Indications for Bentall Surgery in Marfan Syndrome

Primary Size-Based Indications

The American College of Cardiology recommends that patients with Marfan syndrome undergo Bentall procedure when aortic root diameter reaches ≥5.0 cm, or earlier at ≥4.5 cm when additional risk factors are present, particularly when significant aortic regurgitation coexists or valve-sparing surgery is not feasible 1, 2, 3
The fundamental threshold for aortic root surgery in Marfan syndrome is an external aortic diameter of ≥5.0 cm, which is lower than the 5.5 cm threshold used for non-syndromic patients due to the increased propensity for dissection at smaller diameters 4, 5

Surgery should be performed at diameters <5.0 cm when rapid aortic growth ≥0.3-0.5 cm/year on serial imaging with side-by-side comparison is present 4, 3, 6
The American Heart Association suggests that patients with Marfan syndrome and a family history of aortic dissection, especially if dissection occurred at diameters <5.0 cm, should undergo surgery at diameters <5.0 cm 4, 3, 6
Patients with severe (3-4+) aortic regurgitation requiring intervention should undergo surgery at diameters <5.0 cm 4, 5, 6
Women with Marfan syndrome who desire pregnancy and have an aortic diameter >4.0 cm should undergo surgery before conception 4, 5, 7, 6

The Bentall procedure is preferred when significant aortic valve pathology exists with moderate-to-severe (3-4+) aortic regurgitation that cannot be repaired, or when aortic valve cusps are structurally abnormal and unsuitable for preservation 2, 3
The Valve-Sparing (David) procedure should be considered when aortic valve cusps are structurally normal with minimal or no regurgitation, or when surgery is elective and performed by experienced surgeons in a Multidisciplinary Aortic Team 1, 2, 3

The Valve-Sparing procedure avoids anticoagulation but carries a 6-7% risk of developing moderate AR requiring reoperation 1

Pregnant patients with Marfan syndrome and an aortic diameter >4.0 cm before conception should undergo prophylactic surgery prior to pregnancy 4, 5, 7, 6
Frequent cardiovascular monitoring throughout pregnancy and puerperium is mandatory for all Marfan women 4, 5

Delaying surgery until 5.5 cm, as used in non-Marfan patients, is not recommended; dissection risk increases substantially above 5.0 cm 4, 1, 6
Using internal diameter measurements alone is not recommended; guidelines reference external diameters, and internal measurements underestimate true size 4
Assuming medical therapy eliminates surgical need is incorrect; beta-blockers and ARBs slow but do not prevent aortic growth 4, 5
Forgetting lifelong surveillance of remaining aortic segments post-operatively is a critical pitfall, as arch and descending aorta remain at risk 4, 5