Comparison of aortic dissection in patients with and without Marfan's syndrome (results from the International Registry of Aortic Dissection)

doi:10.1016/j.amjcard.2004.04.049

The American Journal of Cardiology

Volume 94, Issue 3, 1 August 2004, Pages 400-402

https://doi.org/10.1016/j.amjcard.2004.04.049 Get rights and content

Abstract

Among 1,049 patients diagnosed with aortic dissection in a multinational registry, patients with Marfan's syndrome were typically younger and had unique presentations. Despite their younger age at presentation, patients with Marfan's syndrome and aortic dissection had a high mortality rate, similar to patients without Marfan's syndrome, an older patient cohort. Our data support the importance of aneurysm surveillance and prophylactic surgical intervention for patients with Marfan's syndrome to potentially reduce the risk of mortality.

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Cited by (73)

Acute type B aortic dissection
2024, Surgery (United Kingdom)
Aortic dissection is infrequent in everyday practice; however, it can result in life-threatening complications and causes more deaths each year in the UK than road traffic collisions. It is one of the family of acute aortic syndromes, which includes penetrating aortic ulcer (PAU) and intramural haematoma (IMH). Type A aortic dissections involve the ascending aorta and arch and almost invariably require prompt surgical treatment due to exceptionally high early 48-hour mortality without surgery. Many type B aortic dissections (TBAD) are not complicated at presentation and can be treated conservatively in high dependency and discharged without intervention. Complicated aortic dissection requires intervention, often with a thoracic endovascular aortic repair (TEVAR). The survival after TBAD is higher than type A dissection that invariably requires emergency operative intervention, with 65% of patients surviving to 1 year. Following acute aortic syndrome, best medical therapy involves tight blood pressure and heart rate control using beta-blockers or calcium channel blockers. Statin therapy may be of benefit. Regular cross-sectional imaging surveillance is important to detect late complications such as aortic dilatation. In type B aortic dissection, aortic dilatation is a common cause of late rupture with only 50% of patients surviving after 5 years without intervention. One of the most important questions at present is whether people at high risk of further aortic dilatation can be identified and intervened on early to prevent these late complications?
Twenty-five years of observations from the International Registry of Acute Aortic Dissection (IRAD) and its impact on the cardiovascular scientific community
2023, Journal of Thoracic and Cardiovascular Surgery
The International Registry of Acute Aortic Dissection (IRAD) celebrated its 25th anniversary in January 2021. This study evaluated IRAD's role in promoting the understanding and management of acute aortic dissection (AD) over these years.
IRAD studies were identified, analyzed, and ranked according to their citations per year (c/y) to determine the most-cited IRAD studies and topics. A systematic search of the literature identified cardiovascular guidelines on the diagnosis and management of acute AD. Consequently, IRAD's presence and impact were quantified using these documents.
Ninety-seven IRAD studies were identified, of which 82 obtained more than 10 cumulative citations. The median c/y index was 7.33 (25th-75th percentile, 4.01-16.65). Forty-two studies had a greater than median c/y index and were considered most impactful. Of these studies, most investigated both type A and type B AD (n = 17, 40.5%) and short-term outcomes (n = 26, 61.9%). Nineteen guideline documents were identified from 26 cardiovascular societies located in Northern America, Europe, and Japan. Sixty-nine IRAD studies were cited by these guidelines, including 38 of the 42 most-impactful IRAD studies. Among them, partial thrombosis of the false lumen as a predictor of postdischarge mortality and aortic diameters as a predictor of type A occurrence were determined as most-impactful specific IRAD topics by their c/y index.
IRAD has had and continues to have an important role in providing observations, credible knowledge, and research questions to improve the outcomes of patients with acute AD.
Acute type B aortic dissection
2021, Surgery (United Kingdom)
Aortic dissection is infrequent in everyday practice; however, it can result in life-threatening complications and causes more deaths each year in the UK than road traffic collisions. It is one of the family of acute aortic syndromes, which includes penetrating aortic ulcer (PAU) and intramural haematoma (IMH). Type A aortic dissections involve the ascending aorta and arch and almost invariably require prompt surgical treatment due to exceptionally high early 48-hour mortality without surgery. Many type B aortic dissections (TBAD) are not complicated at presentation and can be treated conservatively in high dependency and discharged without intervention. Complicated aortic dissection requires intervention, often with a thoracic endovascular aortic repair (TEVAR). The survival after TBAD is higher than type A dissection that invariably requires emergency operative intervention, with 65% of patients surviving to 1 year. Following acute aortic syndrome, best medical therapy involves tight blood pressure and heart rate control using beta-blockers or calcium channel blockers. Statin therapy may be of benefit. Regular cross-sectional imaging surveillance is important to detect late complications such as aortic dilatation. In type B aortic dissection, aortic dilatation is a common cause of late rupture with only 50% of patients surviving after 5 years without intervention. One of the most important questions at present is whether people at high risk of further aortic dilatation can be identified and intervened on early to prevent these late complications?
A 10-year study of surgical results of descending aorta aneurysm repair following chronic aortic dissection
2020, Journal of Vascular Surgery
Patients presenting with descending aortic aneurysms developing after aortic dissection often undergo continued aortic expansion which may require operative interventions to address the risk of aortic rupture. In light of the current advances in various treatment options, including endovascular approaches, we analyzed our experience with open surgical repair (OSR) of aneurysms of the descending aorta following aortic dissection.
