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Condition: Marfan Syndrome

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Total 54 results found since Jan 2013.

Outcomes of aortic valve-sparing root replacement with cusp repair in connective tissue disease
CONCLUSION: Remodelling and aortic valve repair showed excellent durability at 5 years, even in connective tissue disorders.PMID:37640626 | DOI:10.1016/j.acvd.2023.07.005
Source: Archives of Cardiovascular Diseases - August 28, 2023 Category: Cardiology Authors: Pichoy Danial Pierre Demondion Mathieu Debauchez Pascal Leprince Emmanuel Lansac Source Type: research

Open surgical replacement of the descending aorta: single-center experience
ConclusionsThis study showed that the elective open surgical replacement of the descending aorta can be achieved with excellent outcomes in terms of perioperative mortality and morbidity. Prosthetic graft is not a problem with open surgical descending aortic replacement, even in the long term.
Source: Indian Journal of Thoracic and Cardiovascular Surgery - January 3, 2023 Category: Cardiovascular & Thoracic Surgery Source Type: research

A retrospective observational study of serum uric acid and in-hospital mortality in acute type A aortic dissection
Sci Rep. 2022 Jul 19;12(1):12289. doi: 10.1038/s41598-022-16704-3.ABSTRACTThere is currently insufficient evidence of correlation between on-admission serum uric acid and in-hospital mortality of patients with acute type A aortic dissection. Thus, this study analysed the relation between serum uric acid and in-hospital deaths in patients with acute type A aortic dissection. A total of 1048 patients with acute type A aortic dissection participated in this study between January 2010 and December 2018. The independent variable was on-admission serum uric acid, whilst the dependent variable was in-hospital deaths. The covariat...
Source: Atherosclerosis - July 19, 2022 Category: Cardiology Authors: Guifang Yang Xiangping Chai Ning Ding Donghua Yang Qiong Ding Source Type: research

Marfan Syndrome with Aortic Root Disease, Severe Heart Failure and Aortic Dissection- Two Case Reports
PO Njoku, NC Mbadiwe, BJ Onwubere, EC Ejim, BC Anisiuba, TC Iyidobi, CT OnyemaNigerian Journal of Clinical Practice 2022 25(2):205-210 Marfan syndrome is an uncommon inheritable connective tissue disease which affects the cardiovascular system. This paper presents two cases of Marfan Syndrome with predominant aortic root disease that were seen at the Cardiology Clinic of University of Nigeria Teaching Hospital, Enugu, Nigeria. Their biography, clinical features and echocardiography parameters were captured using structured questionnaire. Both were young males in their 4th decade of life, and had advanced aortic root disea...
Source: Nigerian Journal of Clinical Practice - February 16, 2022 Category: Rural Health Authors: PO Njoku NC Mbadiwe BJ Onwubere EC Ejim BC Anisiuba TC Iyidobi CT Onyema Source Type: research

Long-term Use of Preoperative Beta-Blockers Is Associated With Reduced Postoperative Stroke After Thoracic Endovascular Aneurysm Repair
Thoracic endovascular aortic repair (TEVAR) has been established as the treatment of choice for a range of thoracic aortic pathologies. Beta-blockers represent a possible perioperative treatment option to improve TEVAR outcomes due to their role in management of aortic aneurysm, dissection, and Marfan syndrome. We investigated the effect of preoperative beta-blocker usage on outcomes of TEVAR using the Vascular Quality Initiative database.
Source: Journal of Vascular Surgery - August 24, 2021 Category: Surgery Authors: Kevin S. Yei, Niloofar Radgoudarzi, Rami Alhakim, Jonathan Unkart, Mahmoud B. Malas Tags: IP: Interactive Poster Session Source Type: research

Pre-sewn Multi-branched Aortic Graft and 3D-Printing Guidance for Crawford Extent II or III Thoracoabdominal Aortic Aneurysm Repair
Semin Thorac Cardiovasc Surg. 2021 May 7:S1043-0679(21)00194-5. doi: 10.1053/j.semtcvs.2021.03.046. Online ahead of print.ABSTRACTReconstruction of the visceral and segmental arteries is a challenging part of open surgical repair of extensive thoracoabdominal aortic aneurysm (TAAA). For more efficient reconstruction of these branching vessels, a technique of using pre-hand-sewn multi-branched aortic graft (octopod technique) has been adopted with the aid of 3D-printing-guidance in latest cases. The octopod graft has been employed for the extent II or III TAAA repair, in which the commercially available two 4-branched aorti...
Source: Hand Surgery - May 10, 2021 Category: Surgery Authors: Younju Rhee Sung Jun Park Taehun Kim Namkug Kim Dong Hyun Yang Joon Bum Kim Source Type: research

