390 results on '"aortic dilation"'
Search Results
352. BMI is a Significant Predictor of Cost and Resource Utilization in Diabetic Patients Undergoing CABGS
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Amy Wilson-O'Brien, H. Rizzoli, Andrew Newcomb, S. Bappayya, Diem Dinh, David A Reid, and Y. Tsai
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musculoskeletal diseases ,Pulmonary and Respiratory Medicine ,congenital, hereditary, and neonatal diseases and abnormalities ,medicine.medical_specialty ,Younger age ,business.industry ,Mortality rate ,macromolecular substances ,medicine.disease ,Dissection ,Bicuspid aortic valve ,Internal medicine ,medicine ,Cardiology ,cardiovascular diseases ,skin and connective tissue diseases ,Cardiology and Cardiovascular Medicine ,business ,Aortic dilation ,Resource utilization ,Mace - Abstract
up and echo. Diagnosis ofMFS byGhent criteria. Bicuspid aortic valve (BAV) diagnosed by echo.Mean follow-upwas 10± 8 years. Results: Patients with non-MFS TAAD presented later but had similar severity of aortic dilation. Major adverse cardiac events (MACE=dissection, surgery or death)were similar in MFS and non-MFS TAAD but first MACE occurred at younger age in MFS. Death rates and age of death were comparable between MFS and non-MFS TAAD. MFS +ve BAV +ve MFS/BAV −ve p
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- 2011
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353. Acetylcholine-Stimulated Aortic Dilation is Impaired by Diesel Particulate Exposed Macrophages; Investigation of Susceptibility in Mice with Compromised Glutathione Synthesis
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Ian Luttrell, Vicki Morgan, Dave Cox, Timothy V. Larson, Terrance J. Kavanagh, Chad S. Weldy, Francis Kim, James A. Stewart, and Kanchan Chitaley
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medicine.medical_specialty ,Chemistry ,Glutathione synthesis ,Particulates ,Biochemistry ,Diesel fuel ,Endocrinology ,Physiology (medical) ,Internal medicine ,Immunology ,medicine ,Aortic dilation ,Acetylcholine ,medicine.drug - Published
- 2010
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354. Association of aortic dilation with regurgitant, stenotic and functionally normal bicuspid aortic valves
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Richard B. Devereux, Rebecca T. Hahn, Allen H. Mogtadek, and Mary J. Roman
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Aortic valve ,Adult ,Male ,medicine.medical_specialty ,Developmental defect ,Aortic root ,Aortic Valve Insufficiency ,Hemodynamics ,Bicuspid aortic valve ,Internal medicine ,medicine.artery ,Ascending aorta ,Prevalence ,Medicine ,Humans ,Aortic dilation ,Aorta ,business.industry ,Aortic Valve Stenosis ,Middle Aged ,medicine.disease ,Control subjects ,medicine.anatomical_structure ,Echocardiography ,Aortic Valve ,Cardiology ,cardiovascular system ,Female ,Cardiology and Cardiovascular Medicine ,business ,Dilatation, Pathologic - Abstract
To determine whether aortic root dilation associated with a bicuspid aortic valve occurs independently of valvular hemodynamic abnormality, aortic root dimensions were measured by two-dimensional echocardiography in 83 adults with a functionally normal (n = 19), mildly regurgitant (n = 26), severely regurgitant (n = 27) or stenotic (n = 11) bicuspid aortic valve and compared with findings in normal subjects matched for age and gender. Aortic root measurements were made at four levels: anulus, sinuses of Valsalva, supraaortic ridge and proximal ascending aorta. Seventy-one percent of patients with a bicuspid aortic valve were men. When compared with control subjects, all hemodynamic subgroups showed a significantly larger aortic root size at three levels: sinuses of Valsalva, supraaortic ridge and proximal ascending aorta (p less than 0.05 to p less than 0.001). The prevalence of aortic root enlargement among all hemodynamic subgroups ranged from 9% to 59% at the level of the anulus, 36% to 78% at the sinuses, 47% to 79% at the supraaortic ridge and 50% to 64% in the ascending aorta. Thus, there is a high prevalence of aortic root enlargement in patients with a bicuspid aortic valve that occurs irrespective of altered hemodynamics or age. These findings support the hypothesis that bicuspid aortic valve and aortic root dilation may reflect a common developmental defect.
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- 1992
355. Failure to Recognize Newly Identified Aortic Dilations in a Health Care System With an Advanced Electronic Medical Record
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Iraklis I. Pipinos, Terry L. Wahls, Jennifer R.S. Gordon, Peter Cram, Ruth C. Carlos, and Gary E. Rosenthal
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Male ,medicine.medical_specialty ,Time Factors ,Medical Records Systems, Computerized ,Computed tomography ,Documentation ,Kaplan-Meier Estimate ,Cohort Studies ,Aneurysm ,medicine.artery ,Health care ,Internal Medicine ,Humans ,Medicine ,Aortic dilation ,Aged ,Retrospective Studies ,Aged, 80 and over ,Aorta ,medicine.diagnostic_test ,Notice ,business.industry ,Electronic medical record ,Magnetic resonance imaging ,General Medicine ,Middle Aged ,medicine.disease ,Surgery ,Female ,Radiology ,Tomography, X-Ray Computed ,business ,Aortic Aneurysm, Abdominal ,Dilatation, Pathologic - Abstract
Concern is growing about missed test results, but data assessing their effect on patient safety are limited.To examine the frequency with which computed tomography (CT)-documented dilations of the abdominal aorta are accompanied by evidence in the electronic medical record (EMR) that a clinician recognized the abnormality.Retrospective cohort study.2 hospitals in the Veterans Affairs Health Care System.Patients with new dilations of the abdominal aorta detected on CT performed in 2003.Radiology report and EMR evidence that the radiologist notified the clinical service, aneurysm size, and interval between CT and EMR recognition.Computed tomography scans of 4112 patients were reviewed and 440 (11%) aortic dilations were identified, of which 91 were new findings. Radiologists directly notified clinical teams about 5 (5%) new dilations. Clinical teams did not record in the EMR recognition of 53 of 91 (58%) dilations within 3 months of the CT, and 9% of these dilations were 5.5 cm or larger. The median time to recognition of aneurysm in the EMR was 237 days, and no EMR documentation existed for 16 abnormalities (29% of surviving patients) during a mean follow-up of 3.2 years. No evidence indicated that any of the aneurysms ruptured or that patient deaths resulted from the delayed follow-up.Clinicians may have recognized some aneurysms but did not document them in the EMR.Clinicians neglect to note a substantial proportion of new aortic dilations in the EMR. The findings highlight the need for better strategies to ensure documentation of follow-up of tests.
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- 2009
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356. ASSESSMENT OF THORACIC AORTIC DILATION: IS ECHOCARDIOGRAPHY AS GOOD AS MAGNETIC RESONANCE IMAGING?
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H.H. Hart, G. Armstrong, Tony Scott, K Wong, Colin Edwards, Hitesh Patel, and Jonathan P. Christiansen
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Pulmonary and Respiratory Medicine ,medicine.medical_specialty ,medicine.diagnostic_test ,business.industry ,medicine ,Magnetic resonance imaging ,Radiology ,Cardiology and Cardiovascular Medicine ,Aortic dilation ,business - Published
- 2008
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357. 885 Aortic dilation and dissection in patient after aortic valve replacement because of a bicuspid aortic valve
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R. B. A. van den Brink, B.J. Bouma, M. Han, A. Van DerWal, and B. A. J. M. de Mol
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medicine.medical_specialty ,business.industry ,General Medicine ,Dissection (medical) ,medicine.disease ,Bicuspid aortic valve ,Aortic valve replacement ,Internal medicine ,Cardiology ,Medicine ,Radiology, Nuclear Medicine and imaging ,In patient ,Cardiology and Cardiovascular Medicine ,business ,Aortic dilation - Published
- 2006
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358. David-V Procedure in a Patient with Aortic Dilation and Competent Quadricuspid Aortic Valve: Are Genetics to Blame?
