Incidence, morphology, and progression of bicuspid aortic valve in pediatric and young adult subjects with coexisting congenital heart defects

Autor: Jared Palfreeman, Jonathan N. Johnson, Thomas Nienaber, Cecilia Craviari, Donald J. Hagler, Talha Niaz, Frank Cetta, Joseph T. Poterucha
Rok vydání: 2016
Předmět:
Heart Defects
Congenital
Male
medicine.medical_specialty
Adolescent
Heart Ventricles
Coarctation of the aorta
Heart Valve Diseases
Aorta
Thoracic
030204 cardiovascular system & hematology
Severity of Illness Index
03 medical and health sciences
Young Adult
0302 clinical medicine
Bicuspid aortic valve
Bicuspid Aortic Valve Disease
Internal medicine
medicine.artery
Ascending aorta
medicine
Humans
Radiology
Nuclear Medicine and imaging
cardiovascular diseases
030212 general & internal medicine
Young adult
Child
Retrospective Studies
business.industry
Incidence (epidemiology)
Escherichia coli Proteins
Interrupted aortic arch
Infant
Newborn
Infant
General Medicine
medicine.disease
United States
Stenosis
Tetrahydrofolate Dehydrogenase
Echocardiography
Aortic valve stenosis
Aortic Valve
Child
Preschool
Pediatrics
Perinatology and Child Health
cardiovascular system
Cardiology
Disease Progression
Surgery
Female
Cardiology and Cardiovascular Medicine
business
Zdroj: Congenital heart disease. 12(3)
ISSN: 1747-0803
Popis: Background Bicuspid aortic valve (BAV) occurs both as an isolated cardiac lesion and in association with congenital heart defects (CHD). Their aim was to identify the incidence and morphology of BAV in patients with coexisting CHD and compare their disease progression to patients with isolated BAV. Methods The Mayo Clinic echocardiography database was retrospectively analyzed to identify pediatric and young adult patients (≤22 years) who were diagnosed with BAV from 1990 to 2015. The morphology of BAV was determined from the echocardiographic studies before any intervention. Results Overall, 1010 patients with BAV were identified, 619 (61%) with isolated BAV and 391 (39%) with BAV and coexisting CHD. The incidence of BAV was highest in patients with coarctation of the aorta (36%) and interrupted aortic arch (36%). In comparison to patients with isolated BAV, patients with BAV and left-sided obstructive lesions more frequently had right-left cusp fusion (P = .0001). BAV in patients with right-sided obstructive lesions was rare, but they more frequently had right-noncoronary or left-noncoronary cusp fusion (P = .01). No significant progression of aortic stenosis or regurgitation was observed in patients with BAV and coexisting CHD; however in patients with isolated BAV the severity of aortic regurgitation increased with age. In patients with isolated BAV, the ascending aorta diameter (z-score) increased with age, peaked around 8–9 years of age, and was larger in comparison to patients with BAV and coexisting CHD. The sinus of Valsalva diameter (z-score) in patients with BAV and ventricular septal defect was larger than isolated BAV patients after 18 years (P
Databáze: OpenAIRE
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