Disease progression and variation in clinical practice for isolated bicuspid aortic valve in children.

Published on Feb 21, 2018in Congenital Heart Disease1.663
· DOI :10.1111/CHD.12591
Melissa S.W. Yamauchi1
Estimated H-index: 1
(Primary Children's Hospital),
Michael D. Puchalski21
Estimated H-index: 21
(Primary Children's Hospital)
+ 6 AuthorsRichard V. Williams39
Estimated H-index: 39
(Primary Children's Hospital)
Sources
Abstract
Background Disease progression of an isolated bicuspid aortic valve (BAV) in children is poorly understood and adult management guidelines may not be applicable. Thus, we sought to evaluate disease progression of pediatric isolated BAV and its relationship to current management practices. Methods Children with a BAV and ≤mild aortic stenosis (AS) and/or aortic regurgitation (AR) at the time of initial evaluation were included in this retrospective cohort study (1/2005-12/2014). Outcomes included change in z-scores for aortic root and ascending aorta diameters, cardiac interventions, adverse outcomes, recommended follow-up interval, and frequency of cardiac imaging studies at each follow up evaluation, as well as AS/AR severity at final evaluation. Outcomes were analyzed using generalized mixed-effect models with subject and provider clustering. Results BAV disease progression was evaluated in 294 subjects over 4.1 ± 2.4 (range 0.2-9.5) years. Ascending aorta z-scores increased by 0.1/year (P  mild in 9 (3%), 1 subject underwent cardiac intervention, and none had a major complication. Management was evaluated in 454 subjects (1343 encounters) with 27 different cardiologists. The average recommended follow-up interval was 1.5 ± 0.9 years. Younger age at diagnosis, greater aortic root or ascending aorta z-score at diagnosis, ≥mild AS/AR at follow-up, and earlier diagnosis era were associated with shorter recommended follow-up interval (P < .001 for all). Imaging was obtained at 87% of follow-up encounters and was associated with age at encounter with children ≥12 years most frequently imaged (P < .001). Provider accounted for 14% of variability in recommended follow-up interval and 24% of imaging variability (P < .001 for both). Conclusions We found little to no evidence of disease progression in children with an isolated BAV. Given the low risk, close follow-up and frequent cardiac imaging for BAV surveillance may not be warranted for children.
References25
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Last. Karl F. Welke (UIUC: University of Illinois at Urbana–Champaign)H-Index: 1
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Background Dilation of the aorta is a frequent complication in patients with bicuspid aortic valves. The aim of this study was to determine the relationship between the subtype of leaflet fusion, right and noncoronary leaflet (R/N) fusion versus right and left leaflet (R/L) fusion, and the patterns of aortic dilation and valve dysfunction in young patients with bicuspid aortic valves. Methods We performed a retrospective review of 642 patients who presented with bicuspid aortic valves between 19...
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#1Rick A. Nishimura (Mayo Clinic)H-Index: 128
#2Catherine M Otto (UW: University of Washington)H-Index: 85
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The medical profession should play a central role in evaluating evidence related to drugs, devices, and procedures for detection, management, and prevention of disease. When properly applied, expert analysis of available data on the benefits and risks of these therapies and procedures can improve
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The aim of this study was to explore the medium-term clinical outcome and the risk of progression of aortic valve disease and aortic dilation in pediatric patients with isolated bicuspid aortic valve (BAV). 179 pediatric patients with isolated BAV were prospectively followed from January 1995 to December 2010. Patients with severe valve dysfunction at baseline were excluded. Clinical outcome included cardiac death, infective endocarditis, aortic complications, cardiac surgery and percutaneous va...
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The risk of acute aortic events in patients with bicuspid aortic valve (BAV) disease is a controversial issue. The real risk of aortic dissection in patients with BAV disease is unknown. An indirect assessment of this risk, however, could be gained with a more detailed understanding of the pathogenesis of BAV aortopathy. There are two major issues that should be clarified before one addresses the question of aortic dissection risk in BAV patients. The first issue, when analysing the data from pr...
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#1Alessandro Della Corte (Seconda Università degli Studi di Napoli)H-Index: 34
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Objectives This study sought to identify risk factors for rapid growth of the ascending aorta in patients with bicuspid aortic valve (BAV) disease, taking into account its phenotypic variability. Background Phenotypic heterogeneity of BAV-related aortopathy has recently been widely recognized. However, few studies have addressed the determinants of aortic growth so far, not distinguishing among morphological phenotypes. Methods Serial retrospective data on 133 adult outpatients with BAV undergoi...