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Hebeler KR, Squiers JJ, Baumgarten H, DiMaio JM, and Brinkman WT
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Quadricuspid aortic valves (QAVs) are extremely rare. In this case study, we report a David-V valve-sparing aortic root replacement with reimplantation of a native QAV in a patient with aortic dilation, normal valve function, and a family history of aortic dissection. Microscopic pathological examination of the excised section of the aorta revealed scattered small foci of cystic medical degeneration throughout. A genetic predisposition for aortic dilation may be present in patients with QAV, even in the setting of a competent valve. Regular screening for aortic dilation in patients with known QAV should therefore be considered.
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- 2016
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359. Circulating endothelial microparticles are elevated in bicuspid aortic valve disease and related to aortic dilation.
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Alegret JM, Martínez-Micaelo N, Aragonès G, and Beltrán-Debón R
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- Adult, Aortic Diseases metabolism, Aortic Valve metabolism, Aortic Valve pathology, Bicuspid Aortic Valve Disease, Biomarkers blood, Dilatation, Female, Heart Valve Diseases metabolism, Humans, Male, Aortic Diseases pathology, Aortic Valve abnormalities, E-Selectin blood, Heart Valve Diseases pathology, Platelet Endothelial Cell Adhesion Molecule-1 blood
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Background/objectives: The mechanisms underlying aortic dilation in bicuspid aortic valve (BAV) disease are unknown. Circulating endothelial microparticles (EMPs) have emerged as biomarkers of endothelial damage. We sought to evaluate the relationships among EMPs, BAV disease, and aortic dilation., Methods: Four evaluations were used. Circulating EMPs (PECAM(+), E-selectin(+)) were compared between BAV patients and tricuspid aortic valve (TAV) control subjects. The variables related to circulating EMPs were investigated in BAV patients. Circulating EMP levels were compared between BAV and TAV patients with a dilated aorta. Finally, circulating EMPs in BAV patients were evaluated over time with respect to aortic valve surgery (AVS) or aortic surgery., Results: We observed higher levels of circulating PECAM(+) EMPs in the BAV patients than in the control subjects (3.98±0.2 vs. 2.39±0.4 per log PECAM(+) EMPs/μl, p=0.001). Aortic dilation was the most significant variable that correlated with the PECAM(+) EMP levels in the BAV patients (β=0.321, p=0.008). The BAV patients with aortic dilation exhibited higher PECAM(+)EMP levels than the TAV patients with dilated aortas, and this correlation was independent of aortic valve function. We observed a drastic decrease in the circulating PECAM(+) EMPs following AVS and aortic root replacement (4.27±0.6 and 1.75±0.3 per log PECAM(+)EMPs/μl, p=0.002)., Conclusion: The observed pattern of higher circulating PECAM(+) EMP levels links BAV disease to endothelial damage and aortic dilation. Circulating PECAM(+) EMPs were identified as a biological variable related to aortic dilation in patients with BAV disease., (Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.)
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- 2016
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360. [Marfan syndrome in childhood and adolescence].
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Magotteaux S, Bulk S, Farhat N, Sakalihasan N, Defraigne JO, and Seghaye MC
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- Adolescent, Age Factors, Child, Female, Humans, Infant, Male, Marfan Syndrome genetics, Marfan Syndrome pathology, Marfan Syndrome therapy, Marfan Syndrome diagnosis
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The Marfan syndrome is a systemic connective tissue disorder with autosomal dominant inheritance. A mutation of the fibrillin-1 gene, a glycoprotein which is the main constituent of the extracellular matrix, is the cause of the disease. The cardinal features involve the skeletal, ocular and cardiovascular systems. The expression of the Marfan syndrome varies from the severe neonatal presentation to the classical manifestations of the child and young adult, but also comprises isolated features. In children, phenotypical manifestations are age dependent. For these reasons, the diagnosis of Marfan syndrome might be lately revealed by its cardiovascular complications. We report the case of 2 siblings: it illustrates the phenotypic variability that might be observed in a same family, the phenotype evolution with age and the diagnosis challenge in childhood.
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- 2016
361. Analysis of TGFBR1*6A variant in individuals evaluated for Marfan syndrome.
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Somers AE, Hinton RB, Pilipenko V, Miller E, and Ware SM
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- Adolescent, Adult, Aged, Alleles, Child, Child, Preschool, Female, Fibrillin-1 genetics, Genetic Association Studies, Humans, Infant, Loeys-Dietz Syndrome pathology, Male, Marfan Syndrome pathology, Middle Aged, Mutation genetics, Pedigree, Penetrance, Receptor, Transforming Growth Factor-beta Type I, Young Adult, Loeys-Dietz Syndrome genetics, Marfan Syndrome genetics, Protein Serine-Threonine Kinases genetics, Receptors, Transforming Growth Factor beta genetics
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Marfan syndrome (MFS) and Loeys-Dietz syndrome (LDS) are genetic disorders that affect connective tissue as a result of dysregulated TGF-β signaling. MFS is most frequently caused by mutations in FBN1 whereas Loeys-Dietz syndrome results from mutations in TGFBR1 or TGFBR2. There is substantial inter- and intra-familial phenotypic variability among these disorders, suggesting the presence of genetic modifiers. Previously, a polymorphism in the TGFβR1 protein termed the TFGBR1*6A allele was found to be overrepresented in patients with MFS and was identified as a low penetrance allele with suggestion as a possible modifier. To further investigate the importance of this variant, a retrospective review of genetic and phenotypic findings was conducted for 335 patients evaluated for suspicion of MFS or related disorders. In patients with a diagnosis of MFS, the presence of the TFGBR1*6A allele was not associated with phenotypic differences. Similarly, careful phenotyping of patients who carried the TFGBR1*6A allele but did not have MFS did not identify an altered frequency of specific connective tissue features. In this small cohort, the results did not reach significance to identify the TFGBR1*6A allele as a major modifier for aortic dilation, ectopia lentis, or systemic features associated with MFS or other connective tissue disorders. © 2016 Wiley Periodicals, Inc., (© 2016 Wiley Periodicals, Inc.)
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- 2016
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362. Mechanism of aortic root dilation and cardiovascular function in tetralogy of Fallot.
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Seki M, Kuwata S, Kurishima C, Nakagawa R, Inuzuka R, Sugimoto M, Saiki H, Iwamoto Y, Ishido H, Masutani S, and Senzaki H
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- Aorta physiopathology, Dilatation, Pathologic, Humans, Tetralogy of Fallot genetics, Tetralogy of Fallot physiopathology, Aorta pathology, Tetralogy of Fallot pathology, Vascular Stiffness
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The aortic root dilation in tetralogy of Fallot (TOF) is a long-term clinical problem, because a severely dilated aorta can lead to aortic regurgitation, dissection, or rupture, which can be fatal, necessitating surgical intervention. The details of the mechanism of aortic root dilation, however, are unclear. We have shown that aortic stiffness is increased in patients with repaired TOF, and may mirror the histological abnormality of elastic fiber disruption and matrix expansion. This aortic stiffness is related closely to the aortic dilation, indicating that aortic stiffness may be a predictor of outcome of aortic dilation. Furthermore, the aortic volume overload is a very important determinant of aortic diameter in TOF patients before corrective surgery. In addition, a chromosomal abnormality and the transforming growth factor-β signaling pathway, a major contributor to aortic dilation in Marfan syndrome, also affect this mechanism. In this way, aortic dilation in TOF patients is suggested to be a multifactorial disorder. The aim of this review was therefore to clarify the mechanism of aortic dilation in TOF, focusing on recent research findings. Studies linking histopathology, mechanical properties, molecular/cellular physiology, and clinical manifestations of aortic dilation facilitate appropriate treatment intervention and improvement of long-term prognosis of TOF., (© 2016 Japan Pediatric Society.)
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- 2016
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363. Aortic Measurements in Patients with Aortopathy are Larger and More Reproducible by Cardiac Magnetic Resonance Compared with Echocardiography.
