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OBJECTIVES: Anulo-aortic ectasia represents the most common cardiovascular manifestation requiring surgery in Marfan patients. Aim of this report was to analyze the type of presentation and the incidence of cardiovascular lesions and the clinical follow-up after initial surgery, mainly aortic root repair or replacement. METHODS: Between 1/1990 and 6/2003 a total of 71 patients (mean age 29+/-17 years, 8-65 years) received first surgical treatment at our institution. 69 received root repair or replacement. 22 patients presented with acute aortic dissection (31%), out of them, 3 pregnant females and 1 just after delivery. Composite graft replacement was performed in the majority of patients (61/71, 85%). Aortic valve sparing root repair was performed in 7 patients, supra-coronary graft with refixation of the aortic valve in 2 and replacement of the descending aorta in 1 patient. All patients underwent close clinical and imaging follow-up in a specialized outpatient consultation. RESULTS: During a mean follow-up interval of 5.2+/-1.8 years, 14 patients (20.5%) underwent a total of 27 aortic reoperations. Seven patients had one and 6 patients had up to 4 reoperations; 13/14 patients had chronic aortic dissection. There was no hospital mortality and no major cardiac or neurologic morbidity following reoperation. During follow-up, 2 patients suffered from acute type B dissection following aortic root surgery and 3 patients surprisingly died: 2 from a rupture of a normal-sized descending aorta and one from intracranial hemorrhage. These 5 patients had had uncomplicated primary aortic root operation. CONCLUSIONS: The incidence of reoperations is significantly higher in patients who presented initially with acute type A aortic dissection than in those with dilatation only. In addition, this survey demonstrates that unexpected fatal outcome may appear in the remaining native aorta following uncomplicated elective aortic root surgery, even if the aorta is normal-sized. A close follow-up of all Marfan patients is necessary to detect asymptomatic changes requiring surgery because complex elective redo-operations can be performed with a very low perioperative risk.