Clinical and epidemiological description of aortic dissection in Turner's syndrome

Background: Women with Turner's syndrome have an increased risk of congenital cardiac malformations, ischaemic heart disease, hypertension and stroke. Aortic dissection seems to occur with increased frequency. Aim: To describe in more detail aortic dissection as encountered in Turner's syndrome, giving attention to clinical, histological and epidemiological aspects. Materials and methods: Based on a retrospective study, we describe the clinical, karyotypic, and epidemiological aspects of aortic dissection as encountered in cases of Turner's syndrome seen in Denmark and Sweden. Results: The median age at onset of aortic dissection in 18 women was 35 years, ranging from 18 to 61 years. Fourteen of 18 women had a 45,X karyotype, while 2 patients had 45,X/45,XY, and 2 had the 45,X/46,X+r(X) complement, respectively. Echocardiography was performed in 10 of 18 patients before their acute illness, and showed signs of congenital cardiac disease, with either bifoliate aortic valves, dilation of the aortic root, or previous aortic coarctation evident in most patients. In 5 patients evidence of a bifoliate aortic valve was conclusive. Hypertension was present in 5 of 18 patients, while 10 of the patients died from aortic dissection, of so-called type A in 6, type B in 3, while in the final case the origin of dissection could not be determined. Biochemical analysis showed altered ratio between type I and type III collagen. Histology showed cystic medial necrosis in 3 of 7 cases. We estimated an incidence of dissection of 36 per 100,000 Turner's syndrome years, compared with an incidence of 6 per 100,000 in the general population, and a cumulated rate of incidence of 14, 73, 78, and 50 per 100,000 among 0–19, 20–29, 30–39, and 40+ year olds, respectively. Conclusion: Aortic dissection is extremely common in the setting of Turner's syndrome, and occurs early in life. Patients with Turner's syndrome should be offered a protocol for clinical follow-up similar to that provided for patients with Marfan syndrome, and each clinic should embrace a programme for follow-up.

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Long- term prognosis of type A aortic dissection patients with histological pattern of cystic medial necrosis

The Thoracic and Cardiovascular Surgeon ◽

10.1055/s-2007-967317 ◽

2007 ◽

Vol 55 (S 1) ◽

Author(s):

E Girdauskas ◽

T Kuntze ◽

M Borger ◽

T Doenst ◽

M Mochalski ◽

...

Keyword(s):

Aortic Dissection ◽

Type A ◽

Cystic Medial Necrosis ◽

Type A Aortic Dissection ◽

Histological Pattern ◽

Medial Necrosis ◽

Long Term Prognosis

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Chronic Cholestasis Associated with Turner’s Syndrome: 12 Years of Clinical and Histopathological Follow-Up

Digestion ◽

10.1159/000069702 ◽

2003 ◽

Vol 67 (1-2) ◽

pp. 96-99 ◽

Cited By ~ 8

Author(s):

Joram Wardi ◽

Boleslaw Knobel ◽

Mark Shahmurov ◽

Enrique Melamud ◽

Yona Avni ◽

...

Keyword(s):

Turner's Syndrome ◽

Turner’S Syndrome ◽

Chronic Cholestasis

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Candy-Plug Generation II for False Lumen Occlusion in Chronic Aortic Dissection: Feasibility and Early Results

Journal of Endovascular Therapy ◽

10.1177/1526602819871613 ◽

2019 ◽

Vol 26 (6) ◽

pp. 782-786 ◽

Cited By ~ 7

Author(s):

Ahmed Eleshra ◽

Tilo Kölbel ◽

Nikolaos Tsilimparis ◽

Giuseppe Panuccio ◽

Martin Scheerbaum ◽

...

