Marfan's syndrome

Judge, Daniel P.; Dietz, Harry C.

doi:10.1016/s0140-6736(05)67789-6

Cited by 1,064 publications

(913 citation statements)

References 107 publications

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“…1 The cardinal features of this disorder involve the ocular, musculoskeletal, and cardiovascular systems. Cardiovascular pathology, including aortic root dilation, aortic dissection, and myxomatous mitral valve changes, is the leading cause of mortality in MFS.…”

Section: Introduction the Marfan Syndrome (Mfs)mentioning

confidence: 99%

Rationale and design of a randomized clinical trial of β-blocker therapy (atenolol) versus angiotensin II receptor blocker therapy (losartan) in individuals with Marfan syndrome

Lacro

Dietz

Wruck

et al. 2007

American Heart Journal

207

161

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Background-Cardiovascular pathology, including aortic root dilation, dissection, and rupture, is the leading cause of mortality in patients with Marfan syndrome (MFS). The maximal aortic root diameter at the sinuses of Valsalva is considered the best predictor of adverse cardiovascular outcome. Although advances in therapy have improved life expectancy, affected individuals continue to suffer cardiovascular morbidity and mortality. Recent studies in a FBN1-targeted mouse model of MFS with aortic pathology similar to that seen in humans showed that treatment with losartan normalized aortic root growth and aortic wall architecture.

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Section: Introduction the Marfan Syndrome (Mfs)mentioning

confidence: 99%

Rationale and design of a randomized clinical trial of β-blocker therapy (atenolol) versus angiotensin II receptor blocker therapy (losartan) in individuals with Marfan syndrome

Lacro

Dietz

Wruck

et al. 2007

American Heart Journal

207

161

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“…1 It is caused by mutation in the fibrillin-1 gene located on chromosome 15. 2 The cardinal features occur in the skeletal, ocular, and cardiovascular systems, but other organs can also be affected, including the skin, lungs, and dura.…”

Section: Résumémentioning

confidence: 99%

“…8 While epidural anesthesia provides more stable hemodynamics, it may be difficult to perform due to scoliosis, and it has been associated with failure. 1,9 We present two cases of patients with Marfan syndrome and associated DE. Both patients presented for elective Cesarean delivery where neuraxial anesthesia was used successfully but performed very differently.…”

Section: Résumémentioning

confidence: 99%

Regional anesthesia in Marfan syndrome, not all dural ectasias are the same: a report of two cases

