scholarly journals Aortic arch tortuosity, a novel biomarker for thoracic aortic disease, is increased in adults with bicuspid aortic valve

2019 ◽  
Vol 284 ◽  
pp. 84-89 ◽  
Author(s):  
Bader Aldeen Alhafez ◽  
Van Thi Thanh Truong ◽  
Daniel Ocazionez ◽  
Sahand Sohrabi ◽  
Harleen Sandhu ◽  
...  
2015 ◽  
Vol 4 (1) ◽  
Author(s):  
Gibran Minero ◽  
Simon C. Body

As the most common congenital heart defect, understanding the etiology and progression of aortopathy in bicuspid aortic valve (BAV) is imperative to management of patients with BAV. A reasonable hypothesis, based on the strength of evidence for both genetic and hemodynamic causes of BAV-associated thoracic aortic disease (TAD), is that BAV is caused by genetic variant(s) that also predispose to TAD by a common mechanism; presumably by cell-signaling resulting in an embryologic defect that causes BAV and a postnatal risk of TAD that is accentuated by hemodynamic stress of abnormal flow through the BAV valve. Clinical heterogeneity seen in BAVassociated TAD is likely due to individual genetic variation and the severity of hemodynamic alteration.


2020 ◽  
Vol 5 (04) ◽  
pp. 331-334
Author(s):  
Paolo Masiello ◽  
Rocco Leone ◽  
Rossella Maria Benvenga ◽  
Severino Iesu

Abstract Background Type A aortic dissection is an emergency with high morbidity and mortality when surgery is not performed. Few cases are described in the literature about aortic dissection during pregnancy. A correlation between pregnancy and aortic dissection is mainly reported in patients with family history and connective tissue disorders, such as Marfan’s syndrome (MS), Loeys–Dietz’s syndrome, and Ehlers–Danlos’s syndromes, and patients with bicuspid aortic valve (BAV); exceptional cases are also described in patients without risk factors. Case presentation A 22-year-old young woman with MS, ascending aorta dilation, and BAV became pregnant. During labor, she experienced a short-term chest pain with spontaneous resolution. The electrocardiogram (ECG) and cardiac biomarkers were negative for acute coronary artery disease, but no transthoracic echocardiogram (TTE) was performed. A caesarean section was performed without complications. After 1 month, a routine TTE showed a chronic ascending aortic dissection involving the aortic arch and supra-aortic vessels. Due to a normally functioning aortic valve, the David operation was performed (sparing aortic valve) with the replacement of the aortic arch and supra-aortic vessels. Conclusions Aortic dissection is a rare cardiovascular complication that can occur during pregnancy and is associated with very high-risk mortality. We have reported a rare case of undiagnosed type A aortic dissection involving the aortic arch during unplanned pregnancy in patients with BAV and MS, subsequently treated with the David surgery and replacement of ascending aortic arch and supra-aortic vessels. A closer clinical and instrumental follow-up is necessary in this particular group of patients at risk. Awareness of all physicians involved is mandatory.


Cardiology ◽  
2012 ◽  
Vol 123 (2) ◽  
pp. 116-124 ◽  
Author(s):  
Matthew Hornick ◽  
Remo Moomiaie ◽  
Hamid Mojibian ◽  
Bulat Ziganshin ◽  
Zakaria Almuwaqqat ◽  
...  

2020 ◽  
Vol 26 (4) ◽  
pp. 4-12
Author(s):  
A.А. Malska ◽  
◽  
O.B. Kuryliak ◽  

