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DOI: 10.1055/s-2004-816752
Aortic root surgery does not prevent adverse outcome and reoperations on the downstream aorta in marfan patients
Objectives: Anulo-aortic ectasia represents the most common cardiovascular manifestation in Marfan patients. However, a substantial proportion may suffer from significant pathologic changes in the downstream aorta following aortic root repair or replacement.
Material and Methods: Between 1990 and 2003 a total of 72 patients with Marfan syndrome diagnosed by using the Gent nosology and/or mutation analysis received initial surgery (aortic root operation in 69, mitral valve repair in 2, combined operation in 1) at our institution. 21 patients presented with acute type A aortic dissection (29.1%) and 4 female patients presented dissection during pregnancy. Composite graft replacement was performed in 61, supracoronary graft in 2 and aortic valve sparing root repair in 7 patients. All patients underwent close clinical and imaging follow-up in a specialized outpatient consultation.
Results: During a mean follow-up interval of 5±1.8 years, 14 patients (20.5%) underwent a total of 25 reoperations on the proximal (n=2) and on the downstream (n=23) aorta. 8 patients had one and 6 patients had up to 4 reoperations; 13/14 had chronic aortic dissection. There was no hospital mortality and no major cardiac or neurologic morbidity. In addition, 2 patients suffered from acute type B dissection and 3 patients died unexpected, all together following uncomplicated aortic root operation.
Conclusions: This survey demonstrates that unexpected fatal outcome may appear in the downstream aorta following uncomplicated elective aortic root surgery. The necessity for reoperations is significantly higher in those patients who presented initially with acute type A aortic dissection. A close follow-up of all Marfan patients is necessary to detect asymptomatic changes requiring surgery. Complex redo-operations can be performed with a low perioperative risk.