How Common are Aortic Aneurysms?
Discussion Aortic root dilatation or thoracic aortic aneurysm occurs in 6:100,000 individuals > 50 years of age. It is due to aging, hypertension, hypercholesterolemia, and smoking. Tertiary syphilis was a cause in the preantibiotic era. Pediatric aneurysms are very uncommon but the exact prevalence is different due to the various causes. Aneurysms are due to genetic disorders, congenital anomalies or post-surgical repair. In pediatric patients with sudden cardiac deaths, 5.4% are due to ruptured thoracic aortic aneurysms. Learning Point Some causes of pediatric aneurysms include: Familial thoracic aneurysm and dissect...
Source: PediatricEducation.org - February 22, 2021 Category: Pediatrics Authors: Pediatric Education Tags: Uncategorized Source Type: news

Transesophageal echocardiography (TEE) in the detection of intraoperative cardiac arrest: A case report
Rationale: Point-of-care ultrasound is widely used in patients with cardiac arrest, allowing for diagnosing, monitoring, and prognostication as well as assessing the effectiveness of the chest compressions. However, the detection of intraoperative cardiac arrest by Point-of-care ultrasound was rarely reported. Patient concerns: A 21-year-old male with Marfan syndrome which manifested Valsalva sinus aneurysms was admitted for aortic valve replacement. After endotracheal intubation, TEE transducer was inserted to evaluate the cardiac structure and function with different views. Severe aortic valve regurgitation was obse...
Source: Medicine - May 1, 2020 Category: Internal Medicine Tags: Research Article: Clinical Case Report Source Type: research

Limited versus extended repair for acute type A aortic dissection: long-term outcomes of the Beijing approach beyond 10 years.
CONCLUSIONS: In this large series of ATAAD, the FET + TAR technique has achieved satisfactory early and long-term survival and freedom from reoperation up to 15 years. Marfan syndrome and malperfusion syndrome were risk factors for early and late mortality and distal reoperation. This study adds long-term evidence supporting the use of the FET + TAR technique in patients with ATAAD involving the arch and descending aorta. PMID: 32077675 [PubMed - as supplied by publisher]
Source: The Journal of Cardiovascular Surgery - February 17, 2020 Category: Cardiovascular & Thoracic Surgery Authors: Ma WG, Chen Y, Zhang W, Li Q, Li JR, Zheng J, Liu YM, Zhu JM, Sun LZ Tags: J Cardiovasc Surg (Torino) Source Type: research

Optimising Aortic Endovascular Repair in Patients with Marfan Syndrome.
CONCLUSION: The specific protocol described in this study seems to optimise the results of endovascular aortic repair in MFS patients with significant aortic remodelling. PMID: 31865029 [PubMed - as supplied by publisher]
Source: PubMed: Eur J Vasc Endovasc ... - December 17, 2019 Category: Surgery Authors: Pellenc Q, Girault A, Roussel A, De Blic R, Cerceau P, Raffoul R, Milleron O, Jondeau G, Castier Y Tags: Eur J Vasc Endovasc Surg Source Type: research

One-year prognosis of non-traumatic cortical subarachnoid haemorrhage: a prospective series of 34 patients
ConclusionsThe most frequent causes of cSAH in our series were cerebral amyloid angiopathy, ischaemic stroke, and vasculitis. This type of haemorrhage has a worse prognosis than other non-aneurysmal cSAH. There are numerous possible causes, and prognosis depends on the aetiology. In elderly patients, intracranial haemorrhage is frequently associated with cognitive impairment.ResumenIntroducciónLas hemorragias subaracnoideas corticales (HSAc) tienen numerosas etiologías. No hay estudios prospectivos que indiquen su evolución a largo plazo. El objetivo de este trabajo es describir las características clínicas y etiológ...
Source: Neurologia - November 5, 2019 Category: Neurology Source Type: research

Successful Thrombolysis in the Setting of Marfan Syndrome and Chronic Aortic Dissection
Conclusion: To our knowledge, this is the first case of Marfan syndrome with chronic aortic dissection undergoing thrombolysis for acute ischemic stroke with a near-complete recovery of neurological deficit and favorable long-term outcome.
Source: The Neurologist - November 1, 2019 Category: Neurology Tags: Case Report/Case Series Source Type: research