Keyword(s):

Aortic Dissection ◽

Clinical Success ◽

False Lumen ◽

The Other ◽

Type I ◽

Technical Success ◽

Chronic Aortic Dissection ◽

Early Results ◽

Aortic Remodeling

Purpose: To present the early results of false lumen (FL) occlusion in chronic aortic dissection using the Candy-Plug generation II (CP II), which has a self-closing fabric channel that obviates the need for separate occlusion of its center. Materials and Methods: Fourteen consecutive patients (mean age 60±11 years; 10 men) with persistent FL backflow and aneurysm formation at the thoracic segment in chronic aortic dissection underwent thoracic endovascular aortic repair (TEVAR) with FL occlusion using the refined CP II. Primary endpoints were technical success (successful deployment) and clinical success (no FL backflow at the CP II level). Secondary endpoints included 30-day mortality and morbidity and aortic remodeling during follow-up. Results: Technical success was 100%. One patient required additional intraprocedural FL embolization at the CP II level due to persistent FL backflow on final angiography (clinical success 93%), though there was no flow through the CP II center. There were no intraprocedural complications. Immediate complete FL occlusion was achieved in 12 patients; the other 2 required reintervention. One had contrast enhancement in the distal FL proximal to the CP II and was treated with coil embolization. The other patient had persistent type I endoleak at the level of the left subclavian artery (LSA) and underwent left carotid–LSA bypass and proximal stent-graft extension. One patient died due to retrograde type A aortic dissection that was not related to CP II placement. Over a mean 8-month follow-up (range 3–12), 9 patients had computed tomography angiography; 8 patients had evidence of aortic remodeling, while 1 aneurysm sac was stable. Conclusion: The CP II reduces the number of procedural steps and offers good seal, with minimal morbidity and mortality and a high rate of aortic remodeling.

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PERITONEAL ADHESIONS TYPE I, III AND TOTAL COLLAGEN ON POLYPROPYLENE AND COATED POLYPROPYLENE MESHES: EXPERIMENTAL STUDY IN RATS

ABCD Arquivos Brasileiros de Cirurgia Digestiva (São Paulo) ◽

10.1590/0102-6720201700020001 ◽

2017 ◽

Vol 30 (2) ◽

pp. 77-82 ◽

Cited By ~ 2

Author(s):

Lucas Félix ROSSI ◽

Manoel Roberto Maciel TRINDADE ◽

Armando José D`ACAMPORA ◽

Luise MEURER

Keyword(s):

Type I Collagen ◽

Cell Types ◽

Type Iii Collagen ◽

Type I ◽

Abdominal Adhesions ◽

Peritoneal Adhesions ◽

Type Iii ◽

Total Collagen

ABSTRACT Background: Hernia correction is a routinely performed treatment in surgical practice. The improvement of the operative technique and available materials certainly has been a great benefit to the quality of surgical results. The insertion of prostheses for hernia correction is well-founded in the literature, and has become the standard of treatment when this type of disease is discussed. Aim: To evaluate two available prostheses: the polypropylene and polypropylene coated ones in an experimental model. Methods: Seven prostheses of each kind were inserted into Wistar rats (Ratus norvegicus albinus) in the anterior abdominal wall of the animal in direct contact with the viscera. After 90 days follow-up were analyzed the intra-abdominal adhesions, and also performed immunohistochemical evaluation and videomorphometry of the total, type I and type III collagen. Histological analysis was also performed with hematoxylin-eosin to evaluate cell types present in each mesh. Results: At 90 days the adhesions were not different among the groups (p=0.335). Total collagen likewise was not statistically different (p=0.810). Statistically there was more type III collagen in the coated polypropylene group (p=0.039) while type I was not different among the prostheses (p=0.050). The lymphocytes were statistically more present in the polypropylene group (p=0.041). Conclusion: The coated prosthesis was not different from the polypropylene one regarding the adhesion. Total and type I collagen were not different among the groups, while type III collagen was more present on the coated mesh. There was a greater number of lymphocytes on the polypropylene mesh.