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Nejatian A, Yu J, Geva T, White MT, and Prakash A
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- Adolescent, Adult, Aorta diagnostic imaging, Aortic Valve abnormalities, Aortic Valve diagnostic imaging, Bicuspid Aortic Valve Disease, Child, Child, Preschool, Connective Tissue Diseases diagnostic imaging, Female, Heart Valve Diseases diagnostic imaging, Humans, Male, Middle Aged, Observer Variation, Reproducibility of Results, Retrospective Studies, Young Adult, Aorta physiopathology, Aortography, Echocardiography, Magnetic Resonance Imaging, Cine
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Accurate and reproducible aortic measurements are essential in aortopathy patients. Transthoracic echocardiography (TTE) is commonly used but has several limitations. Cardiac magnetic resonance (CMR) can offset these limitations but has not been directly compared with TTE. We compared the reproducibility of CMR and TTE measurements at multiple aortic levels. Patients with a connective tissue disorder (CTD) or bicommissural aortic valve (BAV) (n = 41; 22 CTD, 19 BAV; mean age 18.8 ± 8.9 years) with TTE and CMR imaging performed within 3 months of each other were randomly selected. Two blinded observers measured the aorta at multiple anatomic levels. Intra- and interobserver variability and agreement between techniques were assessed. Aortic root diameter measurements by TTE and CMR were equally reproducible (% error 4-10 %), but TTE measurements were systematically smaller by 5-7 % (p < 0.0001). Systematic differences were larger in BAV (11-12 %, p < 0.0001) due to root asymmetry. CMR measurements of aortic root cross-sectional area were feasible and highly reproducible (% error 5-8 %). Compared with CMR, ascending aorta measurements by TTE were less reproducible, especially in BAV (% error 21-24 vs. 6-7 %, p = 0.01). Distal aortic measurements by TTE were 14-29 % smaller and had poor reproducibility compared with CMR (% error 24-42 vs. 9-10 %; p < 0.0001). CMR measurement of the largest aortic root dimension is more reliable than TTE, especially when the root is asymmetric. Measurements of the thoracic aorta distal to the root by CMR are more accurate and reproducible than by TTE. These data support a role for CMR in aortopathy patients.
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- 2015
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364. Aortic dilation in pediatric patients.
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Zarate YA, Sellars E, Lepard T, Carlo WF, Tang X, and Collins RT 2nd
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- Adolescent, Aortic Diseases complications, Aortic Valve abnormalities, Aortic Valve pathology, Bicuspid Aortic Valve Disease, Child, Child, Preschool, Diagnosis, Differential, Dilatation, Pathologic complications, Dilatation, Pathologic diagnosis, Echocardiography, Female, Heart Defects, Congenital diagnosis, Heart Valve Diseases pathology, Humans, Infant, Infant, Newborn, Male, Prevalence, Retrospective Studies, Risk Factors, Aortic Diseases diagnosis
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Aortic dilation at the level of the aortic root can be caused by a variety of congenital or acquired conditions that lead to weakening of the aortic wall. In this retrospective study, we sought to determine the frequency of different associated diagnoses from children with aortic dilation seen at a single institution. A total of 377 children (68 % male) met study inclusion criteria. Patients were classified based on the suspected or confirmed associated diagnosis in one of the following categories: congenital heart disease (241/377, 64 %), chromosomal (34/377, 9 %), Marfan syndrome (26/377, 7 %), other genetic and non-genetic (22/377, 6 %), Loeys-Dietz syndrome (6/377, 2 %), and unknown (48/377, 13 %). Bicuspid aortic valve was by far the most prevalent congenital heart defect (206/241, 85 %), while Turner syndrome was the most frequent chromosomal abnormality (12/34, 35 %). Patients with Marfan syndrome were more likely to have severe dilation of the ascending aorta (p = 0.002) and to require aortic root replacement surgery (p < 0.001) compared to those in other diagnosis categories., Conclusion: The differential diagnosis of aortic dilation is broad and requires a careful assessment of cardiac anatomy. Evaluation by a clinical geneticist in this setting should be strongly considered given the high frequency of associated genetic conditions., What Is Known: • Aortic dilation is frequent in bicuspid aortic valve and other congenital heart defects. • Aortic dilation can be seen in several connective tissue disorders. Limited information is available in regard to the differential diagnosis of aortic dilation in children., What Is New: • In patients with aortic dilation concurrent congenital heart disease is frequently diagnosed. • Almost 18 % of cases in the present study had a defined presumptive or confirmed genetic diagnosis. We suggest considering a genetics evaluation in the setting of aortic dilation.
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- 2015
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365. 7q11.23 Duplication syndrome: Physical characteristics and natural history.
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Morris CA, Mervis CB, Paciorkowski AP, Abdul-Rahman O, Dugan SL, Rope AF, Bader P, Hendon LG, Velleman SL, Klein-Tasman BP, and Osborne LR
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- Adolescent, Child, Child, Preschool, Chromosomes, Human, Pair 7, Developmental Disabilities etiology, Developmental Disabilities genetics, Face abnormalities, Female, Humans, Infant, Male, Megalencephaly, Pregnancy, Pregnancy Complications genetics, Williams Syndrome genetics, Young Adult, Williams Syndrome etiology
- Abstract
In order to describe the physical characteristics, medical complications, and natural history of classic 7q11.23 duplication syndrome [hereafter Dup7 (MIM 609757)], reciprocal duplication of the region deleted in Williams syndrome [hereafter WS (MIM 194050)], we systematically evaluated 53 individuals aged 1.25-21.25 years and 11 affected adult relatives identified in cascade testing. In this series, 27% of probands with Dup7 had an affected parent. Seven of the 26 de novo duplications that were examined for inversions were inverted; in all seven cases one of the parents had the common inversion polymorphism of the WS region. We documented the craniofacial features of Dup7: brachycephaly, broad forehead, straight eyebrows, broad nasal tip, low insertion of the columella, short philtrum, thin upper lip, minor ear anomalies, and facial asymmetry. Approximately 30% of newborns and 50% of older children and adults had macrocephaly. Abnormalities were noted on neurological examination in 88.7% of children, while 81.6% of MRI studies showed structural abnormalities such as decreased cerebral white matter volume, cerebellar vermis hypoplasia, and ventriculomegaly. Signs of cerebellar dysfunction were found in 62.3%, hypotonia in 58.5%, Developmental Coordination Disorder in 74.2%, and Speech Sound Disorder in 82.6%. Behavior problems included anxiety disorders, ADHD, and oppositional disorders. Medical problems included seizures, 19%; growth hormone deficiency, 9.4%; patent ductus arteriosus, 15%; aortic dilation, 46.2%; chronic constipation, 66%; and structural renal anomalies, 18%. We compare these results to the WS phenotype and offer initial recommendations for medical evaluation and surveillance of individuals who have Dup7., (© 2015 Wiley Periodicals, Inc.)
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- 2015
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366. Effect of statins on aortic root growth rate in patients with bicuspid aortic valve anatomy.
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Regeer MV, van Rosendael PJ, Kamperidis V, Schalij MJ, Bax JJ, Marsan NA, and Delgado V
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- Adult, Aged, Aorta diagnostic imaging, Aortic Aneurysm diagnostic imaging, Aortic Aneurysm etiology, Aortic Valve diagnostic imaging, Aortic Valve drug effects, Aortic Valve growth & development, Bicuspid Aortic Valve Disease, Dilatation, Pathologic, Echocardiography, Doppler, Color, Echocardiography, Doppler, Pulsed, Female, Heart Valve Diseases complications, Heart Valve Diseases diagnostic imaging, Humans, Male, Middle Aged, Retrospective Studies, Time Factors, Treatment Outcome, Aorta drug effects, Aortic Aneurysm prevention & control, Aortic Valve abnormalities, Hydroxymethylglutaryl-CoA Reductase Inhibitors therapeutic use
- Abstract
Bicuspid aortic valve (BAV) anatomy is associated with increased growth rate of the aortic root compared to tricuspid aortic valves. Statins decrease the growth rate of abdominal aneurysms; however their effect on the aortic root growth rate has not been elucidated. The present study evaluated the association between use of statins and aortic root growth in patients with BAV. A total of 199 patients (43 ± 15 years, 69% male) with BAV who underwent ≥ 2 echocardiographic measurements of the aortic root ≥ 1 year apart were included in this retrospective observational study. Median follow-up duration was 4.7 years (interquartile range 2.7-8.3 years). Growth rate (mm/year) of the aortic root was compared between statin users (n = 41) and non-users (n = 158). Statin users were significantly older and had more cardiovascular risk factors than their counterparts. Ascending aorta diameter was significantly smaller at baseline and at follow-up in statin users compared with non-users when adjusted for coronary artery disease, age and medication. The average annual growth rate was 0.08 mm/year (95% confidence interval 0.03-0.13) for the aortoventricular junction, 0.16 mm/year (0.11-0.21) for the sinus of Valsalva, 0.12 mm/year (0.07-0.17) for the sinotubular junction and 0.45 mm/year (0.37-0.53) for the ascending aorta. The dilation rate of the aortic segments was not different between statin users and non-users. In conclusion, in patients with BAV, although the use of statins was associated with smaller ascending aorta, the annual dilation rate of the aortic root was not influenced by the use of statins.
