Surgery for patients with Marfan syndrome with type A dissection involving the aortic arch using total arch replacement combined with stented elephant trunk implantation: the acute versus the chronic.

OBJECTIVE

The optimal surgical repair for patients with Marfan syndrome with type A dissection involving the aortic arch is controversial. We retrospectively reviewed our experience of total arch replacement combined with stented elephant trunk implantation for patients with Marfan syndrome with type A dissection.

METHODS

Between April 2003 and September 2008, 44 patients with Marfan syndrome (acute = 19, chronic = 25) with type A dissection underwent this procedure. Postoperative computed tomography was used to evaluate thrombosis and absorption of the residual false lumen.

RESULTS

In-hospital mortality was 4.55% (2/44) (acute = 0%, 0/19; chronic = 8.00%, 2/25) and follow-up death rate was 4.76% (2/42) (acute = 5.26%, 1/19; chronic = 4.35%, 1/23) during a mean follow-up of 38 ± 17 months. One patient (5.26%, 1/19) with chronic dissection underwent thoracoabdominal aortic replacement 7 months after surgery. Injury to the spinal cord and visceral ischemia were not observed during follow-up. Obliteration of the false lumen around the stented elephant trunk was observed in 76.2% of patients (32/42) (acute = 84.2%, 16/19; chronic = 69.6%, 16/23) as demonstrated by postoperative computed tomography. The distal end of the stent-graft entering the false lumen was observed in 4 patients (21.1%, 4/19) with acute dissection.

CONCLUSIONS

The procedure was a suitable alternative to patients with Marfan syndrome with chronic type A dissection. However, more attention should be paid to patients with Marfan syndrome with acute dissection caused by the fragile dissecting membrane. If this procedure was adopted in patients with Marfan syndrome with acute type A dissection, an entry adjacent to the distal end of the surgical stent-graft, a small true lumen, or an extremely tortuous morphology of the false lumen aorta should be excluded.