title: Congenital valvular aortic stenosis in young adults: predictors for rate of progression of stenosis and aortic dilatation.
abstract: BACKGROUND
  Congenital aortic stenosis (AS) is the most common obstructive left-sided cardiac lesion in young adults, however little is known about the progression in adults. Therefore, we aimed to evaluate the progression rate of AS and aortic dilatation in a large multicenter retrospective cohort of asymptomatic young adults with congenital valvular AS.
  
  
  METHODS
  Data were obtained from chart abstraction. Linear mixed-effects models were used to evaluate the progression of AS and aortic dilatation over time. A joint model combining longitudinal echocardiographic and survival data was used for survival analysis.
  
  
  RESULTS
  A total of 414 patients (age 29 ± 10 years, 68% male) were included. Median follow-up duration was 4.1 (2.5-5.1) years (1587 patient-years). Peak aortic velocity was 3.4 ± 0.7 m/s at baseline and did not change over time in the total patient population (-0.01 ± 0.03 m/s/year). Increased left ventricular mass was significantly associated with faster AS progression (p<0.001). Aortic dilatation was present in 34% at baseline and 48% at follow-up (p<0.001). The aortic diameter linearly increased over time with a rate of 0.7 ± 0.2mm/year. Rate of aortic dissection was 0.06% per patient-year. Seventy patients required an aortic valve intervention (4.4% per patient-year), with AS progression rate as most powerful predictor (HR 5.11 (95% CI 3.47-7.53)).
  
  
  CONCLUSIONS
  In the majority of patients with mild-to-moderate congenital AS, AS severity does not progress over time. However patients with left ventricular hypertrophy are at risk for faster progression and should be monitored carefully. Although aortic dissections rarely occur, aortic dilatation is common and steadily progresses over time, warranting serial aortic imaging.
authors.affiliation: Department of Cardiology, Thoraxcentre, Erasmus University Medical Centre, Rotterdam, The Netherlands.
authors.forename: Denise
authors.initials: D
authors.lastname: van der Linde
authors.forename: Elini-Rosalina
authors.initials: ER
authors.lastname: Andrinopoulou
authors.forename: Erwin N
authors.initials: EN
authors.lastname: Oechslin
authors.forename: Werner
authors.initials: W
authors.lastname: Budts
authors.forename: Arie P J
authors.initials: AP
authors.lastname: van Dijk
authors.forename: Petronella G
authors.initials: PG
authors.lastname: Pieper
authors.forename: Elly M C J
authors.initials: EM
authors.lastname: Wajon
authors.forename: Marco C
authors.initials: MC
authors.lastname: Post
authors.forename: Maarten
authors.initials: M
authors.lastname: Witsenburg
authors.forename: Candice K
authors.initials: CK
authors.lastname: Silversides
authors.forename: Angela
authors.initials: A
authors.lastname: Oxenius
authors.forename: Ad J J C
authors.initials: AJ
authors.lastname: Bogers
authors.forename: Johanna J M
authors.initials: JJ
authors.lastname: Takkenberg
authors.forename: Jolien W
authors.initials: JW
authors.lastname: Roos-Hesselink
mesh_terms: D000328:Adult; D001021:Aortic Valve / Q000002:abnormalities* / Q000473:pathology*; D001024:Aortic Valve Stenosis / Q000175:diagnosis* / Q000401:mortality; D016208:Databases, Factual / Q000639:trends; D018450:Disease Progression; D005260:Female; D005500:Follow-Up Studies; D006330:Heart Defects, Congenital / Q000175:diagnosis / Q000401:mortality; D006801:Humans; D008297:Male; D008875:Middle Aged; D011237:Predictive Value of Tests; D011446:Prospective Studies; D012189:Retrospective Studies; D015996:Survival Rate / Q000639:trends; D055815:Young Adult