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- 2015
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367. Assessment of altered three-dimensional blood characteristics in aortic disease by velocity distribution analysis.
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Garcia J, Barker AJ, van Ooij P, Schnell S, Puthumana J, Bonow RO, Collins JD, Carr JC, and Markl M
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- Adult, Aged, Aortic Diseases physiopathology, Female, Humans, Male, Middle Aged, Aortic Diseases pathology, Blood Flow Velocity physiology, Cardiac Imaging Techniques methods, Imaging, Three-Dimensional methods, Magnetic Resonance Imaging, Cine methods
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Purpose: To test the feasibility of velocity distribution analysis for identifying altered three-dimensional (3D) flow characteristics in patients with aortic disease based on 4D flow MRI volumetric analysis., Methods: Forty patients with aortic (Ao) dilation (mid ascending aortic diameter MAA = 40 ± 7 mm, age = 56 ± 17 years, 11 females) underwent cardiovascular MRI. Four groups were retrospectively defined: mild Ao dilation (n = 10; MAA < 35 mm); moderate Ao dilation (n = 10; 35 < MAA < 45 mm); severe Ao dilation (n = 10; MAA > 45 mm); Ao dilation+aortic stenosis AS (n = 10; MAA > 35 mm and peak velocity > 2.5 m/s). The 3D PC-MR angiograms were computed and used to obtain a 3D segmentation of the aorta which was divided into four segments: root, ascending aorta, arch, descending aorta. Radial chart displays were used to visualize multiple parameters representing segmental changes in the 3D velocity distribution associated with aortic disease., Results: Changes in the velocity field and geometry between cohorts resulted in distinct hemodynamic patterns for each aortic segment. Disease progression from mild to Ao dilation + AS resulted in significant differences (P < 0.05) in flow parameters across cohorts and increased radial chart size for root and ascending aorta segments by 146% and 99%, respectively., Conclusion: Volumetric 4D velocity distribution analysis has the potential to identify characteristic changes in regional blood flow patterns in patients with aortic disease., (© 2014 Wiley Periodicals, Inc.)
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- 2015
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368. Segmental Aortic Stiffness in Children and Young Adults With Connective Tissue Disorders: Relationships With Age, Aortic Size, Rate of Dilation, and Surgical Root Replacement.
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Prakash A, Adlakha H, Rabideau N, Hass CJ, Morris SA, Geva T, Gauvreau K, Singh MN, and Lacro RV
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- Adolescent, Adult, Age Factors, Aorta surgery, Aortic Valve pathology, Aortic Valve surgery, Child, Child, Preschool, Female, Humans, Infant, Magnetic Resonance Angiography, Magnetic Resonance Imaging, Cine, Male, Middle Aged, Retrospective Studies, Young Adult, Aorta pathology, Connective Tissue Diseases pathology, Vascular Stiffness physiology
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Background: Aortic diameter is an imperfect predictor of aortic complications in connective tissue disorders (CTDs). Novel indicators of vascular phenotype severity such as aortic stiffness and vertebral tortuosity index have been proposed. We assessed the relation between aortic stiffness by cardiac MRI, surgical root replacement, and rates of aortic root dilation in children and young adults with CTDs., Methods and Results: Retrospective analysis of cardiac MRI data on children and young adults with a CTD was performed to derive aortic stiffness measures (strain, distensibility, and β-stiffness index) at the aortic root, ascending aorta, and descending aorta. Vertebral tortuosity index was calculated as previously described. Rate of aortic root dilation before cardiac MRI was calculated as change in echocardiographic aortic root diameter z score per year. In 83 CTD patients (median age, 24 years; range, 1-55; 17% <18 years of age; 60% male), ascending aorta distensibility was reduced in comparison with published normative values: median z score, -1.93 (range, -8.7 to 1.3; P<0.0001 versus normals). Over a median follow-up period of 2.7 years, there were no aortic dissections or deaths, but 16 of 83 (19%) patients underwent surgical aortic root replacement. In multivariable analysis, lower aortic root strain (P=0.05) and higher vertebral tortuosity index (P=0.01) were independently associated with aortic root replacement. Lower ascending aorta strain (P=0.02) was associated with a higher rate of aortic root dilation., Conclusions: Higher aortic stiffness is associated with higher rates of surgical aortic replacement and aortic root dilation in children and young adults with CTDs., (© 2015 American Heart Association, Inc.)
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- 2015
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369. Diseases of the Aorta in Elite Athletes.
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Iskandar A and Thompson PD
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- Hemodynamics, Humans, Risk Factors, Aortic Diseases diagnosis, Aortic Diseases therapy, Athletes
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Sudden cardiovascular deaths in athletes are rare and only a fraction are due to aortic events. There has been concern that the hemodynamic load during exercise may lead to aortic dilation, but aortic dimensions in endurance and strength-trained athletes are only slightly larger than those in sedentary comparison subjects. The presence of a bicuspid aortic valve without significant valvular dysfunction and normal aortic dimensions should not influence eligibility to practice sport. Patients with genetic syndromes associated with aortopathy generally should be restricted from vigorous sports participation. This article reviews the diagnosis and management of diseases of the aorta in athletes., (Copyright © 2015 Elsevier Inc. All rights reserved.)
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- 2015
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370. Predictors of ascending aortic dilation in bicuspid aortic valve disease: a five-year prospective study.
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Avadhani SA, Martin-Doyle W, Shaikh AY, and Pape LA
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- Adult, Aortic Valve pathology, Aortic Valve Insufficiency pathology, Bicuspid Aortic Valve Disease, Female, Heart Valve Diseases pathology, Humans, Male, Middle Aged, Predictive Value of Tests, Aorta pathology, Aortic Valve abnormalities, Aortic Valve Insufficiency etiology, Dilatation, Pathologic, Heart Valve Diseases diagnosis
- Abstract
Background: Bicuspid aortic valves are associated with aortic dilation and dissection. There is a paucity of prospective studies evaluating changes in aortic size over time in adult subjects with bicuspid aortic valves., Methods: A total of 115 subjects with asymptomatic bicuspid aortic valves were enrolled from 2003 to 2008 and followed prospectively over 5 years. Clinical and family histories, as well as transthoracic echocardiograms, were obtained at baseline, and echocardiograms were performed annually thereafter., Results: The mean age of subjects was 41.8 ± 12.8 years, and 61% were male. Ascending aortic size at baseline averaged 35.5 ± 5.6 mm and increased in 71.1% of subjects (mean, 0.66 ± 0.05 mm/y; range, 0.2-2.3 mm/y) over an average of 4.8 years. In 15.6% of subjects, the rate of change exceeded 1 mm/y. The average rate of ascending aortic dilation for all subjects was 0.47 ± 0.05 mm/y (P < .001). A family history of aortic valve disease was associated with progression in both unadjusted (P = .029) and logistic regression analyses adjusted for age, gender, and body surface area (odds ratio, 13.7; P = .021). Multivariate analysis did not find leaflet orientation or moderate to severe aortic valve dysfunction as independent predictors of aortic dilation., Conclusions: We found that in subjects with bicuspid aortic valve, studied prospectively, there was an annual rate of ascending aortic dilation of 0.47 mm/y. In contrast to previous reports, leaflet orientation and aortic valve dysfunction were not independent predictors of aortic dilation. A family history of aortic valve disease was associated with a significantly increased risk of increasing ascending aortic size., (Copyright © 2015 Elsevier Inc. All rights reserved.)
