significant strides will be made in further characterizing the exact genetic constitution of the Marfan patient with the hope that this disease may one day be eradicated.

Unfortunately, even today, some patients with ascending aortic aneurysms continue undiagnosed. The Marfan syndrome is often present in athletes, and a high index of suspicion is needed to identify these patients in order to follow them with serial chest computed tomography (CT) or magnetic resonance imaging (MRI), enabling intervention before dissection or rupture occur.

Early mortality for this operation is low, and late morbidity and mortality are quite acceptable with the Bentall repair or its modifications. Concomitant mitral valve surgery can be carried out without an increase in operative risk. Known Marfan patients should undergo serial radiological evaluation to document a change in size of their ascending aorta. Operative repair is indicated for an aneurysm > 5.5 cm. In patients with a family history of dissection, surgery should be undertaken when the aneurysm approaches 5.0 cm.

	References

Top Abstract Introduction Material and methods Results Comments References

 

1. Marfan A.B. Un cas de deformation congenitale des quatre membres, plue prononcee aux extremities, caracterisee par l’allongement des os avec un certain degre d’amincissement. Bul Sco Chir Paris 1896;13:220-225.
2. Baer R.W., Taussig H., Oppenheimer E.H. Congenital aneurymsmal dilatation of the aorta associated with arachnodactyly. Bull Johns Hopkins Hosp 1943;72:309-331.
3. Fleischer K.J., Nausari H.C., Anhalt G.J., Laschinger J.C. Immunohistochemical abnormalities of fibrillin in cardiovascular tissues in Marfan syndrome. Ann Thorac Surg 1997;63:1014-1017.
4. Bentall H.H., DeBono A. A technique for complete replacement of the ascending aorta. Thorax 1968;23:338-339.[Abstract/Free Full Text]
5. Kainulainen K., Pulkkinen L., Savolainen A., et al. Location on chromosome 15 of the gene defect causing Marfan syndrome. N Engl J Med 1990;323:935-939.[Medline]
6. Dietz H.C., Cutting G.R., Pyeritz R.E., et al. Marfan syndrome caused by a recurrent de novo missense mutation in the fibrillin gene. Nature 1991;352:337-339.[Medline]

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This Article Abstract Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to Personal Folders Download to citation manager Author home page(s): William A. Baumgartner Duke E. Cameron Peter S. Greene Vincent L. Gott Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Baumgartner, W. A. Articles by Gott, V. L. Search for Related Content PubMed PubMed Citation Articles by Baumgartner, W. A. Articles by Gott, V. L.