Frequency of Development of Aortic Valve Disease in Unrepaired Perimembranous Ventricular Septal Defects.
We sought to determine the natural history of aortic valve disease in patients with unrepaired perimembranous ventricular septal defects (pVSDs) and to identify echocardiographic parameters predictive of increased risk of surgical repair of pVSD because of aortic valve disease. We retrospectively analyzed all echocardiograms of patients with a diagnosis of pVSD at our institution from January 1999 to January 2015. All available echocardiographic data were collected. Patients were excluded if there was another structural cardiac anomaly other than bicuspid aortic valve, small patent foramen ovale, or ductus arteriosus. The prevalences of aortic valve prolapse and regurgitation, as well as aortic valve disease progression, were determined. A total of 2,114 echocardiograms from 657 patients with unrepaired pVSD were reviewed. Median age at the time of echocardiogram was 1.9 years (interquartile range [IQR] 0.2 to 5.4). Median duration of follow-up was 1.7 years (IQR 0.2 to 7.4). pVSD-associated aortic valve disease prompted surgical intervention in 1.5% (10 of 657) of patients. Median age at the time of surgery was 4.8 years (IQR 1.7 to 8.4). A pVSD-to-aortic annulus diameter ratio of 0.66 ± 0.05 was present in 90% (9 of 10) of patients who underwent surgical closure because of pVSD-associated aortic valve disease. In conclusion, pVSD-associated aortic valve disease is uncommon, and progression of aortic regurgitation is rare. These data suggest that the majority of patients with pVSD do not require frequent follow-up and that frequent follow-up can be saved for a subset with echocardiographic markers placing them at higher risk of aortic valve diseases.
Division of Pediatric Cardiology, University of Arkansas for Medical Sciences, Little Rock, Arkansas. Electronic address: apadiyath@uams.edu.
Asif
A
Padiyath
Division of Pediatric Cardiology, University of Arkansas for Medical Sciences, Little Rock, Arkansas.
Elizabeth S
ES
Makil
Division of Pediatric Cardiology, University of Arkansas for Medical Sciences, Little Rock, Arkansas.
Katherine T
KT
Braley
Division of Pediatric Cardiology, University of Arkansas for Medical Sciences, Little Rock, Arkansas.
Elijah H
EH
Bolin
Biostatistics Program, Department of Pediatrics, University of Arkansas for Medical Sciences, Little Rock, Arkansas.
Xinyu
X
Tang
Biostatistics Program, Department of Pediatrics, University of Arkansas for Medical Sciences, Little Rock, Arkansas.
Jeffery M
JM
Gossett
Division of Pediatric Cardiology, University of Arkansas for Medical Sciences, Little Rock, Arkansas.
R Thomas
RT
Collins
D000293:Adolescent; D001021:Aortic Valve / Q000000981:diagnostic imaging*; D001131:Arkansas / Q000453:epidemiology; D000082882:Bicuspid Aortic Valve Disease; D002648:Child; D002675:Child, Preschool; D018450:Disease Progression; D018618:Echocardiography, Doppler, Color; D005260:Female; D005500:Follow-Up Studies; D006330:Heart Defects, Congenital / Q000175:diagnosis / Q000453:epidemiology* / Q000209:etiology; D006345:Heart Septal Defects, Ventricular / Q000150:complications* / Q000175:diagnosis; D006349:Heart Valve Diseases / Q000175:diagnosis / Q000453:epidemiology* / Q000209:etiology; D006801:Humans; D015994:Incidence; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D011379:Prognosis; D012189:Retrospective Studies; D012307:Risk Factors; D013997:Time Factors