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- 2015
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371. Careful cardiovascular screening and follow-up of women with Turner syndrome before and during pregnancy is necessary to prevent maternal mortality
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Boissonnas, Celine Chalas, Davy, Celine, Bornes, Marie, Arnaout, Leila, Meune, Christophe, Tsatsatris, Vassilis, Mignon, Alexandre, and Jouannet, Pierre
- Subjects
- *
CARDIOVASCULAR system radiography , *CARDIOVASCULAR disease diagnosis , *TURNER'S syndrome , *REPRODUCTIVE technology , *PREGNANCY , *MATERNAL mortality , *OVUM , *AORTIC valve abnormalities , *MEDICAL centers , *PATIENTS , *TRANSPLANTATION of organs, tissues, etc. , *PREVENTION - Abstract
Objective: To report the fatal outcome of a woman with Turner syndrome (TS) undergoing assisted reproductive technology (ART). Design: Case report. Setting: Reproductive medicine center. Patient(s): A 33-year-old woman with TS. Intervention(s): Screening before oocyte donation and treatment of aortic dissection occurring at term pregnancy. Main Outcome Measure(s): Evaluation of cardiovascular risk. Result(s): After a normal cardiac screening, a woman with TS got pregnant as a result of oocyte donation. At 16 weeks of gestation, a bicuspid aortic valve was detected and associated with moderate aortic root dilation. Aortic dissection was diagnosed at 38 weeks of gestation, which required emergent cesarean delivery and aortic root replacement. Despite surgical treatment, early maternal death was recorded. Conclusion(s): Careful cardiac screening and close follow-up before and during pregnancy are necessary in patients with TS. [Copyright &y& Elsevier]
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- 2009
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372. Turner Syndrome Is an Independent Risk Factor for Aortic Dilation in the Young.
- Subjects
- *
TURNER'S syndrome - Abstract
An abstract of the article "Turner Syndrome Is an Independent Risk Factor for Aortic Dilation in the Young," by Leo Lopez, Steven D. Colan, Roque Ventura, and Michael Silberbach is presented.
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- 2008
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373. 1024-81 Aortic Dilation in Young Males with Isolated Normally Functioning Bicuspid Aortic Valve
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Stefano Nislri, Roldano Scognamiglio, Maria Domenica Sorbo, Marco Marin, and Monica Palisi
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Aortic arch ,Body surface area ,medicine.medical_specialty ,business.industry ,Anatomy ,medicine.disease ,Bicuspid aortic valve ,Aneurysm ,medicine.artery ,Internal medicine ,Ascending aorta ,cardiovascular system ,medicine ,Cardiology ,Cardiology and Cardiovascular Medicine ,business ,Aortic dilation ,Young male ,Congenital disorder - Abstract
Bicuspid aortic valve (BAV) is a common congenital disorder frequently coexisting with abnormalities of the aortic arch including aortic coarctation, dissecting aneurysm and aortic root dilation: it has been suggested that these associations result from a common developmental defect. Moreover, aortic root dimensions are influenced by age and body size; thus, in order to test the hypothesis of a common congenital disorder underlying BAV and aortic dilation, we prospectively studied young males with isolated, normally functioning bicuspid aortic valve during the medical examinations for the military service, in comparison with a age-matched group with no cardiovascular abnormalities. Sixty-three patients (aged 17.8 ± 0.6 year, range 17-19) whose echocardiograms showed a normally functioning BAV were studied at the Military Hospital in Verona by 2D echocardiography with PW, CW and Color Doppler. Seventy subjects, comparable for age (17.82 ± 0.7, range 17-19) and body surface area (1.79 ± 0.09 vs 1.82 ± 0.08, respectively) without clinical and echocardiographic evidence of cardiac abnormalities, were used as control group. Aortic root dimensions were measured by 2D-echocardiography at four levels: 1) anulus, 2) sinuses on Valsalva, 3) supraaortic ridge and 4) proximal ascending aorta. Results (mean ± SD): Level BAV Normals p Anulus 2.36 ± 0.31 2.27 ± 0.29 NS Sinuses of Valsalva 3.16 ± 0.38 2.90 ± 0.32 l0.01 Supraaortic ridge 2.64 ± 0.46 2.50 ± 0.28 0.03 Proximal Ascending Aorta 3.12 ± 0.48 2.71 ± 0.29 l0.01 Conclusion Aortic root dimensions are significantly greater in young men with normally functioning BAV than in control subjects comparable for gender, age and body size. Noticeably, the aortic root dimensions in patients with BAV are still within the range of normality. These data confirm the hypothesis that BAV and aortic dilation are expression of a common developmental defect.
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- 1995
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374. D-transposition of the great arteries: the current era of the arterial switch operation.
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Villafañe J, Lantin-Hermoso MR, Bhatt AB, Tweddell JS, Geva T, Nathan M, Elliott MJ, Vetter VL, Paridon SM, Kochilas L, Jenkins KJ, Beekman RH 3rd, Wernovsky G, and Towbin JA
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- Humans, Transposition of Great Vessels surgery, Cardiac Surgical Procedures methods, Vascular Surgical Procedures methods
- Abstract
This paper aims to update clinicians on "hot topics" in the management of patients with D-loop transposition of the great arteries (D-TGA) in the current surgical era. The arterial switch operation (ASO) has replaced atrial switch procedures for D-TGA, and 90% of patients now reach adulthood. The Adult Congenital and Pediatric Cardiology Council of the American College of Cardiology assembled a team of experts to summarize current knowledge on genetics, pre-natal diagnosis, surgical timing, balloon atrial septostomy, prostaglandin E1 therapy, intraoperative techniques, imaging, coronary obstruction, arrhythmias, sudden death, neoaortic regurgitation and dilation, neurodevelopmental (ND) issues, and lifelong care of D-TGA patients. In simple D-TGA: 1) familial recurrence risk is low; 2) children diagnosed pre-natally have improved cognitive skills compared with those diagnosed post-natally; 3) echocardiography helps to identify risk factors; 4) routine use of BAS and prostaglandin E1 may not be indicated in all cases; 5) early ASO improves outcomes and reduces costs with a low mortality; 6) single or intramural coronary arteries remain risk factors; 7) post-ASO arrhythmias and cardiac dysfunction should raise suspicion of coronary insufficiency; 8) coronary insufficiency and arrhythmias are rare but are associated with sudden death; 9) early- and late-onset ND abnormalities are common; 10) aortic regurgitation and aortic root dilation are well tolerated; and 11) the aging ASO patient may benefit from "exercise-prescription" rather than restriction. Significant strides have been made in understanding risk factors for cardiac, ND, and other important clinical outcomes after ASO., (Copyright © 2014 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)
- Published
- 2014
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375. Cardiovascular and genitourinary anomalies in patients with duplications within the Williams syndrome critical region: phenotypic expansion and review of the literature.
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Zarate YA, Lepard T, Sellars E, Kaylor JA, Alfaro MP, Sailey C, Schaefer GB, and Collins RT 2nd
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- Cardiovascular Abnormalities diagnosis, Child, Preschool, Chromosome Mapping, Comparative Genomic Hybridization, Facies, Female, Humans, In Situ Hybridization, Fluorescence, Male, Urogenital Abnormalities diagnosis, Young Adult, Cardiovascular Abnormalities genetics, Chromosome Duplication, Chromosomes, Human, Pair 7, Phenotype, Urogenital Abnormalities genetics, Williams Syndrome genetics
- Abstract
Williams syndrome results from a microdeletion of approximately 1.5 Mb of chromosome 7q11.23. Several patients have been reported with the reciprocal microduplication in association with a variety of phenotypic features including cognitive impairment and typical facial features, though only a few have had birth defects. We report on three probands with duplications within 7q11.23 of variable sizes; two with cardiovascular involvement including aortic dilation and the other with unilateral renal and gonadal agenesis. We offer a comparison with previously reported cases of duplications of 7q11.23. In light of the present cases, we recommend undertaking echocardiographic and renal ultrasound evaluation of patients with documented 7q11.23 duplications. Further, this cytogenetic abnormality should be part of the differential diagnosis for patients with aortic dilation, as well as those with unilateral renal and gonadal agenesis., (© 2014 Wiley Periodicals, Inc.)
