Case report: Implantation of a personal aortic corset to stabilize the size of the root and the ascending aorta in a patient with Marfan syndrome

Herein, we report a case of a patient with Marfan syndrome for whom we successfully stabilized the root and the ascending aorta to prevent further expansion and reduce the aortic rupture risk. For stabilization, a personal aortic corset was used, cut, and formed as per the individual anatomical parameters of the patient (external dimensions of the root and the ascending aorta). Using a personalized approach, we could create a stabilizing aortic framework that matched the anatomical dimensions of the root and the ascending aorta of the patient. Implantation of a personal aortic corset with borderline dimensions of the root and the ascending aorta enabled us to prevent major surgical trauma, artificial circulation, and postoperative anticoagulant therapy as well as lower the risk of further expansion and aorta rupture in our patient, who was predisposed to these events. Postoperative results 1 year later showed the absence of negative dynamics regarding an increase in the size of the root and the ascending aorta or hemodynamic disorders in the aortic valve of our patient with Marfan syndrome.Received 29 January 2021. Revised 15 March 2021. Accepted 16 March 2021.Funding: The study did not have sponsorship.Conflict of interest: Authors declare no conflict of interest.

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Valve-sparing aneurysmal root/ascending aorta replacement in a 9-year-old patient with Marfan syndrome

Patologiya krovoobrashcheniya i kardiokhirurgiya ◽

10.21688/1681-3472-2018-1-61-65 ◽

2018 ◽

Vol 22 (1) ◽

pp. 61

Author(s):

V. N. Bogdanov ◽

I, V. Gladyshev ◽

E. F. Kharisova ◽

I. V. Harenko

Keyword(s):

Marfan Syndrome ◽

Tricuspid Valve ◽

Conflict Of Interest ◽

Clinical Case ◽

Surgical Repair ◽

Severe Form ◽

Ascending Aorta ◽

Aorta Replacement ◽

Valve Sparing ◽

Ascending Aorta Replacement

A clinical case report of a 9-year-old patient with a severe form of Marfan syndrome and significant enlargement of the aortic root and mitral/tricuspid valve failure is presented. The patient underwent successful one-stage surgical repair including reimplantation of the aortic valve using David procedure, mitral valve annuloplication and De Vega valvuloplasty of the tricuspid valve.Received 21 November 2017. Revised 19 February 2018. Accepted 27 February 2018.Funding: The study did not have sponsorship.Conflict of interest: Authors declare no conflict of interest.Author contributions All authors contributed equally to the work.

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Surgical treatment of ascending aorta aneurysms with chest wall deformity in patients with Marfan syndrome

Russian Journal of Thoracic and Cardiovascular Surgery ◽

10.24022/0236-2791-2018-60-1-77-80 ◽

2018 ◽

Vol 60 (1) ◽

pp. 77-80

Author(s):

V.A. Mironenko ◽

S.V. Rychin ◽

S.V. Garmanov ◽

S.M. Ergashev

Keyword(s):

Surgical Treatment ◽

Chest Wall ◽

Marfan Syndrome ◽

Ascending Aorta ◽

Chest Wall Deformity

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A life-threatening spontaneous ascending aortic rupture due to a small penetrating aortic ulcer

SAGE Open Medical Case Reports ◽

10.1177/2050313x211037784 ◽

2021 ◽

Vol 9 ◽

pp. 2050313X2110377

Author(s):

Yasuhito Nakamura ◽

Kiyoshi Doi ◽

Syojiro Yamaguchi ◽

Etsuji Umeda ◽

Osamu Sakai ◽

...

Keyword(s):

Rare Case ◽

Aortic Wall ◽

Aortic Rupture ◽

Ascending Aorta ◽

Bleeding Site ◽

Penetrating Aortic Ulcer ◽

Postoperative Course ◽

Life Threatening ◽

Aortic Dissections

We reported a rare case of spontaneous frank rupture of a small (4 mm) penetrating aortic ulcer in the ascending aorta resulted in catastrophic bleeding. The ulcer only created a pinhole wound in the adventitia without saccular aneurysms, intramural hematomas, or aortic dissections. Notably, the wound could be directly closed because the aortic wall was intact only 5 mm away from the bleeding site. The postoperative course was uneventful, and the patient was discharged on the 11th postoperative day. After 8 months, follow-up computed tomography showed no abnormality of the aortic wall at the repair site.

