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

https://doi.org/10.1016/j.amjcard.2009.05.045Get rights and content

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.

Section snippets

Methods

Since December 1989, all patients ≥15 years old diagnosed with a congenital cardiac anomaly at La Paz University Hospital (Madrid, Spain) were referred to the Adult Congenital Heart Disease Unit and prospectively followed up. Until June 2006, 2,776 patients had been included in our database. In this cohort, we identified 631 patients with BAV diagnosed by 2-dimensional echocardiography or surgical inspection and without associated complex congenital heart disease, except for a ventricular

Results

The median patient age at presentation for the overall population was 32 years (interquartile range 18 to 51), and 68% (n = 425) were men. A total of 29 AACs were found (4.7%), including aortic root or AA dilation ≥55 mm in 20, type A aortic dissection in 7, and ruptured sinus of Valsalva or AA aneurysm in 2. Univariate and multivariate analyses (logistic regression) showed that only the co-existence of a COA was a significant discriminate factor for the primary end point (odds ratio 4.7, 95%

Discussion

The results of the present study have demonstrated that the incidence of aortic root or AACs in patients with isolated BAV, who did not present with these complications at clinical onset, is very low with long-term follow-up. However, patients with an associated COA were at an increased risk of a new aortic root or ascending aorta event. These data highlight the interaction among COA, BAV, and intrinsic disease of the aortic wall9 (Figure 3).

A ninefold increased risk of type A aortic dissection

References (30)

  • F. Robicsek et al.

    The congenitally bicuspid aortic valve: how does it function? Why does it fail?

    Ann Thorac Surg

    (2004)
  • W.G. Guntheroth

    A Critical Review of the American College of Cardiology/American Heart Association practice guidelines on bicuspid aortic valve with dilated ascending aorta

    Am J Cardiol

    (2008)
  • A.Z. Beaton et al.

    Relation of coarctation of the aorta to the occurrence of ascending aortic dilation in children and young adults with bicuspid aortic valves

    Am J Cardiol

    (2009)
  • R.J. Okamoto et al.

    The influence of mechanical properties on wall stress and distensibility of the dilated ascending aorta

    J Thorac Cardiovasc Surg

    (2003)
  • C.A. Warnes et al.

    ACC/AHA 2008 Guidelines for the Management of Adults With Congenital Heart Disease: Executive Summary: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (writing committee to develop guidelines for the management of adults with congenital heart disease): developed in collaboration with the American Society of Echocardiography, Heart Rhythm Society, International Society for Adult Congenital Heart Disease, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons

    Circulation

    (2008)
  • Cited by (0)

    View full text