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- 2014
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376. Pregnancy and cardiovascular risk for women with Turner syndrome.
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Bondy C
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- Female, Guidelines as Topic, Humans, Pre-Eclampsia epidemiology, Pregnancy, Risk Factors, Women's Health, Oocyte Donation, Pregnancy Complications, Cardiovascular epidemiology, Turner Syndrome epidemiology
- Abstract
Most women with Turner syndrome (TS) are infertile due to primary ovarian failure. However, approximately 5% experience spontaneous pregnancy, and recently, more women with TS have used assisted reproductive technology with donated oocytes (ART-OD) to become pregnant. The first generation of Turner patients undergoing ART-OD demonstrated a high rate of fatal aortic dissection in late pregnancy or postpartum. More recent observations, particularly from Nordic countries, suggest a lesser risk of dissection, but confirm a high rate of pre-eclampsia in ART-OD pregnancies. This article reviews publications since 2000 concerning maternal outcomes for pregnancies in women with TS to determine if specific risk factors such as type of pregnancy, age, or presence of underlying congenital cardiovascular disease may identify women at special risk.
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- 2014
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377. Cardiac manifestations of Pallister-Killian syndrome.
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Tilton RK, Wilkens A, Krantz ID, and Izumi K
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- Child, Preschool, Chromosomes, Human, Pair 12, Cohort Studies, Echocardiography, Facies, Female, Humans, Infant, Infant, Newborn, Male, Pregnancy, Prenatal Diagnosis, Chromosome Disorders diagnosis, Heart Defects, Congenital diagnosis, Phenotype
- Abstract
Pallister-Killian syndrome (PKS) is a sporadic multisystem genetic diagnosis characterized by facial dysmorphia, variable developmental delay and intellectual impairment, hypotonia, hearing loss, seizures, differences in skin pigmentation, temporal alopecia, diaphragmatic hernia, congenital heart defects, and other systemic abnormalities. Although congenital heart defects have been described in association with PKS, the full spectrum of heart disease is still not entirely known. Here, we describe the pattern of cardiac findings of 81 probands with PKS who have had at least one cardiac evaluation, demonstrating structural heart difference in 37% of our cohort (n = 30). Septal defects such as atrial or ventricular septal defects (n = 12) were the most commonly seen congenital heart differences. Additional findings included the occasional occurrence of bicuspid aortic valve, aortic dilatation, and cardiac hypertrophy/cardiomyopathy. We suggest cardiac evaluation for all individuals with PKS at the time of diagnosis as well as subsequent longitudinal follow-up to monitor for the development of cardiomyopathy and aortic dilatation., (© 2014 Wiley Periodicals, Inc.)
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- 2014
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378. Aortic valve dysfunction and aortic dilation in adults with coarctation of the aorta.
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Clair M, Fernandes SM, Khairy P, Graham DA, Krieger EV, Opotowsky AR, Singh MN, Colan SD, Meijboom EJ, and Landzberg MJ
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- Adolescent, Adult, Age Factors, Aged, Aortic Aneurysm diagnosis, Aortic Aneurysm therapy, Aortic Coarctation diagnosis, Aortic Coarctation therapy, Aortic Valve abnormalities, Aortic Valve Insufficiency diagnosis, Aortic Valve Insufficiency physiopathology, Aortic Valve Insufficiency therapy, Aortic Valve Stenosis diagnosis, Aortic Valve Stenosis physiopathology, Aortic Valve Stenosis therapy, Bicuspid Aortic Valve Disease, Boston epidemiology, Dilatation, Pathologic, Female, Heart Valve Diseases epidemiology, Humans, Male, Middle Aged, Prevalence, Retrospective Studies, Risk Factors, Severity of Illness Index, Time Factors, Young Adult, Aorta pathology, Aortic Aneurysm epidemiology, Aortic Coarctation epidemiology, Aortic Valve physiopathology, Aortic Valve Insufficiency epidemiology, Aortic Valve Stenosis epidemiology
- Abstract
Objectives: To determine the prevalence of aortic valve dysfunction, aortic dilation, and aortic valve and ascending aortic intervention in adults with coarctation of the aorta (CoA)., Background: Aortic valve dysfunction and aortic dilation are rare among children and adolescents with CoA. With longer follow-up, adults may be more likely to have progressive disease., Methods: We retrospectively reviewed all adults with CoA, repaired or unrepaired, seen at our center between 2004 and 2010., Results: Two hundred sixteen adults (56.0% male) with CoA were identified. Median age at last evaluation was 28.3 (range 18.0 to 75.3) years. Bicuspid aortic valve (BAV) was present in 65.7%. At last follow-up, 3.2% had moderate or severe aortic stenosis, and 3.7% had moderate or severe aortic regurgitation. Dilation of the aortic root or ascending aorta was present in 28.0% and 41.6% of patients, respectively. Moderate or severe aortic root or ascending aortic dilation (z-score > 4) was present in 8.2% and 13.7%, respectively. Patients with BAV were more likely to have moderate or severe ascending aortic dilation compared with those without BAV (19.5% vs. 0%; P < 0.001). Age was associated with ascending aortic dilation (P = 0.04). At most recent follow-up, 5.6% had undergone aortic valve intervention, and 3.2% had aortic root or ascending aortic replacement., Conclusion: In adults with CoA, significant aortic valve dysfunction and interventions during early adulthood were uncommon. However, aortic dilation was prevalent, especially of the ascending aorta, in patients with BAV., (© 2013 Wiley Periodicals, Inc.)
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- 2014
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379. Aortic dilation in Turner syndrome
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S. Anne Hendricks, David B. Allen, and Jay M. Levy
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Adult ,Risk ,medicine.medical_specialty ,Adolescent ,MEDLINE ,Turner Syndrome ,Aneurysm ,Text mining ,Internal medicine ,Turner syndrome ,medicine ,Humans ,Child ,Aortic dilation ,Aorta ,business.industry ,medicine.disease ,Aortic Aneurysm ,Aortic Dissection ,Echocardiography ,Karyotyping ,Pediatrics, Perinatology and Child Health ,Cardiology ,Female ,business ,Dilatation, Pathologic - Published
- 1986
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380. The bicuspid aortic valve and its relation to aortic dilation.
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Yuan SM, Jing H, and Lavee J
- Subjects
- Adolescent, Adult, Age Factors, Aged, Aged, 80 and over, Aorta anatomy & histology, Aortic Valve diagnostic imaging, Aortic Valve physiopathology, Dilatation, Pathologic etiology, Female, Humans, Male, Middle Aged, Organ Size, Retrospective Studies, Severity of Illness Index, Sex Factors, Ultrasonography, Young Adult, Aortic Diseases etiology, Aortic Valve abnormalities, Heart Valve Diseases physiopathology
- Abstract
Background: A bicuspid aortic valve (BAV) is a common congenital heart disease, which affects 1-2% of the population. However, the relationship between BAVs and aortic dilation has not been sufficiently elucidated., Methods: A total of 241 BAV patients who were referred to this hospital for cardiac surgery over a 4.75-year period were included in this study. In addition to the clinical characteristics of the included patients, the morphological features of the aortic valve and aorta, the length of the left main coronary artery, and the laboratory findings (the coagulation and hematological parameters as well as the total cholesterol concentration) were determined and compared with those of the tricuspid aortic valve (TAV) patients., Results: The BAV patients were younger than the TAV patients for a valve surgery in the last 3 months of the study period. The BAV patients were predominantly male. Most of the BAVs that were surgically treated were stenotic, regurgitant, or combined, and only 19 (7.88%) were normally functioning valves. According to echocardiography or operative records, 148 (78.31%) were type A, 31 (16.40%) were type B, and 10 (5.29%) were type C. The left main coronary artery was much shorter in the BAV patients than it was in the TAV patients. There was no significant difference between BAV and TAV patients in the total cholesterol concentrations; whereas differences were noted between patients receiving lipid-lowering therapy and those not receiving lipid-lowering therapy. The dimensions of the aortic root, sinotubular junction, and ascending aorta were beyond normal limits, while they were significantly smaller in the BAV patients than in the TAV patients. They were also much smaller in patients receiving statin therapy than those not receiving statin therapy in both groups. Moreover, the aortic dilation in the BAV group was found to be significantly associated with patient age., Conclusions: The BAV patients developed aortic wall and aortic valve disorders at a younger age than the TAV patients and were predominantly male. Aortic dilation was observed in the aortic root, sinotubular junction, and ascending aortic segments in both the BAV and TAV patients undergoing surgical aortic valve replacement, although the BAV patients had a smaller degree of dilation than the TAV patients, and dilation was also significantly age-related in this group. The shorter left main coronary artery that the BAV patients possess may contribute to the progressive course of aortic dilation that these patients experience. Statin therapy did not affect the aortic annulus in either group, but did decrease the dimensions of the aortic root, sinotubular junction and ascending aorta. In general, statin therapy had a better effect on the aortas of the TAV patients than it did on those of the BAV patients.
