Aortic events in a nationwide Marfan syndrome cohort

Groth, Kristian A.; Stochholm, Kirstine; Hove, Hanne; Kyhl, Kasper; Gregersen, Pernille A; Vejlstrup, Niels; Østergaard, John R.; Gravholt, Claus Højbjerg; Andersen, Niels Holmark

doi:10.1007/s00392-016-1028-3

Cited by 61 publications

(57 citation statements)

References 33 publications

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“…There is a predominance of first time aortic events in men in the age group from 20 to 30 years. Previous studies have also shown that aortic events occur earlier in men than women (Detaint et al, 2010;Faivre et al, 2007;Groth et al, 2017;Rand-Hendriksen et al, 2009). The reason for the gender differences of men undergoing aortic surgery at a younger age, parallel to a shortened life expectancy, is not known.…”

Section: Discussionmentioning

confidence: 96%

“…Other conditions, such as valvular heart disease and myocardial dysfunction with arrhythmias are also known as causes of premature death in MFS (Yetman, Bornemeier, & McCrindle, 2003). Some studies indicate that men experience aortic diseases at younger age than women with MFS (Detaint et al, 2010;Groth et al, 2017;Rand-Hendriksen et al, 2009).…”

Section: Introductionmentioning

confidence: 99%

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Survival, causes of death, and cardiovascular events in patients with Marfan syndrome

Vanem

Geiran

Krohg‐Sørensen

et al. 2018

Molec Gen & Gen Med

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BackgroundTo explore survival, causes of death, and the prevalence of cardiovascular events in a Norwegian Marfan syndrome (MFS) cohort. MFS is a heritable connective tissue disorder associated with reduced life expectancy–primarily due to aortic pathology.MethodsA follow‐up study of 84 MFS adults, initially investigated in 2003–2004. In 2014–2015, 16 were deceased, 47 of 68 survivors consented to new clinical investigations. Analyses of events were performed for 47 survivors and 16 deceased at follow‐up. Standardized mortality ratios (SMR), using the mortality rate of the Norwegian population as reference, were calculated for all 84 and calculated for men and women separately. Causes of death and information on cardiovascular events were retrieved from death certificates and medical records.ResultsStandardized mortality ratios (95% confidence interval): for the whole cohort: 5.24 (3.00–8.51); for men: 8.20 (3.54–16.16); for women: 3.85 (1.66–7.58). Cardiovascular causes were found in 11 of 16 deceased, eight of these related to aortic pathology. Cancer was the cause of death in three patients. At follow‐up, 51% had new cardiovascular events; 59% had undergone aortic surgery. Men experienced aortic events at younger age than women. 32% of the survivors were not followed‐up as recommended.ConclusionLife expectancy is reduced in this MFS cohort compared to the Norwegian population. Cardiovascular complications develop throughout life, particularly aortic pathology, the major cause of death in MFS. Death and aortic pathology seem to occur earlier in men. There is a need to improve follow‐up according to guidelines.

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Section: Discussionmentioning

confidence: 96%

Section: Introductionmentioning

confidence: 99%

Survival, causes of death, and cardiovascular events in patients with Marfan syndrome

Vanem

Geiran

Krohg‐Sørensen

et al. 2018

Molec Gen & Gen Med

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“…However, we found that only about 35% of patients with aortic dissection were female, which was not different for patients with Marfan syndrome compared to those without, despite the higher incidence of pregnancy-associated dissection in Marfan syndrome. Recently published data from a Danish national registry of Marfan syndrome patients confirm that male patients have a 75% higher risk of an aortic event than female patients at any given age (9). Aortic dilatation does not appear to be more prevalent in male than in female adolescents with Marfan syndrome (10).…”

Section: Perspectivesmentioning

confidence: 99%

Aortic dissection in patients with Marfan syndrome based on the IRAD data

Beaufort¹,

Trimarchi²,

Korach³

et al. 2017

Ann. Cardiothorac. Surg.

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Between January 1996 and May 2017, the International Registry on Acute Aortic Dissections has collected information on a total of 6,424 consecutive patients with acute aortic dissection, including 258 individuals with a diagnosis of Marfan syndrome. Patients with Marfan syndrome presented at a significantly younger age compared to patients without Marfan syndrome (38.2±13.2 . 63.0±14.0 years; P<0.001) and in general had fewer comorbidities, although they more frequently had a known aortic aneurysm and history of prior cardiac surgery. We noted significantly larger diameters of the aortic annulus and root in the Marfan syndrome cohort, but no larger diameters more distally. The in-hospital mortality in type A dissection was not significantly different in patients with or without Marfan syndrome, despite the differences in age and comorbidities and the lower incidence of aortic rupture in the Marfan syndrome cohort. In contrast, the in-hospital mortality of Marfan syndrome patients with type B dissection appears to be lower than that of patients without Marfan syndrome. The Marfan syndrome cohort that was treated with open surgery for type B dissection seemed to do especially well, with a 0% mortality rate (n=27). Follow-up data for type A and B dissections combined show an estimated five-year survival rate of 80.1% and an estimated reintervention rate of 55.3% in patients with Marfan syndrome. Such a high rate of reinterventions highlights the need for careful surveillance and treatment for patients with Marfan syndrome surviving the acute phase of aortic dissection.

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“…The first mechanism is the occurrence of spinal cord infarct as a complication of aortic surgery or dissection. Indeed, more than one third of patients with Marfan syndrome will be operated on an aortic event in their life (aortic dissection or preventively in aortic dilation [5]). Even if morbidity during this surgery decreases with novel techniques, spinal cord infarct is still one of their complications [6] and Marfan syndrome is a predictor of its occurrence [7].…”

Section: Discussionmentioning

confidence: 99%

Urinary Disorders and Marfan Syndrome: A Series of 4 Cases

et al. 2017

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Marfan syndrome is a genetic disease responsible for causing cardiovascular, eye and musculoskeletal damages. Urinary disorders are not common. We present 4 cases of chronic urinary tract symptoms, with 2 different pathophysiological processes. Three patients presented with spinal cord infarct following aortic dissection surgery. They were affected by an overactive bladder with detrusor overactivity and detrusor-sphincter dyssynergia. One patient complained of voiding dysfunction, possibly related to dural ectasia. Although a rare outcome, urinary disorders may appear in Marfan syndrome, by the occurrence of surgical complications in aortic surgery or possibility of sacral nerve root compression. If so, medical care is necessary to prevent uro-nephrological complications.

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Aortic events in a nationwide Marfan syndrome cohort

Cited by 61 publications

References 33 publications

Survival, causes of death, and cardiovascular events in patients with Marfan syndrome

Survival, causes of death, and cardiovascular events in patients with Marfan syndrome

Aortic dissection in patients with Marfan syndrome based on the IRAD data

Urinary Disorders and Marfan Syndrome: A Series of 4 Cases

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