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Spontaneous aortic rupture during pregnancy

Orvosi Hetilap ◽

10.1556/oh.2011.29132 ◽

2011 ◽

Vol 152 (23) ◽

pp. 929-933 ◽

Cited By ~ 1

Author(s):

Zita Pánczél ◽

Levente Sára ◽

Péter Tóth ◽

Márta Hubay ◽

Éva Keller ◽

...

Keyword(s):

Sudden Death ◽

Aortic Dissection ◽

Genetic Basis ◽

Aortic Rupture ◽

Relevant Literature ◽

Ascending Aorta ◽

Multiparous Woman ◽

Rare Entity ◽

Third Trimester ◽

The Third

Aortic dissection is a rare entity. Half of the aortic dissection cases occur during pregnancy in women under the age of 40. The authors report a case of a multiparous woman at the third trimester of her sixth pregnancy, who died from a sudden and intractable cardiovascular shock. Autopsy revealed the dissection of the ascending aorta. The case is interesting, especially because in the pregnant woman’s family it was not the first sudden death during pregnancy. Authors review the relevant literature regarding the symptoms and the genetic basis of this rare but potentially lethal complication of pregnancy. Orv. Hetil., 2011, 152, 929–933.

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Management of the pregnant woman with Marfan syndrome complicated by ascending aorta dilation

Archives of Gynecology and Obstetrics ◽

10.1007/s00404-014-3307-4 ◽

2014 ◽

Vol 290 (4) ◽

pp. 797-802 ◽

Cited By ~ 14

Author(s):

Daniel A. Cox ◽

Salil Ginde ◽

Randall S. Kuhlmann ◽

Michael G. Earing

Keyword(s):

Pregnant Woman ◽

Marfan Syndrome ◽

Ascending Aorta

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Serological level of transforming growth factor-β as a predictive biomarker of aortic enlargement in patients with Marfan syndrome

Pediatrician (St Petersburg) ◽

10.17816/ped8161-66 ◽

2017 ◽

Vol 8 (1) ◽

pp. 61-66

Author(s):

Andrey S Rudoy ◽

Alexey M Uryvaev

Keyword(s):

Risk Factors ◽

Growth Factor ◽

Marfan Syndrome ◽

Aortic Root ◽

Transforming Growth Factor ◽

Aortic Rupture ◽

Imaging Techniques ◽

Elevated Serum ◽

Transforming Growth Factor Β ◽

Absolute Size

Marfan syndrome - an inherited, autosomal dominant disease with an expected rate of 3-5/10 000 or fraction of 20-25% of new mutations, accompanied by violation of the connective tissue that occurs as a result of gene mutations FBN1, coding for the synthesis of fibrillin-1, performing the most important role in the modulation physiological bioavailability TGF-β (transforming growth factor-β). Prediction of aortic rupture is based on the identification of risk factors: family history, the absolute size of the aortic root, the rate of expansion of the aorta, which are based on the results of the history and techniques of imaging ultrasound, CT, MRI. At the same time there is a chance of developing aortic rupture under normal aortic root size and the absence of any risk factors, as well as after the prophylactic prosthetic aortic root. This makes it necessary to search for alternative prognostic markers, threatening bundle and rupture of the aorta. Article verified the predictive role of TGF-β as a serological biomarker for assessing the extension of the aortic root in patients with Marfan syndrome (n = 23, F : M / 7 : 16; 33 ± 9.3 years). The article describes the patterns between TGF-β and the size and the reconstruction of the aneurysm of the thoracic aorta. It was found that elevated levels of serum TGF-β1 (49.1 ng/ml Vs 29.15 ng/ml in the control, p < 0.05) in patients with MS diagnosed with an extension of the aortic root (Z > 1.96) can serve as a serological marker to poor prognosis, accompanied by an increase in the size of the aortic root. In patients with normal-sized aorta, and after aortic reconstruction serum TGFβ1 not elevated. Serum TGFβ may be a promising target for therapeutic, diagnostic and prognostic tactics which are not based on imaging techniques.