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- 2010
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381. Effect of statins on aortic root growth rate in patients with bicuspid aortic valve anatomy
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Vasileios Kamperidis, Madelien V. Regeer, Nina Ajmone Marsan, Victoria Delgado, Jeroen J. Bax, Philippe J. van Rosendael, and Martin J. Schalij
- Subjects
Aortic valve ,Adult ,Male ,medicine.medical_specialty ,Statin ,Time Factors ,Bicuspid aortic valve ,medicine.drug_class ,Heart Valve Diseases ,3-Hydroxy-3-methylglutaryl coenzyme A reductase inhibitors ,Coronary artery disease ,Aortic aneurysm ,Bicuspid Aortic Valve Disease ,medicine.artery ,Internal medicine ,Ascending aorta ,medicine ,Humans ,Radiology, Nuclear Medicine and imaging ,cardiovascular diseases ,Aorta ,Aged ,Retrospective Studies ,Echocardiography, Doppler, Pulsed ,Original Paper ,business.industry ,Sinotubular Junction ,Anatomy ,Middle Aged ,medicine.disease ,Aortic Aneurysm ,Echocardiography, Doppler, Color ,medicine.anatomical_structure ,Treatment Outcome ,Echocardiography ,Radiology Nuclear Medicine and imaging ,Aortic Valve ,Cardiology ,cardiovascular system ,Female ,Hydroxymethylglutaryl-CoA Reductase Inhibitors ,business ,Cardiology and Cardiovascular Medicine ,Aortic dilation ,Dilatation, Pathologic - Abstract
Bicuspid aortic valve (BAV) anatomy is associated with increased growth rate of the aortic root compared to tricuspid aortic valves. Statins decrease the growth rate of abdominal aneurysms; however their effect on the aortic root growth rate has not been elucidated. The present study evaluated the association between use of statins and aortic root growth in patients with BAV. A total of 199 patients (43 ± 15 years, 69 % male) with BAV who underwent ≥2 echocardiographic measurements of the aortic root ≥1 year apart were included in this retrospective observational study. Median follow-up duration was 4.7 years (interquartile range 2.7–8.3 years). Growth rate (mm/year) of the aortic root was compared between statin users (n = 41) and non-users (n = 158). Statin users were significantly older and had more cardiovascular risk factors than their counterparts. Ascending aorta diameter was significantly smaller at baseline and at follow-up in statin users compared with non-users when adjusted for coronary artery disease, age and medication. The average annual growth rate was 0.08 mm/year (95 % confidence interval 0.03–0.13) for the aortoventricular junction, 0.16 mm/year (0.11–0.21) for the sinus of Valsalva, 0.12 mm/year (0.07–0.17) for the sinotubular junction and 0.45 mm/year (0.37–0.53) for the ascending aorta. The dilation rate of the aortic segments was not different between statin users and non-users. In conclusion, in patients with BAV, although the use of statins was associated with smaller ascending aorta, the annual dilation rate of the aortic root was not influenced by the use of statins.
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382. D-Transposition of the Great Arteries The Current Era of the Arterial Switch Operation
- Author
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Villafañe, Juan, Lantin-Hermoso, M. Regina, Bhatt, Ami B., Tweddell, James S., Geva, Tal, Nathan, Meena, Elliott, Martin J., Vetter, Victoria L., Paridon, Stephen M., Kochilas, Lazaros, Jenkins, Kathy J., Beekman, Robert H., Wernovsky, Gil, and Towbin, Jeffrey A.
- Subjects
congenital heart defect ,prostaglandin E ,coronary insufficiency ,adult congenital heart disease ,congenital heart surgery and sequelae ,aortic dilation - Abstract
This paper aims to update clinicians on “hot topics” in the management of patients with D-loop transposition of the great arteries (D-TGA) in the current surgical era. The arterial switch operation (ASO) has replaced atrial switch procedures for D-TGA, and 90% of patients now reach adulthood. The Adult Congenital and Pediatric Cardiology Council of the American College of Cardiology assembled a team of experts to summarize current knowledge on genetics, pre-natal diagnosis, surgical timing, balloon atrial septostomy, prostaglandin E1 therapy, intraoperative techniques, imaging, coronary obstruction, arrhythmias, sudden death, neoaortic regurgitation and dilation, neurodevelopmental (ND) issues, and lifelong care of D-TGA patients. In simple D-TGA: 1) familial recurrence risk is low; 2) children diagnosed pre-natally have improved cognitive skills compared with those diagnosed post-natally; 3) echocardiography helps to identify risk factors; 4) routine use of BAS and prostaglandin E1 may not be indicated in all cases; 5) early ASO improves outcomes and reduces costs with a low mortality; 6) single or intramural coronary arteries remain risk factors; 7) post-ASO arrhythmias and cardiac dysfunction should raise suspicion of coronary insufficiency; 8) coronary insufficiency and arrhythmias are rare but are associated with sudden death; 9) early- and late-onset ND abnormalities are common; 10) aortic regurgitation and aortic root dilation are well tolerated; and 11) the aging ASO patient may benefit from “exercise-prescription” rather than restriction. Significant strides have been made in understanding risk factors for cardiac, ND, and other important clinical outcomes after ASO.
- Full Text
- View/download PDF
383. Extra-anatomic bypass with open-plugging stent graft for extensive dissected aortic aneurysm
- Author
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Yoshiaki Saito, Kazuyuki Daitoku, Yasuyuki Suzuki, Ryosuke Kowatari, Masahito Minakawa, and Ikuo Fukuda
- Subjects
medicine.medical_specialty ,Extra anatomic bypass ,business.industry ,medicine.medical_treatment ,Lumen (anatomy) ,Stent ,medicine.disease ,Article ,Surgery ,Aortic aneurysm ,Aneurysm ,Descending aorta ,medicine.artery ,medicine ,cardiovascular system ,Thoracotomy ,cardiovascular diseases ,Cardiology and Cardiovascular Medicine ,Aortic dilation ,business - Abstract
Treatment for an extensively dissected aortic aneurysm is a surgical challenge. Open surgery using a left thoracotomy is promising but can be dangerous in patients with pulmonary comorbidity. We treated a 63-year-old man with chronic type B aortic dissection with aneurysmal change and ascending aortic dilation. The thoracoabdominal aorta was also dissected, dilated, and tapered; thus, a simple hybrid strategy was not possible, even with open fenestration. We performed ascending aortic replacement with reconstruction of the cervical vessels and extra-anatomic bypass from the ascending to descending aorta, with aneurysmal isolation. A stent graft was inserted at the true lumen of the residual aneurysm to reduce endopressure. Total thrombosis and reduction in size of the aneurysm was achieved, and the patient recovered well, without complications.
