The Association Between Aortic Valve Leaflet Morphology and Patterns of Aortic Dilation in Patients With Bicuspid Aortic Valves.
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 1994 and 2014. Of these patients, 210 (33%) had aortic dilation (z score >3) by echocardiogram. For each patient, the most recent study or the last study before intervention was reviewed.


RESULTS
Median patient age was 15 years (range, 0 to 40 years) with patients with R/N fusion being younger. The most prevalent subtype was R/N fusion (R/N, n = 114, 54% versus R/L, n = 96, 46%). Dilation of the ascending aorta was seen more often in patients with R/N fusion (R/N, 88% versus R/L, 68%; p = 0.004), whereas the prevalence of dilation of the sinuses of Valsalva was significantly higher in patients with R/L fusion (R/L, 46% versus R/N, 20%; p = 0.01). The magnitude of dilation differed as well. The z value of the sinuses of Valsalva was significantly higher in patients with R/L fusion (R/L, 2.03 versus R/N, 1.2; p = 0.003), whereas the z values of the ascending aorta and sinotubular junction were similar between the groups. Patients with R/N fusion were more likely to have aortic stenosis, and within the R/N group, patients with aortic insufficiency had a greater degree of ascending aorta dilation (p = 0.04).


CONCLUSIONS
Our study suggests that in young patients with bicuspid aortic valves and aortic dilation, aortic valve morphology may be associated with the patterns of aortic dilation and valve dysfunction. Patients with R/N fusion were more likely to have ascending aorta dilation, whereas patients with R/L fusion were more likely to have dilation of the aortic root. In addition, patients with R/N fusion presented at a younger age and were more likely to have aortic stenosis. Recognition of these differences may eventually be helpful for patient counseling and the planning of follow-up.
Section of Pediatric Cardiovascular Surgery and Pediatric Cardiology, University of Illinois College of Medicine at Peoria, Children's Hospital of Illinois, OSF Saint Francis Medical Center, Peoria, Illinois.
Mark
M
Ruzmetov
Section of Pediatric Cardiovascular Surgery and Pediatric Cardiology, University of Illinois College of Medicine at Peoria, Children's Hospital of Illinois, OSF Saint Francis Medical Center, Peoria, Illinois.
Jitendra J
JJ
Shah
Section of Pediatric Cardiovascular Surgery and Pediatric Cardiology, University of Illinois College of Medicine at Peoria, Children's Hospital of Illinois, OSF Saint Francis Medical Center, Peoria, Illinois.
Randall S
RS
Fortuna
Section of Pediatric Cardiovascular Surgery and Pediatric Cardiology, University of Illinois College of Medicine at Peoria, Children's Hospital of Illinois, OSF Saint Francis Medical Center, Peoria, Illinois. Electronic address: karl.f.welke@osfhealthcare.org.
Karl F
KF
Welke
D000293:Adolescent; D000328:Adult; D001013:Aorta, Thoracic / Q000000981:diagnostic imaging*; D001021:Aortic Valve / Q000002:abnormalities* / Q000000981:diagnostic imaging*; D000082882:Bicuspid Aortic Valve Disease; D002648:Child; D002675:Child, Preschool; D004108:Dilatation, Pathologic / Q000000981:diagnostic imaging / Q000209:etiology; D018618:Echocardiography, Doppler, Color / Q000379:methods*; D005260:Female; D005500:Follow-Up Studies; D006349:Heart Valve Diseases / Q000150:complications / Q000175:diagnosis*; D006801:Humans; D007223:Infant; D008297:Male; D012189:Retrospective Studies; D012720:Severity of Illness Index; D055815:Young Adult