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Elective Aortic Root Surgery in Marfan Syndrome Appears Safe and Durable: Presented at STS

By Ed Susman

FORT LAUDERDALE, FL -- January 31, 2008 -- Surgery to repair and replace the aortic root in Marfan syndrome patients can be accomplished with little perioperative mortality and with durable results, doctors reported here at the Society of Thoracic Surgery 44th Annual Meeting.

"Aortic rupture or dissection is the most common cause of premature death among persons with Marfan syndrome," said Duke E. Cameron, MD, Professor of Surgery, James T. Dresher Sr. Professor, Director of Paediatric Cardiac Surgery, and Co-Director of The Dana and Albert "Cubby" Broccoli Centre for Aortic Diseases, Johns Hopkins University School of Medicine, Baltimore, Maryland, United States.

Marfan syndrome is the most common inherited connective tissue disorder. It shortens a person's lifespan by a third, Dr. Cameron said.

In his oral presentation on January 28, he presented the 30-year history of surgical treatment in 372 cases of Marfan syndrome at Johns Hopkins University. There were 265 males and 107 females, with about 13% of these being children. The mean age at surgery was 32.9 years.

"Elective prophylactic aortic root surgery has low operative risk and prevents the most common cause of premature death in Marfan syndrome," Dr. Cameron said.

Dr. Cameron said the main indication for surgery in Marfan cases is the expanded size of the aorta. Surgery is indicated for patients with aortas larger than 5.5 cm in diameter, family history of aortic rupture or dissection, acute or chronic dissection, rapid enlargement of the aorta greater then 1 cm per year, or progressive aortic insufficiency in a moderate aneurysm.

In the Hopkins case series, 72% of the repairs were performed with mechanical devices; 23% of cases were valve-sparring surgeries, and 5% of cases were used bioprostheses, he said.

All but 45 of the repairs were performed as elective surgery, Dr. Cameron said. Those 45 emergent cases also resulted in the only two deaths that occurred within 30 days of the surgical procedure, which represented 4.4% of urgent surgeries, 0.5% of the 372 cases reported.

In the newer valve-sparing cases, 8 of the 85 individuals experienced late grade 3 or 4 aortic insufficiency; 6 patients required aortic valve replacement; and two had late mortality. Overall 74 of the patients in the series died, 10 due to dissection or rupture of the residual aorta or iliac artery, and 9 due to arrhythmia; in 26 cases the cause of death was unknown.

A multivariate analysis determined that causes of late mortality were due to age (3% increased risk), which was, however, statistically significant (P =.002). Mitral valve surgery had an 85% increased risk (P =.039), and preoperative dissection had a 90% increased risk (P =.054).

Late morbidity included 19 cases of thromboembolism, endocarditis and coronary dehiscence. Three patients succumbed to endocarditis; one patient died due to dehiscence.


[Presentation title: Aortic Root Replacement in 372 Marfan Patients: Evolution of Operative Repair Over 30 Years. Abstract 2]

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