Aim. To determine the rate of aortic coarctation, the correlation of its anatomical forms - critical and not critical, and the frequency of combination with the associated pathology; to define the features of the clinical course of its different anatomical forms; and to analyze the remote results of the surgical correction of this defect. Material and Methods. The article represents the statistical analysis of outpatient medical records and case histories of 86 children with aortic coarctation in Lviv region. In the course of the research, retrospective and epidemiological studies were carried out; clinical (data acquisition of medical history, physical examination), instrumental (Doppler echocardiography, ECG, X-ray imaging of organs of the thoracic cavity), and statistical methods were used. Results and Discussion. It was determined that over the period of 2008-2020 years, out of 74 neonates with CoA registered at Lviv Regional Children's Hospital (Health Care of Mother and Child) 40,54% had the critical CoA form, while 59 (46%) - uncritical CoA form. In children with the critical CoA form, the most frequent findings were hypoplasia of the aortic arch (56,67%), open aortic duct (53,33%), and open oval window (53,33%); in 36,6% cases CoA was combined with the bicuspid aortic valve, interatrial septal defect, and transposition of great vessels. However, the uncritical CoA form was more frequently combined with the bicuspid aortic valve (52,27%), and hypoplastic aortic arch (31,82%); aortic stenosis was revealed in 20,45% of children. After the surgical correction in 43,59% of the operated patients with uncritical CoA, excessive arterial hypertension was observed, while in critical form, the frequency of excessive arterial hypertension among the operated patients amounted to 10%. According to our research, after the plasty of the critical CoA, aortic recoarctation was observed in 3.33% of the operated patients, whereas, in case of the uncritical CoA form, it occurred in 30,77% of the operated ones. Conclusions. Coarctation of the aorta is a congenital heart disease with relatively high incidence, amounting to 5-8% out of all congenital defects of the heart. In newborns, it is manifested by acute cardiac failure, while in elder children it is presented with arterial hypertension. Echocardiographic examination after Doppler analysis is the basic procedure of diagnostics and allows for precise determination of the CoA anatomy. The majority of cardiologists recommend prompt surgical intervention after the diagnosis has been made, and, particularly, in patients with hypertension. At present, the available surgical methods include surgical excision of the aortic obstruction, and catheter intervention (the balloon angioplasty and stent implantation) . After the surgical correction has been performed, arterial hypertension persists. The duration of hypertension after the coarctation correction depends on its duration before the diagnosis is made and the timing of surgical correction of the defect. With the child's growth , recoarctation may occur. In such patients, normal arterial pressure can be determined in the state of rest, but it may increase in the upper extremities during physical exertion. Key words: aortic coarctation, associated pathology, excessive arterial hypertension, recoarctation


2014 ◽  
Vol 34 (suppl_1) ◽  
Author(s):  
Sandy von Salisch ◽  
Josephina Haunschild ◽  
Martin Misfeld ◽  
Michael A Borger ◽  
Stefan Dhein ◽  
...  

Background: Bicuspid aortic valve is the most frequent congenital cardiac abnormality and associated with proximal aortic disease (i.e. aneurysm, dissection or rupture). Granular media calcinosis(GMC)--suggested to increase stiffness and play a pathogenetic role in dissecting aneurysm--has not yet been quantified in BAV. Methods: Specimen of the proximal aortic wall from 76 patients--32 with tricuspid (TAV) and 44 with bicuspid aortic valve (BAV)--were obtained during surgery to quantify media thickness and GMC by von Kossa staining (panel C), comparing the convexity (Cvx) and concavity (Ccv) in BAV vs. TAV. Results: Interlamellar GMC affected the most central layers of the media and those adjacent to the outer adventitia with a doubling within both--the Cvx and Ccv--of pts with BAV compared to patients with TAV (13.3±9.6 vs. 6.6±7.4 and 12.8±10.8 vs. 6.4±7.1; p<0.05, panel A) was seen, but neither a difference in calcification between the Ccx and the Ccv side within the BAV nor the TAV group. No association between age and calcification grade , neither in the Cvx nor the Ccv (r=0.132, p=0.218 and 0.103, p=0.341) was seen. There was a significant difference in the total media thickness between BAV and TAV at the Cvx (867±162μm vs . 993±158μm; p<0.05) and the Ccv (1005 ± 236 vs 1223 ± 217μm; p<0.05, panel B). Independent of aortic valve morphology, the Cvx was thinner than the Ccv side (TAV: 993 ± 158 vs.1223 ± 217μm; p<0.001; BAV: 869 ± 162 vs.1005 ± 236μm; p<0.05, panel B). Conclusion: BAVs had significantly thinner media and twice as much GMC than their tricuspid peers possibly associated with the loosening of the bond between the elastic lamellae causing a decrease in elasticity possibly explaining a higher risk for dissection and rupture.


2012 ◽  
Vol 13 (7) ◽  
pp. 460-461
Author(s):  
Antonios N. Pavlidis ◽  
Athanasios Tsoukas ◽  
Peter G. Danias ◽  
Manolis S. Kallistratos ◽  
Ioannis Levantakis ◽  
...  

Author(s):  
Massimiliano M Marrocco-Trischitta ◽  
Mattia Glauber

Abstract Contemporary guidelines on thoracic aortic repair provide inconsistent reporting standards for the definition of aortic arch classification in Types I, II and III. The different reported criteria cannot be used interchangeably, due to a very low level of concordance, and this finding has relevant implications for the comparisons between studies using different classifications, and between different datasets of multicentre trials, which are not consistently analyzed with the same criteria. Also, the reported definitions, which were originally proposed for predicting difficult carotid stenting and therefore were conceived for healthy aortic arches, can be influenced by the pathological derangements of the aortic wall, including aneurysms and dissections. In this respect, the Madhwal’s classification, which is based on the diameter of the left common carotid artery, appears to be the more suitable one for aortic arch classification in patients with thoracic aortic disease because it provides relevant clinical information along with an adequate reproducibility.


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