Abstract: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 perf… Show more
“…Regarding distal aortic dissection in patients with MFS, Carrel et al reported that 14 of 71 (20.5%) patients with MFS who had undergone surgery for annulo-aortic ectasia subsequently underwent a total of 27 aortic reoperations during a mean follow-up period of 5.2 years. 12 More importantly, initial aortic root surgery was performed for AAAD in 13 of the 14 patients. The long-term outcome of aortic root replacement in MFS patients differs between those who undergo surgery for aortic dissection and those without dissection because patients with MFS tend to require multi- ple surgical interventions if chronic dissection remains in the native distal aorta.…”
Section: Discussionmentioning
confidence: 99%
“…6 Aortic dissection in this subgroup is most often related to an underlying connective tissue disease, such as Marfan syndrome (MFS); 6,7 however, some younger patients do not have any underlying connective tissue disease. Clinical outcomes among patients with MFS and acute type A aortic dissection (AAAD) are generally known, [8][9][10][11][12] but there are few reports based solely on age rather than etiology that describe patient characteristics and treatment outcomes among young patients with AAAD.…”
Background: Acute type A aortic dissection (AAAD) is rare in young people. The early- and long-term outcomes after surgery for AAAD in patients aged ≤45 years was investigated.
Methods and Results:Subjects were 355 patients who had undergone emergency surgery for AAAD. The patients were grouped as those aged ≤45 years (n=30; mean age, 38.3 years; younger group) and those aged >45 years (n=325; mean age, 65.3 years; older group). Clinical and prognostic variables were compared between the groups. Male sex, Marfan syndrome, and severe aortic regurgitation were more prevalent in the younger group. In-hospital mortality (16.7% vs. 8.6%, P=0.15) and postoperative patency of the distal aorta (90.8% vs. 59.1%, P<0.01) were more frequent in the younger group. The leading causes of late death were aortic rupture in the younger group (75.0%) and malignancy in the older group (27.5%). Although actuarial survival at 10 years was similar (64.5% vs. 62.5%), freedom from aortic reoperation at 10 years was decreased in the younger group (49.4% vs. 85.0%, P=0.012). A distal aorta >45 mm (P<0.001), Marfan syndrome (P<0.01), and age ≤45 years (P=0.045) were shown to be independent risk factors for reoperation.
Conclusions:Early-and long-term surgical outcomes are not better for patients ≤45 years, and the risk for reoperation is high in this group. Careful follow up is important in young patients with AAAD. (Circ J 2011; 75: 2135 - 2143
“…Regarding distal aortic dissection in patients with MFS, Carrel et al reported that 14 of 71 (20.5%) patients with MFS who had undergone surgery for annulo-aortic ectasia subsequently underwent a total of 27 aortic reoperations during a mean follow-up period of 5.2 years. 12 More importantly, initial aortic root surgery was performed for AAAD in 13 of the 14 patients. The long-term outcome of aortic root replacement in MFS patients differs between those who undergo surgery for aortic dissection and those without dissection because patients with MFS tend to require multi- ple surgical interventions if chronic dissection remains in the native distal aorta.…”
Section: Discussionmentioning
confidence: 99%
“…6 Aortic dissection in this subgroup is most often related to an underlying connective tissue disease, such as Marfan syndrome (MFS); 6,7 however, some younger patients do not have any underlying connective tissue disease. Clinical outcomes among patients with MFS and acute type A aortic dissection (AAAD) are generally known, [8][9][10][11][12] but there are few reports based solely on age rather than etiology that describe patient characteristics and treatment outcomes among young patients with AAAD.…”
Background: Acute type A aortic dissection (AAAD) is rare in young people. The early- and long-term outcomes after surgery for AAAD in patients aged ≤45 years was investigated.
Methods and Results:Subjects were 355 patients who had undergone emergency surgery for AAAD. The patients were grouped as those aged ≤45 years (n=30; mean age, 38.3 years; younger group) and those aged >45 years (n=325; mean age, 65.3 years; older group). Clinical and prognostic variables were compared between the groups. Male sex, Marfan syndrome, and severe aortic regurgitation were more prevalent in the younger group. In-hospital mortality (16.7% vs. 8.6%, P=0.15) and postoperative patency of the distal aorta (90.8% vs. 59.1%, P<0.01) were more frequent in the younger group. The leading causes of late death were aortic rupture in the younger group (75.0%) and malignancy in the older group (27.5%). Although actuarial survival at 10 years was similar (64.5% vs. 62.5%), freedom from aortic reoperation at 10 years was decreased in the younger group (49.4% vs. 85.0%, P=0.012). A distal aorta >45 mm (P<0.001), Marfan syndrome (P<0.01), and age ≤45 years (P=0.045) were shown to be independent risk factors for reoperation.
Conclusions:Early-and long-term surgical outcomes are not better for patients ≤45 years, and the risk for reoperation is high in this group. Careful follow up is important in young patients with AAAD. (Circ J 2011; 75: 2135 - 2143
“…However, following proximal aortic surgery, particularly in the presence of chronic dissection, the risk of aneurysmal dilatation of the distal aorta is substantial. Distal aortic complications are a primary cause of late death following root surgery [3,7], and 5-35% of MFS patients require further surgery depending upon the extent of previous surgery and the rigor of follow-up [8,19]. Risk factors for late reoperations are acute or chronic dissection at the time of initial surgery, aneurysms involving major peripheral arteries, hypertension and a smoking history [20].…”
Section: Discussionmentioning
confidence: 99%
“…1). While there is abundant literature regarding proximal aortic procedures in MFS, there are little data on the progression and management of distal aortic complications [4,5,[8][9][10]. In this study, we report a consecutive series of MFS patients with distal dissection.…”
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. #
“…After surgery, many patients need second operations for aneurysms or dissections distal from the aortic root (figure 3). 8,9 In fact, the Marfan syndrome may affect any vessel in which elastin fibres are an important structural component of its wall. This applies to the entire aorta, although elastin is found in diminishing quantities from its proximal to its distal part.…”
Prolonged survival of patients with Marfan syndrome after aortic root replacement has led to an increased number of patients with aortic complications beyond the root. Elective replacement of the aortic root removes the most important predilection site for aneurysms, but the distal aorta remains at risk. Predictors for aortic growth and adverse events in the distal aorta include aortic diameter, aortic distensiblity, previous aortic root replacement, hypertension and aortic regurgitation. After aortic dissection, the initial false lumen diameter is an independent predictor for late aneurysm formation.
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