Cardiac magnetic resonance imaging of congenital bicuspid aortic valves and associated aortic pathologies in adults.
OBJECTIVE
Bicuspid aortic valve (BAV) represents the most frequent congenital cardiac abnormality resulting in premature valvular degeneration and aortic dilatation. In a large series of consecutive patients, we evaluated the distribution of BAV types and the associated valvular and aortic abnormalities.


RESULTS
We investigated 266 patients (58 ± 14 years) with BAV using a 1.5 T cardiac magnetic resonance (CMR) scanner. Valve morphology was described according to the Sievers classification. The aortic valve orifice area, aortic regurgitation (AR) fraction, and aortic dilation were quantified. Two hundred and forty-two data sets were available for analysis; 24% had BAV without a valvular lesion. The predominant valvular lesion was aortic stenosis (AS) with 51%. Lone AR was found in 17%. A combined lesion of AS and AR was found in 9%. Those with AS were older than the overall average (64 ± 12 vs. 57 ± 15 years, P < 0.001). The patients with AR and those without valvular abnormality were younger than average (49 ± 13 and 50 ± 12 years vs. 57 ± 15 years, P < 0.01 respectively). Comparing two observers Kappa coefficient was 0.77 for differentiation of six valve morphologies and 0.80 for the differentiation of bicuspid and tricuspid valve. Aortic dilatation was found in 39% of cases with no discernible preference for any specific BAV-type and mainly affecting the ascending aorta.


CONCLUSIONS
CMR can non-invasively differentiate various morphologies in BAV with low inter-observer variability. Valvular pathologies vary across age. Aortic dilatation is frequent in BAV independent from valvular morphology or lesion. In future CMR might help to guide management in patients with BAV.
Working Group Cardiac MRI, Humboldt University Berlin, Charite Campus Buch, Experimental and Clinical Research Center, Berlin, Germany Cardiology and Nephrology Department, HELIOS Klinikum Berlin Buch, 13125, Berlin, Germany ralf.wassmuth@charite.de.
Ralf
R
Wassmuth
Working Group Cardiac MRI, Humboldt University Berlin, Charite Campus Buch, Experimental and Clinical Research Center, Berlin, Germany Cardiology and Nephrology Department, HELIOS Klinikum Berlin Buch, 13125, Berlin, Germany.
Florian
F
von Knobelsdorff-Brenkenhoff
Working Group Cardiac MRI, Humboldt University Berlin, Charite Campus Buch, Experimental and Clinical Research Center, Berlin, Germany Cardiology and Nephrology Department, HELIOS Klinikum Berlin Buch, 13125, Berlin, Germany.
Henriette
H
Gruettner
Working Group Cardiac MRI, Humboldt University Berlin, Charite Campus Buch, Experimental and Clinical Research Center, Berlin, Germany Cardiology and Nephrology Department, HELIOS Klinikum Berlin Buch, 13125, Berlin, Germany.
Wolfgang
W
Utz
Working Group Cardiac MRI, Humboldt University Berlin, Charite Campus Buch, Experimental and Clinical Research Center, Berlin, Germany Cardiology and Nephrology Department, HELIOS Klinikum Berlin Buch, 13125, Berlin, Germany.
Jeanette
J
Schulz-Menger
D000328:Adult; D000368:Aged; D001021:Aortic Valve / Q000002:abnormalities* / Q000473:pathology; D001022:Aortic Valve Insufficiency / Q000175:diagnosis*; D001024:Aortic Valve Stenosis / Q000175:diagnosis*; D000082882:Bicuspid Aortic Valve Disease; D015331:Cohort Studies; D016001:Confidence Intervals; D016208:Databases, Factual; D005260:Female; D006349:Heart Valve Diseases / Q000151:congenital / Q000473:pathology*; D006801:Humans; D007090:Image Interpretation, Computer-Assisted; D019028:Magnetic Resonance Imaging, Cine / Q000379:methods*; D008297:Male; D008875:Middle Aged; D015588:Observer Variation; D011446:Prospective Studies; D015203:Reproducibility of Results
Aorta; Aortic stenosis; Bicuspid aortic valve; Magnetic resonance imaging