Effect of beta-adrenergic blockade on aortic root rate of dilation in the Marfan syndrome

Salim, Mubadda A.; Alpert, Bruce S.; Ward, Jewell C.; Pyeritz, Reed E.

doi:10.1016/0002-9149(94)90762-5

Cited by 129 publications

(88 citation statements)

References 12 publications

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“…19,30 Some patients respond better than others, responders tending to be younger and showing improved aortic distensibility, reduced pulse wave velocity, smaller pre-treatment aortic diameters (less than 4 cm in one study). 19,26,28,30 -32 Poor response may be associated with more extensive elastic fibre degeneration, either due to a more severe mutation or more advanced disease.…”

Section: Cardiovascular System In Marfan Syndromementioning

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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Section: Cardiovascular System In Marfan Syndromementioning

confidence: 99%

Marfan syndrome: clinical diagnosis and management

2007

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“…The study showed an increase of 2.1 ±1.6 mm/year in the non-treated group versus 0.7±1.8 mm/year and 1.1±1.1 mm/year in two groups of patients treated with atenolol or propanolol, all of them with p < 0.05 19 .…”

Section: Discussionmentioning

confidence: 84%

Avaliação clínico-cardiológica e ecocardiográfica, seqüencial, em crianças portadoras da síndrome de Marfan

Lopez¹,

Pérez²,

Moisés³

et al. 2005

Arq. Bras. Cardiol.

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OBJECTIVETo describe the clinical cardiac manifestations and temporal evolution of Marfan syndrome in children; to estimate the incidence of annuloaortic ectasia and mitral valve prolapse; and to evaluate tolerability and efficacy of beta-blockers in these patients. METHODSDuring one year, 21 children with Marfan syndrome underwent serial clinical and echocardiographic examinations. Echocardiograms assessed: the presence of mitral valve prolapse, aortic root diameter, mitral and aortic valves regurgitation, and aortic enlargement during beta-blocker therapy. Eleven patients had two measurements of the aortic root taken one year apart. RESULTSThe children were asymptomatic throughout the study. Mitral prolapse was found in 11 (52%) children. Annuloaortic ectasia occurred in 16 (76%) patients and found to be mild in 42.8%, moderate in 9.5%, and severe in 23.8%. One of these patients underwent aortic valve replacement and repair of the ascending aorta by the Bentall-De Bono technique, with good results. Heart rate decreased by 13.6% (from 85 to 73 bpm; p < 0.009) with the use of beta-blockers; however, aortic root diameter increased by 1.4 mm/year (p<0.02). One child could not be given beta-blockers due to bronchial asthma, and no significant side effects were observed in the remaining children, including one who also had bronchial asthma. Original Article Original Article Original Article Original Article Original ArticleOriginal Article CONCLUSIONThe children remained asymptomatic throughout the study, the use of beta-blockers led to a significant decrease in heart rate, and no significant adverse effects were observed. Contrary to the literature, incidence of annuloaortic ectasia was high among the study population, greater than that of mitral valve prolapse, even during beta-blocker therapy.

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“…14 The treatment has been shown to be effective in both adults and children. [15][16][17] . Beta blockers inhibit chronotropic, inotropic, and vasodilatory responses to betaadrenergic stimulation thus reducing stress on the aorta.…”

Section: Cardiovascular Managementmentioning

confidence: 99%

“…He described an observation of disproportionate long limbs in Gabrielle, a 5-year-old girl. 1 The defect is as a result of mutation in the fibrillin 1 gene on chromosome 15, while other cases may be due to mutation in TGFâR1 or TGFâR2. 2 This abnormality of the protein leads to variable clinical problems particularly affecting the skeletal, ocular, and cardiovascular systems.…”

Section: Introductionmentioning

confidence: 99%