Patients who underwent open repair for aneurysmal changes of the descending aorta after chronic dissection were retrospectively studied. The 30-day operative mortality rate, midterm survival, and major complications were analyzed. Patients were divided into two categories; primary chronic type B aortic dissection and remnant repaired type A aortic dissection (RTAAD).
There were 149 patients with enlargement of the descending thoracic aorta developing after aortic dissection. Of these, 49 patients had medical management, while the remaining 100 patients received OSR. These patients were included in the present analysis. The 30-day mortality and permanent paraplegia rates were 9% and 4%, respectively. The 1-, 3-, and 5-year survival rates were 83%, 80.9%, and 76.1%, respectively. The 1- and 5-year survival rates between the primary chronic type B aortic dissection and remnant RTAAD groups showed no significant between-group differences at 86.7% and 84.3%, and 80% and 71.3%, respectively (P = .289). The overall outcomes of other complications such as renal injury, bleeding reoperation, and extracorporeal membrane oxygenation support showed no significant between-group differences, including an insignificantly higher neurologic complication rate in the remnant RTAAD group. The survival rate in patients with Marfan syndrome was significantly higher than in the patients without Marfan syndrome (P = .033).
OSR for descending aortic aneurysms developing after chronic aortic dissection showed good early and mid- to long-term outcomes, with acceptably low complication rates. OSR for descending aortic aneurysm after chronic aortic dissection associated with Marfan syndrome also showed good early and mid- to long-term outcomes.
Presentation, surgical intervention, and long-term survival in patients with Marfan syndrome
2020, Journal of Vascular Surgery
Patients with Marfan syndrome (MFS) often present with acute catastrophic aortic events at a young age and have a shortened life span. This study examines the impact of presentation and demographics on late survival in patients with MFS.
Adults with confirmed MFS in our thoracic aortic center dataset were identified and statistical analysis performed to identify the incidence and predictors of aortic interventions and late mortality.
We identified 301 patients with a MFS initial diagnosis at age 17 years (interquartile range, 4-30 years) with presentation into our thoracic aortic center at 21 years (interquartile range, 8-34 years). The average follow-up in our center was 10 ± 10 years. Clinical features were 41% male, 86% white race, coronary artery disease 28%, hypertension 40%, peripheral vascular disease 19%, and anti-impulse agent in 51% (β-blocker, angiotensin-converting enzyme inhibitor/angiotensin receptor blocker, calcium channel blocker). Distribution of operative aortic pathology was isolated to the ascending aorta (70%) and descending aorta (8%). One hundred seventy-eight patients (59%) required primary aortic surgery (36% emergent). Primary procedures were cardiac (aortic valve/root) in nature in 94%. Seventy-four patients (42%) required multiple aortic procedures at a mean of 9.2 ± 6.9 years, involving the thoracoabdominal aorta in 65%, thoracic aorta in 37%, and abdominal aorta in 21%. Patients who required multiple aortic procedures were more likely (P < .05) to have coronary artery disease (50% vs 30%), and peripheral vascular disease (43% vs 18%). Multiple aortic procedures were also more likely (P < .05) in patients who developed de novo distal dissection (14% vs 0%), had prior dissection (47% vs 18%), or unknown MFS at the time of the initial procedure (27% vs 63%). Multivariable analysis identified prior dissection as an independent predictor of need for emergent surgery (odds ratio, 13.20; 95% confidence interval, 4.64-37.30; P < .05), as well as additional aortic surgery (odds ratio, 4.42; 95% confidence interval, 1.87-10.50; P < .05). Kaplan-Meier analysis showed similar 10-year survival with or without aortic interventions (82% with vs 89% without; P = .08). Late survival was decreased in patients undergoing emergent initial procedures (66% vs 89%; P < .01), as well as those undergoing multiple operations (74% vs 86%; P = .03).
These data indicate that, in the modern era, the mode of presentation and need for multiple procedures have a detrimental impact on late survival. Additionally, the presence of acute or chronic dissection predicts the need for additional aortic procedures during follow-up.
An axillary-subclavian artery aneurysm in pre-rupture revealing Marfan syndrome
2019, JMV-Journal de Medecine Vasculaire
Le syndrome de Marfan est une maladie génétique du tissu conjonctif de transmission autosomique dominante, caractérisée par un grand polymorphisme clinique et dont le pronostic dépend des complications cardiovasculaires associées, dominées par les atteintes aortiques proximales. Les anévrismes artériels périphériques sont rares au cours du syndrome de Marfan et sont exceptionnellement révélateurs de la maladie. Nous rapportons le cas d’un volumineux anévrisme de l’artère axillo-sous-clavière en pré-rupture révélant un nouveau cas de syndrome de Marfan. L’intervention chirurgicale a consisté en une mise à plat de l’anévrisme et rétablissement de la continuité artérielle par greffe veineuse ; les suites opératoires étaient simples.
Marfan syndrome is an autosomal dominant disorder of connective tissue which has many clinical symptoms and whose prognosis depends on associated cardiovascular complications, dominated by proximal aortic disorders. Peripheral arterial aneurysms are rare during Marfan syndrome and are exceptionally indicative of the disease. We report the case of a large aneurysm of the axillary-subclavian artery in pre-rupture revealing a new case of Marfan syndrome. Treatment consisted in surgical repair by resection of the aneurysm and performing a venous bypass graft; the postoperative course was uneventful.

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^☆: This study was supported by a grant from the Varbedian Fund for Aortic Research, University of Michigan, Ann Arbor, Michigan.

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Comparison of aortic dissection in patients with and without Marfan's syndrome (results from the International Registry of Aortic Dissection)☆

Abstract

J Am Coll Cardiol

Am J Cardiol

Ann Thorac Surg

J Am Coll Cardiol

Am Heart J

Am J Cardiol