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

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Abstract

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.

Section snippets

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

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

Comment

The introduction of the composite graft–valve procedure by Bentall and DeBono in 1968 dramatically changed the bleak outlook for patients with Marfan aneurysm of the ascending aorta. A recent survey of 10 major Marfan surgical centers worldwide reported an operative mortality rate of 1.5% for Marfan patients undergoing elective repair of aortic root aneurysms [10]. In that survey, 455 Marfan patients had elective repair of aortic root aneurysm; 117 had urgent repair (1 to 7 days after surgical

Acknowledgements

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

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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.

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