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384. Descending aortic replacement after Nuss for pectus excavatum in a Marfan patient—Case report
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Michael B. Gotway, D. Craig Miller, Patrick A. DeValeria, MennatAllah M. Ewais, and Dawn E. Jaroszewski
- Subjects
Marfan syndrome ,musculoskeletal diseases ,congenital, hereditary, and neonatal diseases and abnormalities ,medicine.medical_specialty ,Future risk ,Case Report ,Nuss procedure ,03 medical and health sciences ,0302 clinical medicine ,Pectus excavatum ,Internal medicine ,medicine.artery ,Medicine ,cardiovascular diseases ,skin and connective tissue diseases ,Aortic dilation ,business.industry ,Descending aorta ,medicine.disease ,Surgery ,030220 oncology & carcinogenesis ,cardiovascular system ,Cardiology ,business ,030217 neurology & neurosurgery - Abstract
IntroductionThe Nuss procedure for pectus excavatum (PE) repair has been successfully performed in Marfan syndrome (MFS) patients however there is concern for future risk of aortic dilation/rupture and need for emergent access with support bars in place.Case presentationWe present a 45 year-old male with MFS that required descending aortic replacement shortly after modified Nuss repair.DiscussionThe majority of MFS patients have severe PE and repair with the Nuss procedure is not uncommon. The risk for life threatening aortic dilation, dissection, or rupture in such patients is a concern when utilizing this technique. Our work has been reported in line with the CARE criteria.ConclusionNuss repair should be considered in MFS patients with technique modifications and careful consideration of future risk of aortic dilation and rupture.
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385. [Untitled]
- Subjects
0301 basic medicine ,Aortic valve ,medicine.medical_specialty ,Telomerase ,Calcified aortic valve ,business.industry ,Cell Biology ,030204 cardiovascular system & hematology ,medicine.disease ,Telomere ,03 medical and health sciences ,Stenosis ,030104 developmental biology ,0302 clinical medicine ,medicine.anatomical_structure ,Internal medicine ,medicine ,Cardiology ,business ,Aortic dilation ,Aortic valve regurgitation ,Developmental Biology ,Calcification - Abstract
BackgroundShort telomere length (TL) is associated with age-related diseases, in particular cardiovascular diseases. However, whether the onset and course of aortic stenosis (AS) is linked to TL in aortic valves remains unknown.ObjectivesTo assess telomere dynamics (TL and telomerase activity) in aortic valves and the possible implication of TL in onset and course of AS.MethodsDNA was extracted from aortic valves obtained from 55 patients (78.2% men; age, 37–79 years), who had undergone replacement surgery due to AS (AS group, n = 32), aortic valve regurgitation and aortic dilation (Non-AS group, n = 23). TL was measured by telomere restriction fragment analysis (TRF) in calcified and non-calcified aortic valve areas. Telomerase activity was evaluated using telomerase repeat amplification protocol (TRAP) in protein extracts from non-calcified and calcified areas of valves obtained from 4 additional patients (50% men; age, 27–70 years).ResultsTL was shorter in calcified aortic valve areas in comparison to non-calcified areas (n = 31, 8.58 ± 0.73 kb vs. 8.12 ± 0.75 kb, p < 0.0001), whereas telomerase activity was not detected in any of those areas. Moreover, patients from AS group displayed shorter telomeres in non-calcified areas than those from the Non-AS group (8.40 ± 0.64 kb vs. 8.85 ± 0.65, p = 0.01).ConclusionsShort telomeres in aortic valves may participate in the development of AS, while concurrently the calcification process seems to promote further local decrease of TL in calcified areas of valves.
386. Bicuspid Aortic Valve Disease Beyond the Aortic Root Potential Prognostic Implications for Ascending Aortic Dilation**Editorials published in the Journal of the American College of Cardiologyreflect the views of the authors and do not necessarily represent the views of JACCor the American College of Cardiology
- Author
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Farasat, S. Morteza
- Subjects
flow-mediated vasodilation ,MMP-2 ,cardiovascular system ,bicuspid valve ,aortic dilation - Full Text
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387. Aortic Rupture Into the Esophagus During Angiography
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H. Thompson Dale, Kenneth A. Popio, James A. DeWeese, and Kenneth Thomson
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Aortic dissection ,medicine.medical_specialty ,medicine.diagnostic_test ,business.industry ,General Medicine ,medicine.disease ,Surgery ,medicine.anatomical_structure ,Aneurysm ,Angiography ,cardiovascular system ,medicine ,Upper gastrointestinal bleeding ,Radiology ,Esophagus ,Aortic dilation ,business ,Aortic rupture ,Cystic medial necrosis - Abstract
A 67-year-old woman was examined and found to have symptoms and chest roentgenogram suggesting acute aortic dissection. During emergency angiography, massive upper gastrointestinal bleeding developed, and the woman died. The angiogram showed a raised intimal flap of acute dissecting aneurysm. Postmortem examination showed extensive cystic medial necrosis, aortic dilation, and an unusual combination of anatomic abnormalities, which explained the previously unreported false-positive intimal flap. The site of aortic rupture was not localized, but a large tear was found in the esophagus. ( JAMA 239:1880-1881, 1978)
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- 1978
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388. Baseline 2D PC-MRI hemodynamic markers correlate to aorta growth in serially monitored bicuspid aortic valve patients
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James C. Carr, Nicholas Naro, Michael Markl, Alexander P. Taylor, Jeremy D. Collins, Patrick M. McCarthy, Jyothy Puthumana, and Alex J. Barker
- Subjects
Medicine(all) ,medicine.medical_specialty ,Pathology ,Aorta ,Radiological and Ultrasound Technology ,Aortic hemodynamics ,business.industry ,Hemodynamics ,Heart defect ,Dissection (medical) ,medicine.disease ,Bicuspid aortic valve ,medicine.artery ,Internal medicine ,Poster Presentation ,cardiovascular system ,Cardiology ,Medicine ,Radiology, Nuclear Medicine and imaging ,Cardiology and Cardiovascular Medicine ,business ,Aortic dilation ,Angiology - Abstract
Background Bicuspid aortic valve (BAV) is the most common congenital heart defect whereby a significant percentage of patients experience dissection or progressive aortic dilation. There is increasing evidence that aortic hemodynamics such as wall shear stress (WSS) and flow jet eccentricity may exacerbate the development of aortopathy; however, these relationships have been investigated using experimental cardiac MRI protocols. We hypothesize that standard 2D through-plane encoded PC-MRI can aid in identifying BAV patients most at-risk for progressive aortic dilation.
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389. VENTRICULAR DYSFUNCTION AND AORTIC DILATION IN PATIENTS WITH RECESSIVE DYSTROPHIC EPIDERMOLYSIS BULLOSA
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John L. Jefferies, Jeffrey A. Towbin, Thomas J. Ryan, and Anne W. Lucky
- Subjects
Pathology ,medicine.medical_specialty ,genetic structures ,integumentary system ,business.industry ,medicine.disease ,Skin fragility ,Heart failure ,Recessive dystrophic epidermolysis bullosa ,medicine ,In patient ,Epidermolysis bullosa ,skin and connective tissue diseases ,Cardiology and Cardiovascular Medicine ,business ,Aortic dilation - Abstract
Epidermolysis bullosa (EB) is a group of diseases characterized by skin fragility and blistering with minimal stimulus and occurring in 8:1,000,000 children. Previously, recessive dystrophic EB (RDEB) has been associated with patient reported or clinically diagnosed heart failure. The current
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390. COARCTATION MAY BE ASSOCIATED WITH MORE RAPID AORTIC DILATION IN INDIVIDUALS WITH BICUSPID AORTIC VALVE: INSIGHTS FROM THE GENTAC REGISTRY
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Maria Yates, Claudia Pedroza, Weilu Han, Federico Asch, GenTAC Consortium, and Siddharth Prakash
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medicine.medical_specialty ,business.industry ,medicine.disease ,Bicuspid aortic valve ,Increased risk ,Internal medicine ,Cardiology ,medicine ,cardiovascular system ,In patient ,cardiovascular diseases ,Cardiology and Cardiovascular Medicine ,Aortic dilation ,business - Abstract
Bicuspid aortic valve (BAV) is associated with thoracic aortic aneurysms and dissections. The prevalence of aortic coarctation is increased in patients with BAV. We hypothesize that patients with coarctation are at increased risk for aortic complications compared to those with isolated BAV and may
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