Dissecting aortic aneurysm in Marfan"s syndrome

by S. R. F. Whittaker

Publisher: Lancet Office in London

Written in English
Published: Downloads: 302
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Subjects:

  • Marfan syndrome.,
  • Dissecting aortic aneurysms.

Edition Notes

StatementS.R.F. Whittaker, J. D. Sheehan, M.D..
ContributionsSheehan, J. D., M.D.
The Physical Object
Pagination4p. :
ID Numbers
Open LibraryOL20826382M

Aortic aneurysms are a common finding in elderly patients. Rupture of an aortic aneurysm is a catastrophic event associated with a very high mortality. Objective/s. To review the current literature on aortic aneurysmal disease, including the recommended referral threshold, surveillance guidelines and treatment options. Discussion. To the Editor: Data from Gott et al. (April 29 issue) 1 show no correlation between the aortic-root diameter and the probability of aortic dissection in patients .   The following are key points to remember from this update on clinical trials of losartan with and without beta-blockers to block aneurysm growth in patients with Marfan syndrome: Thoracic aortic aneurysms leading to acute aortic dissections are a significant cause of morbidity and mortality despite significant advances in surgical treatment. CARDIAC SURGERY. People with Marfan syndrome frequently have problems with their heart and blood vessels. Sometimes, these problems are very serious. The most common complication affects the aorta (the main blood vessel carrying blood from the heart to the rest of the body). Heart valves may be affected as well.

As with other connective tissue disorders, Marfan syndrome is associated with a predisposition to the development of progressive thoracic aortic aneurysm (TAAs) and dissection (TAAD). Accurate diagnosis can lead to changes in clinical management, including surveillance of the aorta and surgical repair of the aorta when necessary, as well as.   What are aorta disease and Marfan syndrome? ANSWER The aorta is the large artery that leaves the heart and provides oxygen-rich blood to the rest of your body. These two things can cause the aorta. Aortic Aneurysm Marfan Syndrome Thoracic Aortic Aneurysm Pectus Excavatum Emergent Repair These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm : Jane V. Stewart. •Ascending Aorta and arch aneurysm •Stanford Type A aortic dissection, Severe Aortic Regurgitation. •Sinus Rhythm •Good LV Function •No CCF Conclusion: •Marfan syndrome – rare disease variable presentation with major cardiovascular manifestation. •Aortic aneurysm and dissections incidence more common than general population.

Recent studies have suggested the number of cases of aortic disease is increasing over time, which may be related to improved diagnostic modalities and/or increased awareness of aortic disease. 1,2 Left untreated, one-third of patients with an acute ascending aortic dissection will die within the first 24 hours and 50% will die within 48 hours.   In the cardiovascular system: Aneurysms of the aorta, including dissecting aneurysms, valvular and septal defects, coarctation of the aorta, dissecting aneurysms of other major vessels, including pulmonary artery, aneurysm of the sinuses of Valsalva, and ruptured aortic cusps. Hernias and hypermobility of the joints are by: The Marfan Syndrome, Loeys-Dietz Syndrome, Familial Thoracic Aortic Aneurysms & Dissections, and Related Disorders NGS Panel is designed to be a cost effective method for detecting mutations in genes causing aneurysms of the aorta and its major branches.   Annuloaortic ectasia and dissection of the ascending aorta are common cardiovascular complications of Marfan's syndrome. True aneurysms of the subclavian arteries in these patients are rare. We present an unusual case of a year-old woman who over the span of 10 years sequentially developed an aneurysm of the ascending aorta (annuloaortic ectasia), Cited by:

Dissecting aortic aneurysm in Marfan"s syndrome by S. R. F. Whittaker Download PDF EPUB FB2

What is thoracic aortic aneurysm and dissection. A thoracic aortic aneurysm is an enlargement of the aorta (the main blood vessel that carries blood away from the heart to the rest of the body) in the part of the body called the thoracic cavity (the chest area enclosed by the ribs and containing the lungs and heart).

If the aneurysm is not surgically repaired, it can lead to aortic dissection. Abstract. A patient with the Marfan syndrome died suddenly from aortic rupture and dissection in the early puerperium of her second pregnancy.

