Valve-sparing aortic root replacement in Marfan syndrome

Duke E. Cameron, Luca Vricella

Research output: Contribution to journalArticle

Abstract

Marfan syndrome is the most common inherited connective tissue disorder, affecting approximately 1 in 10,000 live births. The cardinal features of Marfan syndrome are the abnormalities of the skeleton (tall stature, arachnodactyly, and joint hyperelasticity), eye (lens subluxation), and aorta (root aneurysm with proclivity toward rupture and dissection). Aortic catastrophe accounts for most of the premature mortality among Marfan patients, a risk that climbs steeply during adolescence and results in death of half of Marfan patients by the age of 40 years. Most of the improvement in life expectancy that has been achieved in Marfan syndrome is attributable to early recognition of aortic root aneurysms and prophylactic replacement with composite grafts (mechanical valve prostheses within Dacron conduits) before rupture or dissection occurs. Despite the excellent early and late results with composite grafts, there has been growing interest in operative procedures that replace the sinuses but preserve the aortic valve leaflets, to avoid anticoagulation and minimize the risk of prosthesis-related endocarditis. These procedures are still in evolution and late resuts are not yet known, but as with mitral repair in the setting of myxomatous disease, valve-sparing procedures in Marfan syndrome have weathered a storm of initial criticism and skepticism and are steadily gaining acceptance.

Original languageEnglish (US)
Pages (from-to)103-111
Number of pages9
JournalSeminars in thoracic and cardiovascular surgery. Pediatric cardiac surgery annual
Volume8
Issue number1
DOIs
StatePublished - 2005

Fingerprint

Marfan Syndrome
Aortic Valve
Prostheses and Implants
Dissection
Rupture
Arachnodactyly
Lens Subluxation
Transplants
Crystalline Lens
Polyethylene Terephthalates
Premature Mortality
Aortic Aneurysm
Operative Surgical Procedures
Live Birth
Life Expectancy
Endocarditis
Skeleton
Connective Tissue
Aneurysm
Aorta

Keywords

  • Aortic catastrophe
  • Connective tissue disorder
  • Marfan syndrome

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine
  • Pediatrics, Perinatology, and Child Health

Cite this

Valve-sparing aortic root replacement in Marfan syndrome. / Cameron, Duke E.; Vricella, Luca.

In: Seminars in thoracic and cardiovascular surgery. Pediatric cardiac surgery annual, Vol. 8, No. 1, 2005, p. 103-111.

Research output: Contribution to journalArticle

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