JACC Case Rep. 2026 Mar 18:107403. doi: 10.1016/j.jaccas.2026.107403. Online ahead of print.
ABSTRACT
BACKGROUND: Marfan syndrome-associated aortic root aneurysms carry a high risk of dissection and sudden death, supporting guideline-directed prophylactic surgery at ≥50 mm.
CASE SUMMARY: A 34-year-old man with Marfan syndrome and a strong family history was diagnosed at age 22 years with a 63-mm aortic root aneurysm and moderate aortic regurgitation. He declined recommended Bentall surgery and was lost to follow-up for 12 years. He re-presented with dyspnea and palpitations. Imaging showed a 111 × 108 mm aortic root aneurysm with severe aortic regurgitation and atrial fibrillation. He underwent successful modified Bentall surgery with mechanical valve replacement and AlboGraft conduit and remained asymptomatic at 5 months.
DISCUSSION: Extreme late presentation markedly increases surgical complexity and preventable morbidity.
TAKE-HOME MESSAGES: Extreme late presentation of Marfan aortopathy transforms a standardized prophylactic operation into a technically demanding procedure with significant clinical consequences, even when repair is successful. Delayed intervention permits progression to advanced disease with preventable morbidity, underscoring the importance of timely, guideline-directed management.
PMID:41848446 | DOI:10.1016/j.jaccas.2026.107403