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A case of traumatic thoracic aorta rupture - A life threatening emergency

Journal of Cardiovascular and Thoracic Research ◽

10.15171/jcvtr.2019.41 ◽

2019 ◽

Vol 11 (3) ◽

pp. 248-250

Author(s):

Rupesh Kumar ◽

Javid Raja ◽

Ganesh Kumar Munirathinam ◽

Anand Kumar Mishra ◽

Rana Sandeep Singh ◽

...

Keyword(s):

Motor Vehicle ◽

Motor Vehicle Accident ◽

Aortic Rupture ◽

Medical Attention ◽

Vehicle Accident ◽

Complete Tear ◽

Life Threatening ◽

Injury Treatment ◽

Aorta Rupture ◽

Aortic Transection

Traumatic aortic transection is a life threatening emergency where there is a near-complete tear through all the layers of the aorta due to trauma. This condition is most often lethal and requires immediate medical attention. Symptoms of an aortic rupture may include severe chest pain, back pain, abdominal pain and signs of external chest injury. Treatment should be prompt in hemodynamically unstable patient in the form of endovascular or open surgical technique. We present a twenty nine year old male with aortic transection following motor vehicle accident where an interposition tube graft was placed after trimming the lacerated segments of the aorta under cardiopulmonary bypass. The patient is doing well with two years of follow up at our institution.

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Early and Long-Term Results of a Valve-Sparing Operation for Marfan Syndrome

Circulation ◽

10.1161/circ.100.suppl_2.ii-29 ◽

1999 ◽

Vol 100 (suppl_2) ◽

Cited By ~ 1

Author(s):

Emma J. Birks ◽

Carole Webb ◽

Anne Child ◽

Rosemary Radley-Smith ◽

Magdi H. Yacoub

Keyword(s):

Infective Endocarditis ◽

Marfan Syndrome ◽

Ascending Aorta ◽

Long Term Results ◽

Thromboembolic Complications ◽

Actuarial Survival ◽

Acute Dissection ◽

Valve Sparing

Background —We have previously described the experience, rationale, and development of a valve preserving technique, but its role in patients with Marfan syndrome has not previously been defined. Here, we attempt to determine the early and long-term results, timing, and determinants of outcome of this operation in patients with Marfan syndrome. Methods and Results —Since 1979, 82 patients (73.2% of all patients with Marfan syndrome undergoing resection of aneurysm of the ascending aorta) were operated on using this technique. Ages ranged from 2 to 69 years (mean, 33.9 years). In all, there were 4 early deaths (4.9%), 2 with acute dissection and 2 with chronic aneurysm operated on as emergencies. There were no early deaths in 67 patients operated on electively. Actuarial survival for patients operated for chronic aneurysm was 94.2%, 94.2%, and 94.2% at 1, 5, and 10 years, respectively; that for acute dissection was 72.7%, 63.6%, and 63.6%; and that for chronic dissection was 100%, 85.7%, and 75.0%. The probability of needing reoperation was 5.7%, 17.3%, and 17.3% at 1, 5, and 10 years. There were no instances of infective endocarditis or thromboembolic complications except in 2 patients operated on early in the series who had cusp extension. At the end of the follow-up, trivial or no aortic regurgitation was demonstrated in 33.3%, mild in 45.6%, moderate in 21.1%, and severe in 0. Conclusions —Valve-sparing operations are feasible in most patients with Marfan syndrome; they are applicable to patients with both dissection and chronic aneurysm. The early and long-term results are encouraging. Results are better in the absence of dissection, and prophylactic operation is warranted in some cases.

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