Although the association of the Marfan syndrome and pregnancy is extremely rare, the case reported here being only the fifth on record, the concurrence of dissecting aneurysm or aortic dissection with pregnancy is more by: Aortic aneurysms in Marfan syndrome can be broad and multiple because of the congenital fragility of the aortic wall, which is known as cystic medial necrosis (CMN), 11 In addition, the aneurysms are usually the dissecting type and are often located in the thoracic region because the ascending aorta is subjected to continuous pressure Cited by: OBJECTIVES: True abdominal aortic aneurysm (AAA) in patients with Marfan syndrome is relatively rare because most aortic aneurysms in this disease are dissecting aneurysms in the thoracic area.

Therefore, its clinical characteristics and long-term outcome are still by: Marfan syndrome is caused by defects in a gene called fibrillin Fibrillin-1 plays an important role as the building block for Dissecting aortic aneurysm in Marfans syndrome book tissue in the body.

The gene defect also causes the long bones of the body to grow too much. People with this syndrome have tall height and long arms and legs.

How this overgrowth happens is not well. Background. Aortic dissection is the most frequent cause of premature death in Marfan’s syndrome. Low-risk elective surgery of the abnormal aortic root has the potential to prevent this s. We examine genetic, structural, and pathophysiological mechanisms of aortic dissection and discuss the surgical methods used when dissection by: A year-old man with a history of Marfan's syndrome was admitted to the hospital with back and chest pain secondary to a dissecting aortic aneurysm.

He later underwent surgical aortic bypass. Aortic dissection (AD) occurs when an injury to the innermost layer of the aorta allows blood to flow between the layers of the aortic wall, forcing the layers apart. In most cases, this is associated with a sudden onset of severe chest or back pain, often described as "tearing" in character.

Also, vomiting, sweating, and lightheadedness may occur. Other symptoms may Complications: Stroke, mesenteric ischemia. About 40% of Marfan syndrome patients will die immediately if aortic dissection occurs.

The risk of death is between 1% and 3% per hour after the dissection event. Even with emergency surgery, the risk of death is between 10% and 20%. The aorta is the largest artery in the body and is the blood vessel that carries oxygen-rich blood away from the heart to all parts of the body.

An aortic aneurysm is an abnormal enlargement or bulging of the wall of the aorta. Vidyasagar Kalahasti, Director of the Marfan Clinic answers your questions about aortic aneurysm and Marfan syndrome.

Marfan syndrome (MFS) is a genetic disorder of the connective tissue. The degree to which people are affected varies. People with Marfan tend to be tall and thin, with long arms, legs, fingers and toes. They also typically have flexible joints and scoliosis. The most serious complications involve the heart and aorta, with an increased risk of mitral valve prolapse and Complications: Scoliosis, mitral valve.

People with Marfan syndrome are at up to times greater risk of aortic dissection (a tear or rupture between layers of the aortic wall) than the general population. That’s why it’s important to know the signs of an aortic dissection and what to g that you have Marfan syndrome puts you at an advantage in the event that you experience unexplained chest, back, or.

Video Thoracoabdominal aortic aneurysm repair in Marfan syndrome: how we do it. Clinical vignette A year-old male patient was diagnosed at an outside hospital with an acute non-complicated type-B aortic dissection, 5 months prior to his recent : Rana O.

Afifi, Akiko Tanaka, Ibrahim Yazji, Hazim J. Safi, Anthony L. Estrera. Habashi JP, Judge DP, Holm TM, et al. Losartan, an AT1 antagonist, prevents aortic aneurysm in a mouse model of Marfan syndrome. Science ; Groenink M, den Hartog AW, Franken R, et al.

Losartan reduces aortic dilatation rate in adults with Marfan syndrome: a randomized controlled trial. Eur Heart J ;Cited by: 4.

We report a retrospective cohort of Marfan patients who survived an aortic dissection. Dissection occurred in either the ascending aorta (AscAo) (n = 37), the descending aorta (DescAo) (n = 20), or both (As + DescAo, n = 43).During a mean follow-up of ± years (complete for 88% of the patients), 17 patients died and 52 had a clinical event (new aortic Cited by: due to dissecting aneurysm, 49 occurred in young womenandofthese 24 were pregnant at the time ofthe fatal rupture.

Thisproportion, 50%approxi-mately, is supported by Mandel, Evans, and Walford (), who found that of 70 reported instances of dissecting aneurysm in young women, 37 were pregnant or in the puerperium at the time of by: Introduction. Marfan syndrome is an autosomal dominant genetic disorder resulting from a mutation in FBN1, leading to ocular, skeletal, pulmonary, and cardiovascular manifestations.

