Aortic root surgery in Marfan syndrome

Choudhary, Shiv Kumar; Goyal, Aayush

doi:10.1007/s12055-018-0761-9

Cited by 6 publications

(6 citation statements)

References 52 publications

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“…The most commonly encountered and the most characteristic feature of a patient suffering from MFS, which is also a major diagnostic criterion along with lens subluxation in the 2010 revised Ghent nosology, is the increase in the diameter of the aortic root. This dilation of the aortic root, which can ultimately lead to dreaded complications like aortic dissection and aneurysm, is the single most important and frequent reason of mortality in a patient with MFS [23]. The condition's prevalence is shown to be more in adults than in children and has been suggested in a study by Roman et al according to which the prevalence of aortic root dilation is marginally higher in adults in comparison to children (approximately 90% vs 80%) [24].…”

Section: Aortic Aneurysm and Dissectionmentioning

confidence: 99%

“…Regarding surgical options, the most effective procedure is aortic root replacement. It is of two types, namely total root replacement (TRR) and valve-sparing root replacement (VSRR) [23]. TRR was introduced by Bentall and De Bono and involves the replacement of the whole of the aortic root with a prosthetic graft along with mechanical valves as a replacement for the organic valves of the aorta [23].…”

Section: Managementmentioning

confidence: 99%

“…It is of two types, namely total root replacement (TRR) and valve-sparing root replacement (VSRR) [23]. TRR was introduced by Bentall and De Bono and involves the replacement of the whole of the aortic root with a prosthetic graft along with mechanical valves as a replacement for the organic valves of the aorta [23]. In the case of postsurgical graft infection, surgical removal of the infected graft is the preferred choice of treatment [47].…”

Section: Managementmentioning

confidence: 99%

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Cardiac Complications in Marfan Syndrome: A Review

Singh¹,

Wanjari²

2022

Cureus

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Marfan syndrome (MFS) is a rare inherited disorder of the connective tissue with an autosomal dominant mode of inheritance which happens as a result of a mutation in the fibrillin-1 (FBN1) gene located on chromosome 15q21.1. This mutation results in the defective formation of microfibrils and increased levels of active transforming growth factor beta (TGF beta), leading to defective connective tissue synthesis. These changes affect various parts of the body but most notably affected are the heart, eyes, and the musculoskeletal system. The standard presenting features of a person suffering from MFS are tall stature with a large arm span, kyphosis, congenital dislocation of the lens (ectopia lentis) and cardiovascular manifestations. The 2010 modified Ghent criteria are used to diagnose MFS on the basis of parameters such as cardiovascular, eye, and musculoskeletal disorders. The cardiovascular manifestations in a patient with MFS are the leading causes of mortality. The most common and dreaded complication is an aortic aneurysm and subsequent dissection. Cardiomyopathy and arrhythmia are also potential killers in such patients. This article aims to look at the various cardiac complications mentioned above and gain an understanding of their pathogenesis, incidence, and outcome. It also includes a brief overview of the rare complication post-Bentall graft infection, and its cause, diagnosis, and management. Various articles by several different authors from around the world were searched for information regarding the pathogenesis, incidence, and outcomes of these patients and are referenced below.

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Section: Aortic Aneurysm and Dissectionmentioning

confidence: 99%

Section: Managementmentioning

confidence: 99%

Section: Managementmentioning

confidence: 99%

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Cardiac Complications in Marfan Syndrome: A Review

Singh¹,

Wanjari²

2022

Cureus

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“…У 1896 р. хворобу детально описав французький педіатр А. Марфан, ім'ям якого її і назвали. На сьогодні доведено, що синдром Марфана має аутосомно-домінантний тип успадкування з високою проникністю мутантного гена [2,4]. При цьому захворюванні порушується синтез колагену і еластину у зв'язку з пошкодженням гена 15-ї хромосоми, відповідального за синтез фібриліну, білка, що є важливим компонентом сполучної тканини, оскільки формує її еластичність і скоротливість [2,6,7].…”

unclassified

“…Консервативне лікування уражень серцево-судинної системи не ефективне. Причиною смерті хворих, переважно віком 20-40 років, є розшарування (розрив) аорти, рідше -хронічна серцево-судинна недостатність [1,2]. Хірургічне лікування такої патології дає змогу зберегти і продовжити життя цій категорії хворих.…”

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Marfan syndrome: diagnosis and treatment of cardiovascular lesions

Кравченко¹,

Kravchenko²,

Osadovska³

et al. 2021

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Purpose – to analise of results, long-term experience of surgical treatment of cardiovascular diseases in patients with Marfan syndrome to improve quality and prolong life. Materials and methods. A conducted prospective analysis of consecutive patients (292) with Marfan syndrome treated at the institute during 1980–2018. The age of patients was 7–57 years, mean 31.6±9.4 years. Among them, men – 220 (75.3%), women – 72 (24.7%). Marfan syndrome was diagnosed according to the criteria of Gent Nosology (2010). 24 (8.2%) patients were not operated on for various reasons. The remaining 268 (91.8%) were managed surgicaly: 257 – ascending aortic aneurysm; 4 – abdominal; 7 patients – only mitral valve insufficiency. Results. Hospital mortality was 9.3% (25 patients). Long-term results were studied in 224 (92.2%) patients from all who had been discharged from the clinic within 6 months – 20 years on average 63 months. Good long-term results were admitted in 145 (64.7%), satisfactory in 41 (18.3%), unsatisfactory in 14 (6.3%) patients. 24 (9.9%) patients died in the long term observation. Conclusions. Aortic aneurysms in patients with Marfan syndrome are formed at a young age. The most common cause sof death in the unoperated cases are rupture of aneurism or heart failure. The «gold-standarg» remains the Bentall De Bono operation. The patients who underwent surgical treatment requires observation during all-life period. The research was carried out in accordance with the principles of the Helsinki Declaration. The study protocol was approved by the Local Ethics Committee of these Institutes. The informed consent of the patient was obtained for conducting the studies. The authors declare no conflicts of interests. Key words: Marfan syndrome, aortic aneurysm, aortic dissection.

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