Presentation and outcome of Marfan's syndrome patients with dissection and thoraco-abdominal aortic aneurysm

Abstract

Background: In Marfan's syndrome, there is a paucity of data regarding intervention criteria for surgery of the dissected thoraco-abdominal aorta. Methods: A retrospective analysis of 22 Marfan's patients with distal aortic dissection managed between September 1999 and April 2006 was performed. Serial diameters and linear expansion rates were calculated from imaging studies and the outcome of intervention was analysed.Results: There were 14/22 male patients (median age 38 years), and 18 had prior aortic surgery. Surgery was recommended in 20 patients and undertaken in 19 (1 died prior to operation). Of the operated patients, 2 presented with rupture, 2 with airway obstruction, 1 with intermittent paraplegia and 14 underwent planned surgery for increased expansion rate or pain. All patients had residual type A or chronic type B dissection. The median aortic dimension at surgery was 6.7 cm (interquartile range (IQR) 5.5-8.2). The preoperative mean expansion rate increased from 0.5 cm/year to 1.7 cm/year ( p < 0.001), prior to operation. Fifteen patients underwent Crawford Extent II, two underwent Extent I and two underwent Extent III repair. Profound hypothermia and CSF drainage was used in 16 and 18 patients, respectively. There was no early mortality, paraplegia or renal failure. At a median postoperative follow-up of 56 months (range 6-86), the survival of the operated cohort was 90%. Conclusions: Thoraco-abdominal aortic aneurysm repair in Marfan's syndrome can be performed with good outcomes. Intervention should be based on size or accelerated expansion. Any role of endovascular management needs careful consideration. #

Endovascular treatment of type B dissection in patients with marfan syndrome: Mid‐term outcomes and aortic remodeling

“…These results confirm the data previously published by Gott et al . Mid‐term outcomes of MFS patients with distal dissections and prior surgery has been described by Kalkat et al . These authors did not have 30‐day mortality, paraplegia or paraparesis after surgical repair.…”

Successful Kidney Transplantation for End-Stage Renal Disease in Marfan's Syndrome

“…The incidence of acute renal injury after thoracoabdominal aortic aneurysm and aortic dissection repair has been reported to be about 30%, with renal failure that requires hemodialysis occurring in about 3% of patients with or without Marfan's syndrome [6]. In terms of kidney transplantation, a report of successful preservation of threatened renal function by renal autotransplantation, following dissection of the descending aorta, has been published in a patient with Marfan's syndrome [7].…”

Descending thoracic aortic aneurysm in a female patient with Marfan syndrome

“…Chronické aneurysma s disekcí a hrozící rupturou je indikováno k emergentní operaci. 5 Nepravé výdutě (pseudoaneurysmata) jsou vždy indikovány k operaci co nejdříve pro veliké riziko ruptury.…”