Prognostic significance of the pattern of aortic root dilation in the Marfan syndrome

Roman, Mary J.; Rosen, Stacey E.; Kramer-Fox, Randi; Devereux, Richard B.

doi:10.1016/0735-1097(93)90559-j

Cited by 229 publications

(130 citation statements)

References 21 publications

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“…5,18,19 Increase in aortic diameter with age is usually invoked to explain progressively increasing prominence of the aortic knuckle in routine chest radiographs. Autopsy studies have shown a clear-cut increase in aortic surface area with age 20 (Figure 4), whereas cross-sectional studies of aortic diameter by angiography 21 and ultrasound [22][23][24][25][26] have shown lesser, but still definite, increase with age, except in the most proximal part of the ascending aorta. Gender differences are largely explicable on the basis of smaller body size in women.…”

Section: T He October 2004 High Blood Pressure Researchmentioning

confidence: 99%

Aortic Diameter, Aortic Stiffness, and Wave Reflection Increase With Age and Isolated Systolic Hypertension

O’Rourke

Nichols²

2005

Hypertension

479

359

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Section: T He October 2004 High Blood Pressure Researchmentioning

confidence: 99%

Aortic Diameter, Aortic Stiffness, and Wave Reflection Increase With Age and Isolated Systolic Hypertension

O’Rourke

Nichols²

2005

Hypertension

479

359

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“…LV, left ventricle; LA, left atrium; Ao, aorta. After Roman et al 17 relatives), and in 66% (63% of probands, 67% of relatives) it could be unequivocally excluded. Ten patients from five families had FTAA, on the basis of a personal or family history or aortic root dilatation or dissection in the absence of other Marfan syndrome features.…”

Section: Evaluating the Use Of The Ghent Criteriamentioning

confidence: 99%

“…The cardiovascular assessment requires measurement of the aortic diameter at the Sinuses of Valsalva, usually by transthoracic echocardiography (Figure 4), and comparison with normal values based on age and body surface area, calculated from height and weight. 14,17 Other imaging techniques such as transoesophageal echocardiography or MRI scanning may be helpful in some cases. Assessment of the skeletal system should include pelvic X-ray to detect protrusion acetabulae, 18 if a positive finding would provide system involvement, or change skeletal system involvement to a major criterion, such that a positive Marfan diagnosis could then be made in conjunction with other system findings.…”

Section: In Briefmentioning

confidence: 99%

Marfan syndrome: clinical diagnosis and management

2007

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In association withMarfan syndrome: clinical diagnosis and management Marfan syndrome is a multisystem connective tissue disorder usually associated with mutation in fibrillin, and occasionally with mutation in TGFBR1 or 2. The clinical diagnosis is made using the Ghent nosology, which will unequivocally diagnose or exclude Marfan syndrome in 86% of cases. Use of a care pathway can help implementation of the nosology in the clinic. The penetrance of some features is age dependent, so the nosology must be used with caution in children. Molecular testing may be helpful in this context. The nosology cannot be used in families with isolated aortic dissection, or with related conditions such as Loeys -Dietz syndrome, although it may help identify families for further diagnostic evaluation because they do not fulfill the nosology, despite a history of aneurysm. Prophylactic medical (eg b-blockade) and surgical intervention is important in reducing the cardiovascular complications of Marfan syndrome. Musculoskeletal symptoms are common, although the pathophysiology is less clear -for example, the correlation between dural ectasia and back pain is uncertain. Symptoms in other systems require specialist review such as ophthalmology assessment of refractive errors and ectopia lentis. Pregnancy is a time of increased cardiovascular risk for women with Marfan syndrome, particularly if the aortic root exceeds 4 cm at the start of pregnancy. High-intensity static exercise should be discouraged although low-moderate intensity dynamic exercise may be beneficial. The diagnosis and management of Marfan syndrome requires a multidisciplinary team approach, in view of its multisystem effects and phenotypic variability.

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“…16, 19 MRI and computed tomography were not routinely used. The Japanese Circulation Society recommends an operation for patients with a sinus of Valsalva >5 cm (class IIa, level C) in all cases of Marfan syndrome.…”

Section: Measurement Of Aortic Diameter and Indication For Surgerymentioning

confidence: 99%

Pregnancy-Associated Aortic Dilatation or Dissection in Japanese Women With Marfan Syndrome

Katsuragi

Ueda

Yamanaka

et al. 2011

Circ J

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Background: Aortic dilatation and dissection are severe complications of pregnancy that may cause maternal death. The purpose of the present study was to investigate risk factors for aortic dilatation or dissection in pregnant Japanese women with Marfan syndrome. Methods and Results:A total of 28 patients with Marfan syndrome were investigated retrospectively during pregnancy and after delivery at 1 institution. These patients were divided into 2 groups: those who experienced aortic dilatation or dissection (group D, n=11) and those who did not (group ND, n=17). In group D, aortic dilatation or dissection occurred in 7 cases during pregnancy (2 in the 2 nd trimester, 5 in the 3 rd trimester) and 4 cases after birth. The 2 cases in the 2 nd trimester involved aortic dilatation >60 mm and those patients underwent hemiarch replacement and a David operation, respectively. Delivery by cesarean section (64% vs. 18%, P<0.05), sinus of Valsalva ≥40 mm (86% vs. 21%, P<0.05), aortic size index (size of sinus of Valsalva/body surface area) ≥25 mm/m 2 (7/7, 100% vs. 0/14, 0%, P<0.

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Prognostic significance of the pattern of aortic root dilation in the Marfan syndrome

Abstract: The present study indicates that generalized aortic root dilation is a potent marker of an increased risk for subsequent aortic complications in Marfan syndrome.

Cited by 229 publications

References 21 publications

Aortic Diameter, Aortic Stiffness, and Wave Reflection Increase With Age and Isolated Systolic Hypertension

Aortic Diameter, Aortic Stiffness, and Wave Reflection Increase With Age and Isolated Systolic Hypertension

Marfan syndrome: clinical diagnosis and management

Pregnancy-Associated Aortic Dilatation or Dissection in Japanese Women With Marfan Syndrome

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