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Aortic Root/Valve Management in Patients with Connective Tissue Syndromes - is Limited Resection Justified?
Fabian A. Kari1, Elizabeth H. Stephens2, Bartosz Rylski1, Maximilian Russe3, Matthias Siepe1, Friedhelm Beyersdorf1.
1Heart Center, University of Freiburg, Freiburg, Germany, 2Columbia University New York, New York City, USA, NY, USA, 3University of Freiburg, Freiburg, Germany.

Objectives: The ideal treatment of the aortic root and valve in patients with connective tissue syndromes presenting with aortic root aneurysm or aortic dissection remains subject to debate. Methods: Prospective collection of clinical and functional follow-up data was performed at our institution's connective tissue syndrome-dedicated clinic. Kaplan Meier, LogRank calculations and the Cox proportional Hazards Model were used to identify differences between treatment groups with respect to type of aortic root and valve management. Results: 203 patients with confirmed Marfan-Syndrome (MFS 1, n=189), Loeys Dietz Syndrome (n=9) or Familial Thoracic Aortic Dissection (n=2) were followed clinically. Cumulative clinical follow-up comprised 1028 patient-years. Over a mean follow-up period of 8.1±7.6 years n=130 (65%) patients were in need of an elective aortic aneurysm repair (n=85, 65%) or an emergency (n=45, 35%) aortic procedure for aortic aneurysm or acute/subacute aortic dissection. N=55 (42%) underwent a composite valve graft procedure, n=58 (45%) a David valve-sparing aortic root replacement procedure (n=7 mod. David II, n=51 David I). In n=17 (13%) patients the aortic root was not fully replaced during the initial procedure. This was done more often in the setting of a dissection, with the root either being unaffected by the dissection or resuspended. An untreated aortic root at the time of primary procedure resulted in freedom from reoperation of 34% (9-61%) at 15 years vs. 87% (74-92%) for a procedure including root replacement (p=0.039). Freedom from root or valve reoperation after the David procedure was 95% (79-99%) and 88% (65-96%) at 4 and 8 years. Conclusions: Prophylactic surgery using a valve-sparing technique can be considered the best root treatment for connective tissue disorders currently available. Aortic root replacement should be performed during acute aortic dissection repair if a connective tissue disorder is known or likely even in the setting of a non-dissected root.

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