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#1Christiane Pees (Boston Children's Hospital)H-Index: 7
#2Ina Michel-Behnke (Boston Children's Hospital)H-Index: 6
Bicuspid aortic valve (BAV) is a common congenital malformation with the known sequela of ascending aortic dilation. The morphology of the BAV and the elasticity of the adjacent ascending aorta appear to influence the outcome. We prospectively examined 48 pediatric patients with an isolated, native BAV for the morphology and size of the aortic valve, aortic root, sinotubular junction, and ascending aorta and their elasticity indexes. A cohort of 48 matching subjects with tricuspid aortic valves ...
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#1Susan M. Fernandes (Boston Children's Hospital)H-Index: 33
#2Paul KhairyH-Index: 70
Last. Ronald V. LacroH-Index: 32
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Background The aorta in patients with bicuspid aortic valve (BAV) is larger and grows more rapidly than in patients with tricommissural aortic valve. Young patients with BAV can have significant aortic dilation that places them at risk for morbidity and mortality. Objective The aims of this study were to determine the rate of growth of the aorta in young patients with BAV and to identify predictors of significant dilation and rapid aortic growth. Methods 333 patients were randomly selected from ...
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#1Hector I. Michelena (Mayo Clinic)H-Index: 47
#2Amber Khanna (Mayo Clinic)H-Index: 8
Last. Maurice Enriquez-SaranoH-Index: 106
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Design, Setting, and Participants In this retrospective cohort study, we conducted comprehensive assessment of aortic complications of patients with BAV living in a population-based setting in Olmsted County, Minnesota. We analyzed long-term follow-up of a cohort of all Olmsted County residents diagnosed with definite BAV by echocardiography from 1980 to 1999 and searched for aortic complications of patients whose bicuspid valves had gone undiagnosed. The last year of follow-up was 2008-2009. Ma...
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Objective To evaluate the outcomes associated with isolated bicuspid aortic valve (BAV) during childhood and adolescence. Study design Analysis of a large single institutional cohort of children (n = 981) with isolated BAV was undertaken to determine the prevalence of significant ascending aortic dilation and risk of cardiac events. Subjects with known genetic disorders, critical aortic stenosis (intervention required in infancy), or additional lesions such as coarctation of the aorta were exclu...
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#1Leo Lopez (Boston Children's Hospital)H-Index: 19
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n Society of Echocardiography designates this educational activity for of 1.5 AMA PRA Category 1 Credits . Physicians should only claim credit te with the extent of their participation in the activity. CCI recognize the ASE’s certificates and have agreed to honor the credit their registry requirements for sonographers. n Society of Echocardiography is committed to ensuring that its educan and all sponsored educational programs are not influenced by the special y corporation or individual, and it...
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#1Jesse Boyett Anderson (UW: University of Wisconsin-Madison)
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BACKGROUND Prior to the recent release of appropriate use criteria for imaging valvulopathies in children, follow-up of valvular lesions, including isolated bicuspid aortic valve, was not standardised. We describe current follow up, treatment, and intervention strategies for isolated bicuspid aortic valve with varying degrees of stenosis, regurgitation, and dilation in children up to 18 years old and compare them with newly released appropriate use criteria. METHODS Online survey was sent to mem...
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#1Talha NiazH-Index: 8
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1. Talha Niaz, MBBS* 2. Jonathan N. Johnson, MD*,† 3. Frank Cetta, MD*,† 4. Timothy M. Olson, MD*,† 5. Donald J. Hagler, MD*,† 1. *Division of Pediatric Cardiology, Department of Pediatric and Adolescent Medicine, and 2. †Department of Cardiovascular Medicine, Mayo Clinic, Rochester, MN * Abbreviations: AHA : : American Heart Association BAV : : bicuspid aortic valve TTE : : transthoracic echocardiography Bicuspid aortic valve is the most common congenital heart defect in children, adolescents, ...
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Bicuspid aortic valve (BAV) is a common congenital heart diagnosis and is associated with aortopathy. Current guidelines for aortic resection have been validated but are based on aortic diameter, which is insufficient to predict acute aortic events. Clinical and translational collaboration is necessary to identify biomarkers that can individualize the timing of prophylactic surgery for BAV aortopathy. We describe our multidisciplinary BAV program, including research protocols aimed at biomarker ...
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Bicuspid aortic valve (BAV) disease demonstrates a range of clinical presentations and complications. We aim to use cardiac MRI (CMR) to evaluate left ventricular (LV) parameters, myocardial strain and aortic hemodynamics in pediatric BAV patients with and without aortic stenosis (AS) or regurgitation (AR) compared to tricuspid aortic valve (TAV) controls. We identified 58 pediatric BAV patients without additional cardiovascular pathology and 25 healthy TAV controls (15.3 ± 2.2 years) who underw...
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