Spectrum of Aortic Operations in 300 Patients With Confirmed or Suspected Marfan Syndrome

LeMaire, Scott A.; Carter, Stacey A.; Volguina, Irina V.; Laux, Anne T.; Milewicz, Dianna M.; Borsato, Garry W.; Cheung, Catherine K.; Bozinovski, John; Markesino, Jennifer M.; Vaughn, William K.; Coselli, Joseph S.

doi:10.1016/j.athoracsur.2006.01.070

Cited by 114 publications

(83 citation statements)

References 29 publications

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“…However, such outcomes may not be generalisable to the whole cardiovascular surgical community [13] and it is axiomatic that the institutional risk of surgery remains less than the risk of rupture. LeMaire et al reported a 2% 30-day mortality, a 1% stroke incidence and a 5% paraplegia/ paraparesis incidence in 81 confirmed MFS thoraco-abdominal repairs [10], and the outcomes in the current series are comparable. However, despite this, with a small denominator, subsequent repeatability and quality control can be less assured.…”

Section: Discussionsupporting

confidence: 71%

“…Large North American aortic surgery centres recommend elective intervention in chronic MFS dissections when the diameter exceeds 4.5-5.5 cm [10]. The outstanding outcomes from such centres may justify this approach.…”

Section: Discussionmentioning

confidence: 99%

“…The short axis aortic diameter of the maximally dilated aortic segment was measured with a previously validated calliper method [12]. This allowed calculation of a linear expansion rate per annum (ER) and percentage expansion rate (%ER) [10]. In order to describe the relative contribution of false and true luminae-to-aneurysm growth, we measured the diameter of the true and false luminal channels in a varying plane perpendicular to the central site of the intimal flap at four levels (Level 1, the upper descending aorta; Level 2, the bifurcation of trachea; Level 3, the lower edge of left atrium and Level 4, origin of coeliac artery).…”

Section: Methodsmentioning

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.…”

Section: Introductionmentioning

confidence: 99%

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

Kalkat

Rahman

Kotidis

et al. 2007

European Journal of Cardio-Thoracic Surgery

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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. #

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Section: Discussionsupporting

confidence: 71%

Section: Discussionmentioning

confidence: 99%

Section: Methodsmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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

Kalkat

Rahman

Kotidis

et al. 2007

European Journal of Cardio-Thoracic Surgery

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“…It is very important to emphasize that there is 30-40% morbidity and mortality in patients who undergo an emergency or urgent surgical procedure; with this in mind, we suggest treating TAA as soon as possible, even because this indication is largely shared, as confirmed by several scientific publications [1][2][3][4].…”

mentioning

confidence: 99%

FORUM EKSPERTÓW Thoraco-abdominal aneurysm repair in Marfan patients

Zanetti¹

2012

kitp

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Outcomes After Endovascular Aortic Intervention in Patients With Connective Tissue Disease

et al. 2023

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ImportanceEndovascular treatment is not recommended for aortic pathologies in patients with connective tissue diseases (CTDs) other than in redo operations and as bridging procedures in emergencies. However, recent developments in endovascular technology may challenge this dogma.ObjectiveTo assess the midterm outcomes of endovascular aortic repair in patients with CTD.Design, Setting, and ParticipantsFor this descriptive retrospective study, data on demographics, interventions, and short-term and midterm outcomes were collected from 18 aortic centers in Europe, Asia, North America, and New Zealand. Patients with CTD who had undergone endovascular aortic repair from 2005 to 2020 were included. Data were analyzed from December 2021 to November 2022.ExposureAll principal endovascular aortic repairs, including redo surgery and complex repairs of the aortic arch and visceral aorta.Main Outcomes and MeasuresShort-term and midterm survival, rates of secondary procedures, and conversion to open repair.ResultsIn total, 171 patients were included: 142 with Marfan syndrome, 17 with Loeys-Dietz syndrome, and 12 with vascular Ehlers-Danlos syndrome (vEDS). Median (IQR) age was 49.9 years (37.9-59.0), and 107 patients (62.6%) were male. One hundred fifty-two (88.9%) were treated for aortic dissections and 19 (11.1%) for degenerative aneurysms. One hundred thirty-six patients (79.5%) had undergone open aortic surgery before the index endovascular repair. In 74 patients (43.3%), arch and/or visceral branches were included in the repair. Primary technical success was achieved in 168 patients (98.2%), and 30-day mortality was 2.9% (5 patients). Survival at 1 and 5 years was 96.2% and 80.6% for Marfan syndrome, 93.8% and 85.2% for Loeys-Dietz syndrome, and 75.0% and 43.8% for vEDS, respectively. After a median (IQR) follow-up of 4.7 years (1.9-9.2), 91 patients (53.2%) had undergone secondary procedures, of which 14 (8.2%) were open conversions.Conclusions and RelevanceThis study found that endovascular aortic interventions, including redo procedures and complex repairs of the aortic arch and visceral aorta, in patients with CTD had a high rate of early technical success, low perioperative mortality, and a midterm survival rate comparable with reports of open aortic surgery in patients with CTD. The rate of secondary procedures was high, but few patients required conversion to open repair. Improvements in devices and techniques, as well as ongoing follow-up, may result in endovascular treatment for patients with CTD being included in guideline recommendations.

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Spectrum of Aortic Operations in 300 Patients With Confirmed or Suspected Marfan Syndrome

Cited by 114 publications

References 29 publications

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

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

FORUM EKSPERTÓW Thoraco-abdominal aneurysm repair in Marfan patients

Outcomes After Endovascular Aortic Intervention in Patients With Connective Tissue Disease

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