Author + information
- Duke Cameron, MD∗ ()
- ↵∗Reprint requests and correspondence:
Dr. Duke Cameron, Division of Cardiac Surgery, Zayed 7107, Johns Hopkins Hospital, 1800 Orleans Street, Baltimore, Maryland 21287.
Marfan syndrome is the most common inherited connective tissue disorder in humans, but has an uncommonly unfavorable natural history (1). Life expectancy is shortened by about one-third, usually because of aortic dissection and rupture in the third decade of life (2). Over the past 40 years, remarkable progress has been made to improve the outlook for Marfan syndrome patients, and life expectancy is now estimated to be nearly normal. This has been the result of earlier clinical recognition of the syndrome in affected individuals, better imaging of the cardiovascular disease, medical therapy to reduce the rate of aortic dilation and complications, and finally prophylactic surgical aortic root replacement in patients with enlarged aortas at greatest risk of vascular catastrophe. The gold standard operation, the so-called Bentall operation in which the aortic root, ascending aorta, and valve are replaced with a Dacron prosthesis and a prosthetic valve, has established itself as one of the most effective, safe, reproducible, and durable operations in cardiac surgery and has saved countless lives (3).
The Bentall operation typically entails replacement of the aortic valve, which is often an innocent bystander of the aortic disease, notwithstanding its abnormal histological appearance. Replacement of the valve, although technically simpler and more expedient than preserving it, introduces a new set of problems. Mechanical valves mandate a lifetime of anticoagulation, bioprostheses have limited durability in young patients, and both types of valves carry a small risk of endocarditis and thromboembolism of 1% to 2% per year, but a significant cumulative lifetime risk.
Since the late 1980s, 2 surgical pioneers, Sir Magdi Yacoub in the United Kingdom and Tirone David in Toronto, have championed operations that replace the aortic root aneurysm with Dacron to prevent dissection and rupture but preserve the patient’s valve (4,5). Occasionally valve leaflet repairs are necessary as well to address regurgitation created by the root aneurysm or by intrinsic leaflet abnormalities. These “valve-sparing root replacement” operations have undergone multiple iterations and in the current form are known as “David” or “reimplantation” procedures. Their appeal has been freedom from the limitations imposed by valve prostheses. Experience from multiple centers throughout the world has shown that these operations have an operative risk similar to the Bentall, as well as short- and mid-term results on par with those of the Bentall overall (less thromboembolism, bleeding, and endocarditis but at the price of a slightly higher rate of reoperation for late aortic valve incompetence) (6,7).
In this issue of the Journal, the paper by David et al. (8) is a landmark report because of the large number of Marfan patients who had valve-sparing surgery (the largest single-institution experience) and the long-term follow-up. The results are noteworthy for excellent late survival and a low incidence of late aortic regurgitation, surgically treated or otherwise. The report also reminds us of the importance of late surveillance and clinical monitoring, as the most common cause of late death remains distal aortic dissection, and late valve failure remains an ongoing risk. David also showed us that valve cusp repair does not hinder long-term success and that operations that stabilize the annulus (“reimplantation” procedures) are more durable than those that do not (“remodeling” procedures).
These are extraordinary outcomes from an experienced master and pioneer surgeon, but can these results be replicated in other clinical centers by other surgeons? A multi-institutional study sponsored by the Marfan Foundation is under way and seeks to answer this question with a prospective, nonrandomized trial that compares outcomes of valve-sparing and traditional root replacement surgery in Marfan patients (9). At present, >300 patients from 19 institutions have been enrolled; approximately 75% have had valve-sparing operations and 25% have undergone the Bentall procedure. Operative mortality has been low (0.6%) and similar in the 2 groups, but there is a concerning 7% incidence of at least moderate regurgitation in the valve-sparing group at 1 year. Until more is known from this and other studies, the series of David et al. remains a beacon of what is possible, if not probable.
↵∗ Editorials published in the Journal of the American College of Cardiology reflect the views of the authors and do not necessarily represent the views of JACC or the American College of Cardiology.
Dr. Cameron has reported that he has no relationships relevant to the contents of this paper to disclose.
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