CONTEXT Aortic stiffness is increased in Marfan syndrome contributing to aortic dilatation and rupture, the major cause of premature death in this population. Angiotensin-converting enzyme inhibitors have been shown to reduce arterial stiffness. OBJECTIVE To determine whether perindopril therapy reduces aortic stiffness and attenuates aortic dilatation in patients with Marfan syndrome. DESI...

A probable autosomal recessive mode of inheritance is described in a family with two affected sisters. The sisters showed the typical picture of Marfan syndrome and were of normal intelligence. Both parents and all four grandparents were personally examined and found to be normal. Homocystinuria was ruled out on repeated examinations. This family suggests genetic heterogeneity in Marfan syndrom...

Acute aortic dissection (AAD) during pregnancy can be fatal to both the pregnant mother and the baby, particularly in patients with the Marfan syndrome. We report a case of the modified Bentall procedure in surgery for AAD in a 31-year-old pregnant woman at 24 weeks of gestation with the Marfan syndrome. The patient recovered well after the operation, but unfortunately, the fetus could not be s...

Early onset Marfan Syndrome (eoMFS) is a rare, severe form of Marfan Syndrome (MFS). The disease has a poor prognosis and most patients present with resistance to heart failure treatment during the newborn period. This report presents two cases of eoMFS with similar clinical features diagnosed in the newborn period and who died at an early age due to the complications related to the involvement...

ont matter & 2006 006.09.007 thor. Tel.: +44 117 9. : Andrew.McBride@ rtin.gargan@ubht Summary Marfan syndrome is an inherited connective tissue disorder with multi-organ system involvement caused by mutations in the gene encoding the glycoprotein fibrillin-1 (FBN1). The condition presents to the orthopaedic surgeon with an array of musculoskeletal problems. This article will review the pathoge...

Background and Purpose—Small case series have associated Marfan syndrome with cerebral and spinal ischemia or hemorrhage. However, there has been no investigation of the frequency and etiology of neurovascular disorders in a large series of Marfan patients. Methods—We conducted a retrospective, hospital-based study of all Marfan syndrome patients seen in an 8-year period. Records were reviewed ...

BACKGROUND AND PURPOSE Small case series have associated Marfan syndrome with cerebral and spinal ischemia or hemorrhage. However, there has been no investigation of the frequency and etiology of neurovascular disorders in a large series of Marfan patients. METHODS We conducted a retrospective, hospital-based study of all Marfan syndrome patients seen in an 8-year period. Records were reviewe...

Concomitant heart transplantation and ascending aorta replacement is infrequent. In the few cases where this simultaneous procedure was performed, most patients were diagnosed with Marfan syndrome. We report the combined procedure of heart transplantation and ascending aorta replacement using the donor's ascending aorta, in a 70-year-old man who was not diagnosed with Marfan syndrome. This comb...