Aortic assessment of bicuspid aortic valve patients and their first-degree relatives.
Background Bicuspid aortic valve patients have an increased risk of aortic dilatation. A deficit of nitric oxide synthase has been proposed as the causative factor. No correlation between flow-mediated dilation and aortic diameter has been performed in patients with bicuspid aortic valves and normal aortic diameters. Being a hereditary disease, we compared echocardiographic features and endothelial function in these patients and their first-degree relatives. Methods Comprehensive physical examinations, routine laboratory tests, transthoracic echocardiography, and measurements of endothelium-dependent and non-dependent flow-mediated vasodilatation were performed in 18 bicuspid aortic valve patients (14 type 1 and 4 type 2) and 19 of their first-degree relatives. Results The first-degree relatives were younger (36.7 ± 18.8 vs. 50.5 ± 13.9 years, p = 0.019) with higher ejection fractions (64.6% ± 1.7% vs. 58.4% ± 9.5%, p = 0.015). Aortic diameters indexed to body surface area were similar in both groups, the except the tubular aorta which was larger in bicuspid aortic valve patients (19.3 ± 2.7 vs. 17.4 ± 2.2 mm·m-2, p = 0.033). Flow-dependent vasodilation was similar in both groups. A significant inverse correlation was found between non-flow-dependent vasodilation and aortic root diameter in patients with bicuspid aortic valve ( R = -0.57, p = 0.05). Conclusions Bicuspid aortic valve patients without aortopathy have larger ascending aortic diameters than their first-degree relatives. Endothelial function is similar in both groups, and there is no correlation with ascending aorta diameter. Nonetheless, an inverse correlation exists between non-endothelial-dependent dilation and aortic root diameter in bicuspid aortic valve patients.
Department of Cardiology, University Hospital, Montevideo, Uruguay.
Pablo
P
Straneo
Department of Cardiology, University Hospital, Montevideo, Uruguay.
Gabriel
G
Parma
Department of Cardiology, University Hospital, Montevideo, Uruguay.
Natalia
N
Lluberas
Department of Cardiology, University Hospital, Montevideo, Uruguay.
Alvaro
A
Marichal
Department of Cardiology, University Hospital, Montevideo, Uruguay.
Gerardo
G
Soca
Department of Cardiology, University Hospital, Montevideo, Uruguay.
Leandro
L
Cura
Department of Cardiology, University Hospital, Montevideo, Uruguay.
Juan J
JJ
Paganini
Department of Cardiology, University Hospital, Montevideo, Uruguay.
Daniel
D
Brusich
Department of Cardiology, University Hospital, Montevideo, Uruguay.
Lucia
L
Florio
Department of Cardiology, University Hospital, Montevideo, Uruguay.
Victor
V
Dayan
D000328:Adult; D001013:Aorta, Thoracic / Q000000981:diagnostic imaging* / Q000503:physiopathology; D017545:Aortic Aneurysm, Thoracic / Q000175:diagnosis / Q000453:epidemiology / Q000209:etiology*; D001021:Aortic Valve / Q000002:abnormalities*; D001027:Aortography; D000082882:Bicuspid Aortic Valve Disease; D004452:Echocardiography; D005190:Family; D005260:Female; D006349:Heart Valve Diseases / Q000150:complications* / Q000175:diagnosis; D006801:Humans; D015994:Incidence; D008297:Male; D008875:Middle Aged; D011446:Prospective Studies; D012189:Retrospective Studies; D012307:Risk Factors; D014583:Uruguay / Q000453:epidemiology; D014664:Vasodilation
Aorta; Aortic aneurysm; Aortic valve; Aortography; Dilatation; Heart defects; congenital; pathologic