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Liver Abnormalities in Turner’s Syndrome – Effects of Estrogen Replacement Therapy

10.21203/rs.3.rs-201313/v1 ◽

2021 ◽

Author(s):

Fedor István ◽

Eva Zold ◽

Zsolt Barta

Keyword(s):

Replacement Therapy ◽

Estrogen Replacement Therapy ◽

Inflammatory Diseases ◽

Hormone Replacement ◽

Turner's Syndrome ◽

Estrogen Replacement ◽

Turner’S Syndrome ◽

Estrogen Production ◽

Organ Systems ◽

Increased Risk

Abstract BackgroundTurner’s syndrome is one of the most frequently reported sex chromosomal abnormality, affecting approximately 40 in every 100,000 live female births. Due to insufficient estrogen production, induction of puberty and sexual development requires hormone replacement. The syndrome affects several organ systems with diverse clinical features (cardiovascular, reproductive, hepato-biliary). There is also an increased risk of developing immune-mediated inflammatory diseases (IMID). Hepatobiliary alterations embrace a broad spectrum of possible manifestations, from asymptomatic mild hypertransaminasemia to overt hepatitis and even cirrhosis. Although exogenous estrogen hormones might cause liver dysfunction, in Turner’s syndrome hormone replacement can even alleviate the derangement of laboratory values and might prove beneficial in preventing the progression of hepatic architectural alterations.FindingsWe report two patients, in whom cessation of estrogen replacement therapy lead to worsening of hepatic and cholestatic enzyme values. These changes were later alleviated by recommencing estrogen hormone administration. We aim to summarize the available literature on estrogen hormone replacement therapy in Turner’s syndrome. We also provide a brief overview on the role of estrogen hormones in the pathology associated with the syndrome. ConclusionsOur findings are confirming, that estrogen replacement therapy has beneficial effects on hepatic enzymes and liver related laboratory studies in Turner’s syndrome. Therefore it is recommended for physicians not to withdraw estrogen replacement, even with elevated concentrations of liver and cholestatic enzymes in Turner’s syndrome patients.

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Periaortic Abscess following DeBakey Type-1 Aortic Dissection Repair with Dacron Graft—Early Diagnosis and Management

Case Reports in Cardiology ◽

10.1155/2019/6915356 ◽

2019 ◽

Vol 2019 ◽

pp. 1-5

Author(s):

Rishi Raj ◽

Dileep Unnikrishnan ◽

Aasems Jacob ◽

Kumar Ashish ◽

Amulya Prakash ◽

...

Keyword(s):

Early Diagnosis ◽

Aortic Dissection ◽

Fluid Collection ◽

Dacron Graft ◽

Surgical Drainage ◽

Aortic Graft ◽

Diagnosis And Management ◽

Increased Risk

A 71-year-old male with history of DeBakey type-1 aortic dissection and repair with dacron graft three months prior to presentation was brought to the emergency room with complaints of high-grade fevers, chills, and shortness of breath. Chest X-ray revealed right lower lobe infiltrates and widened superior mediastinum. A follow-up CT chest with contrast showed fluid collection around the aortic graft. He was started on intravenous broad-spectrum antibiotics, and a TEE was done for further evaluation of periaortic fluid collection which showed findings to suggest periaortic abscess. The patient underwent surgical drainage of the abscess and was found to have an abscess around the surgical aortic graft which was drained followed by two weeks of antibiotic treatment. The patient was discharged to a rehabilitation facility and remained asymptomatic at three-month follow-up appointment. Type-1 aortic dissection is a medical emergency requiring acute surgical intervention, and despite significant advancements in diagnosis and management, the immediate and long-term complications remain high leading to increased risk of mortality. Our patient developed spontaneous periaortic abscess three months postoperatively requiring intensive antibiotic therapy along with surgical drainage. Our case emphasizes the importance of early diagnosis and management of late complications of periaortic abscess in patients with aortic dissection repair.

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