Baghirzada

Krings

Carvalho

2012

Can J Anesth/J Can Anesth

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Background The anesthetic management of women with Marfan syndrome and dural ectasia undergoing Cesarean delivery remains controversial. We present two cases of patients with Marfan syndrome and dural ectasia where neuraxial anesthesia was used successfully. Case 1 A 31-yr-old G1P0 female with Marfan syndrome presented for elective Cesarean delivery at 35 3/7 weeks' gestation. The magnetic resonance imaging of her spine revealed significant dural ectasia in the lumbosacral area with a mean lumbar dural sac area of 4.71 cm 2 . Intrathecal administration of 0.75% hyperbaric bupivacaine 9 mg produced only limited perineal analgesia. The epidural catheter was titrated, and 0.5% bupivacaine 150 mg were required to achieve a T4 sensory level. Case 2 A 34-yr-old G1P0 female with Marfan syndrome presented for elective Cesarean delivery at 37 weeks' gestation. The intrathecal administration of 0.75% hyperbaric bupivacaine 13.5 mg produced a T5 sensory level. Magnetic resonance imaging of her spine prior to discharge confirmed the diagnosis of moderate dural ectasia with a mean lumbar dural sac area of 3.61 cm 2 . Discussion The two patients described in this report responded differently to spinal anesthesia, most likely based on the severity of their dural ectasia. Although preoperative magnetic resonance imaging may help to identify patients at risk for a failed spinal, we suggest considering a combined spinal-epidural technique in cases of dural ectasia. RésuméContexte La prise en charge anesthe´sique de femmes ayant un syndrome de Marfan et une ectasie de la dure-me`re au cours d'un accouchement par ce´sarienne reste un sujet controverse´. Nous pre´sentons le cas de deux patientes ayant un syndrome de Marfan et une ectasie de la dure-me`re chez qui une anesthe´sie neuraxiale a e´te´re´alise´e avec succe`s. Cas n8 1 Une femme de 31 ans, G1P0, ayant un syndrome de Marfan a e´te´admise pour accouchement par ce´sarienne programme´a`35 semaines et 3 jours de grossesse. L'imagerie par re´sonance magne´tique de la colonne verte´brale a re´ve´le´une ectasie significative de la dure-me`re dans la re´gion lombosacre´e avec une surface moyenne du sac dural lombaire de 4,71 cm 2 . L'administration intrathe´cale de 9 mg de bupivacaı¨ne hyperbare a`0,75 % n'a procure´qu'une analge´sie pe´rine´ale limite´e. La dose du cathe´ter pe´ridural a e´te´titre´e et 150 mg de bupivacaı¨ne 0,5 % ont e´te´ne´cessaires pour atteindre le niveau sensitif D4. Cas n8 2 Une femme de 34 ans, G1P0, ayant un syndrome de Marfan a e´te´admise pour accouchement par ce´sarienne programme´a`37 semaines de grossesse. L'administration intrathe´cale de 13,5 mg de bupivacaı¨ne Author contributions Leyla Baghirzada and Jose C.A. Carvalho cared for patients, reviewed the literature, and wrote the manuscript. Jose C.A. Carvalho and Timo Krings revised the manuscript, and Leyla Baghirzada submitted the final version of the manuscript. Timo Krings interpreted the magnetic resonance imagings for both patients.

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“…In these connective tissue disorders a medial degeneration of the coronary arteries is thought to lead to weakness of the arterial wall and increased susceptibility to spontaneous dissections. 15,16 An association between SCAD and systemic lupus erythematosus (SLE) has also been reported. 17,18 In this disease a general vasculitis is considered to be the pathological mechanism leading to chronic inflammation of the vessels and susceptibility to spontaneous dissections.…”

Section: Other Causes and Conditionsmentioning

confidence: 99%

Spontaneous coronary artery dissection: current insights and therapy

Tanis¹,

Stella²,

Pijlman³

et al. 2008

NHJL

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Spontaneous coronary artery dissection (SCAD) is a very rare cause of acute coronary syndromes in young otherwise healthy patients with a striking predilection for the female gender. The pathological mechanism has not been fully clarified yet. However, several diseases and conditions have been associated with SCAD, such as atherosclerosis, connective tissue disorders and the peripartum episode. In this paper we present a review of the literature, discussing the possible mechanisms for SCAD, therapeutic options and prognosis. The review is illustrated with two SCAD patients who had a recurrence of a spontaneous dissection in another artery within a few days after the initial event. Because of the susceptibility to recurrent spontaneous dissections we propose at least one week of observation in hospital. Further, we will elaborate on the possible conservative and invasive treatment strategies in the acute phase of SCAD. Primary percutaneous coronary intervention remains the reperfusion strategy of choice; however, in small and medium-sized arteries with normalised flow conservative treatment is defendable. In addition, after the acute phase evaluation of possible underlying diseases is necessary, because it affects further treatment. (Neth Heart J 2008;16:344-9.)

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Marfan's syndrome

Cited by 1,064 publications

References 107 publications

Rationale and design of a randomized clinical trial of β-blocker therapy (atenolol) versus angiotensin II receptor blocker therapy (losartan) in individuals with Marfan syndrome

Rationale and design of a randomized clinical trial of β-blocker therapy (atenolol) versus angiotensin II receptor blocker therapy (losartan) in individuals with Marfan syndrome

Regional anesthesia in Marfan syndrome, not all dural ectasias are the same: a report of two cases

Spontaneous coronary artery dissection: current insights and therapy

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