1 Aortic aneurysm and dissection are responsible for premature morbidity and mortality in this disorder. 2 Guidelines recommend serial imaging surveillance of the aortic root and Cited by: 6. Since connective tissue provides the tensile strength of the aortic wall, the lack of structural support to the aorta can lead to the formation of an aneurysm and aortic dissection.

The risk of rupture increases with the diameter of the aorta and therefore all patients with Marfan syndrome or other connective tissue disorders should be followed. Background Aortic dissections involving the descending aorta are a major clinical problem in patients with Marfan syndrome.

Objectives The purpose of this study was to identify clinical parameters associated with type B aortic dissection and to develop a risk model to predict type B aortic dissection in patients with Marfan syndrome.

Methods Patients with the diagnosis Cited by: Introduction. Marfan syndrome (MFS) is a rare connective tissue disorder attributed to a defect in the fibrillin-1 gene. Aortic aneurysms and dissection are common causes of morbidity and.

CARDIOVASCULAR stigmata are present in 30% to 60% of patients with Marfan's syndrome and dissecting aneurysm occurring in a medionecrotic aorta accounts for at least one third of the cardiovascular manifestations. 1 In the past, the diagnosis of aortic dissection was either inferred from the clinical signs or made at autopsy.

2,3 Angiography has not been widely Cited by: 4. T he primary cause of morbidity and mortality in patients with Marfan syndrome (MFS) is related to the propensity to aortic aneurysm formation and associated dissection. Noninvasive cardiovascular imaging has contributed to the improved survival noted among patients with MFS in the current era.

Ascending aortic dilatation is usually readily detected with transthoracic. "Back From the Edge" - Borderline Personality Disorder - Call us: - Duration: NewYork-Presbyterian Hospital Recommended for you. Aneurysmal dilatation of the aortic annulus and ascending aorta is seen measuring upto X cm.

with an intimal dissection flap is seen involving the ascending aorta just above the aortic root and the LVOT and spiraling across the entire ascending aorta, aortic arch and the descending aorta down to the right and left common iliac and common femporal arteiries.

The Marfan syndrome (MFS) is an autosomal dominantly inherited disorder of connective tissue with multisystem involvement. It is caused by mutations in the FBN1 gene on chromos which encodes a glycoprotein called fibrillin-1, a component of the extracellular matrix.

Over mutations have been identified in the fibrillin-1 gene associated with MFS, other genes related Cited by: 1. Marfan syndrome is a disorder of connective tissue. This is the tissue that strengthens the body's structures.

Disorders of connective tissue affect the skeletal system, cardiovascular system, eyes, and skin. Marfan syndrome is caused by defects in a gene called fibrillin Fibrillin-1 plays an important role as the building block for.

An aortic dissection is an emergency with your aorta, which is the largest blood vessel in your body and the main artery that takes blood from your heart to the rest of your body. During an. INTRODUCTION. Cardiovascular problems, such as aortic dissection and rupture, comprise the most life-threatening aspects of Marfan syndrome.

The aortic root is most frequently affected and the prophylactic surgical replacement of the aortic root might prevent further aortic catastrophe in most of such patients [].The present study verifies this hypothesis by clarifying Cited by: 5.

after the first aortic dissection (mean [ ± years). Patients identified in this series were signifi-cantly younger at first dissection and more likely to carry the diagnosis of Marfan syndrome. Aortic aneurysm diameter was quantified before and after the new dissection event and demonstrated a.

Introduction. Marfan syndrome (MFS) is an autosomal dominant systemic connective tissue disorder caused by mutations in the fibrillin-1 gene, with a prevalence of approximately two to three patients per 10 individuals (1).In patients with MFS, the aorta gradually dilates, ultimately leading to aortic aneurysm formation and aortic by:.

Aneurysm of the descending thoracic aorta with a diameter of 45 mm over a length of 50 mm. Note that both subclavian arteries (left > right) are also dilated. 2 case question available Q: What tends to be most frequent cause of death of patients with Marfan syndrome?

Genetic testing by Lehigh Valley Health Network's heart and vascular program helped reveal Marfan syndrome in Michael Brennan after his treatment for a thoracic aortic aneurysm that became an.

We describe a patient with the Marfan syndrome who presented with an acute aortic dissection. She underwent composite graft replacement of the aortic root.

She returned two years later with dyspnoea and stridor due to tracheal compression by a large chronic dissection of the thoracic aorta. Marfan patients are at risk of chronic dissection involving the remaining Cited by: 7.