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Previous Volume 340:1307-1313 April 29, 1999 Number 17 Next

	Full Text PDF Editorial by Devereux, R. B. Letters Add to Personal Archive Add to Citation Manager Notify a Friend E-mail When Cited PubMed Citation

ABSTRACT

Background Replacement of the aortic root with a prosthetic graft and valve in patients with Marfan's syndrome may prevent premature death from rupture of an aneurysm or aortic dissection. We reviewed the results of this surgical procedure at 10 experienced surgical centers.

Methods A total of 675 patients with Marfan's syndrome underwent replacement of the aortic root. Survival and morbidity-free survival curves were calculated, and risk factors were determined from a multivariable regression analysis.

Results The 30-day mortality rate was 1.5 percent among the 455 patients who underwent elective repair, 2.6 percent among the 117 patients who underwent urgent repair (within 7 days after a surgical consultation), and 11.7 percent among the 103 patients who underwent emergency repair (within 24 hours after a surgical consultation). Of the 675 patients, 202 (30 percent) had aortic dissection involving the ascending aorta. Forty-six percent of the 158 adult patients with aortic dissection and a documented aortic diameter had an aneurysm with a diameter of 6.5 cm or less. There were 114 late deaths (more than 30 days after surgery); dissection or rupture of the residual aorta (22 patients) and arrhythmia (21 patients) were the principal causes of late death. The risk of death was greatest within the first 60 days after surgery, then rapidly decreased to a constant level by the end of the first year.

Conclusions Elective aortic-root replacement has a low operative mortality. In contrast, emergency repair, usually for acute aortic dissection, is associated with a much higher early mortality. Because nearly half the adult patients with aortic dissection had an aortic-root diameter of 6.5 cm or less at the time of operation, it may be prudent to undertake prophylactic repair of aortic aneurysms in patients with Marfan's syndrome when the diameter of the aorta is well below that size.

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.

Full Text of this Article

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Replacement of the Aortic Root in Marfan's Syndrome
Bubb M. R., Treasure T., Chow T., Gallivan S., Gott V. L., Greene P. S., Cameron D. E.
Extract | Full Text  
N Engl J Med 1999; 341:1473-1474, Nov 4, 1999. Correspondence

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