Risk of Aortic Root or Ascending Aorta Complications in Patients With Bicuspid Aortic Valve With and Without Coarctation of the Aorta

• Published in: The American journal of cardiology 2009-10, Vol.104 (7), p.1001-1006
• Main Authors: Oliver, Jose Maria, MD, Alonso-Gonzalez, Rafael, MD, Gonzalez, Ana Elvira, MD, Gallego, Pastora, MD, Sanchez-Recalde, Angel, MD, Cuesta, Emilio, MD, Aroca, Angel, MD, Lopez-Sendon, Jose Luis, MD
• Format: Article
• Language: English
• Subjects: Abnormalities, Multiple - diagnostic imaging; Abnormalities, Multiple - epidemiology; Adolescent; Adult; Analysis of Variance; Aneurysm, Dissecting - diagnostic imaging; Aneurysm, Dissecting - epidemiology; Aneurysm, Dissecting - etiology; Aneurysm, Ruptured - diagnostic imaging; Aneurysm, Ruptured - epidemiology; Aneurysm, Ruptured - etiology; Aneurysms; Aorta - diagnostic imaging; Aorta - surgery; Aortic Coarctation - complications; Aortic Coarctation - diagnostic imaging; Aortic Coarctation - surgery; Aortic Valve - abnormalities; Aortic Valve - surgery; Biological and medical sciences; Blood and lymphatic vessels; Cardiology; Cardiology. Vascular system; Cardiovascular; Cardiovascular disease; Cohort Studies; Congenital heart diseases. Malformations of the aorta, pulmonary vessels and vena cava; Diseases of the aorta; Diseases of the peripheral vessels. Diseases of the vena cava. Miscellaneous; Echocardiography, Doppler; Endocardial and cardiac valvular diseases; Female; Heart; Heart Aneurysm - diagnostic imaging; Heart Aneurysm - etiology; Heart Aneurysm - mortality; Heart Aneurysm - surgery; Heart Defects, Congenital - complications; Heart Defects, Congenital - diagnostic imaging; Heart surgery; Humans; Incidence; Logistic Models; Male; Medical procedures; Medical sciences; Middle Aged; Mitral Valve - diagnostic imaging; Mitral Valve - surgery; Multivariate Analysis; Probability; Retrospective Studies; Risk Assessment; Risk factors; Survival Analysis; Young Adult
• ISSN: 0002-9149; 1879-1913
• DOI: 10.1016/j.amjcard.2009.05.045

The actual incidence of ascending aorta complications (AACs) in adults with bicuspid aortic valve (BAV) and the role of associated coarctation of the aorta (COA) as an independent risk factor for AACs remain unknown. From the Adult Congenital Heart Disease database at La Paz Hospital, 631 patients in whom a BAV was diagnosed by echocardiography or surgical inspection since December 1989 were identified. These patients were then further subdivided into 2 groups according to the presence of an associated COA. AACs included aortic aneurysms (ascending aorta ≥55 mm) and aortic dissection, rupture, or perforation. Patients with a BAV and COA had a greater prevalence of AACs (8.0%) than those with an isolated BAV (3.7%; p = 0.037). The coexistence of COA was the only significant predictor of AACs (odds ratio 4.7, 95% confidence interval 1.5 to 15; p = 0.01). From the total patient group with a BAV, the clinical and echocardiographic data were reviewed for 341 patients without an AAC at baseline (97 with and 244 without COA) who had undergone serial examinations >1 year apart. The median follow-up was 7 years (interquartile range 3.5 to 10.2; total 2,436 patient-years). A new AAC occurred in 13 patients (0.5/100 patient-years). The incidence of AACs was 1.3/100 patient-years in the COA group versus 0.2/100 patient-years in the non-COA group (hazard ratio 7.5, 95% confidence interval 2.0 to 28, p = 0.002). All acute aortic events (dissection or rupture) at follow-up occurred in patients with a BAV and COA. In conclusion, the long-term incidence of AACs in patients with isolated BAV is low, but patients with BAV and associated COA are at increased risk.