J Cardiovasc Imaging.  2019 Apr;27(2):105-118. 10.4250/jcvi.2019.27.e19.

Mid-term Clinical Outcomes in a Cohort of Asymptomatic or Mildly Symptomatic Korean Patients with Bicuspid Aortic Valve in a Tertiary Referral Hospital

• Sun BJ
• Oh JK
• Lee SH
• Jang JY
• Lee JH
• Lee S
• Kim DH
• Song JM
• Kang DH
• Song JK

• KMID: 2442744
• DOI: http://doi.org/10.4250/jcvi.2019.27.e19

Abstract

BACKGROUND
Although bicuspid aortic valve (BAV) is one of the most common congenital heart diseases, clinical outcome data regarding BAV are still limited. We evaluated clinical characteristics and mid-term clinical outcomes of asymptomatic Korean patients with bicuspid aortic valve.
METHODS
We initiated a prospective registry in 2014 at a tertiary referral hospital. To develop a cohort of asymptomatic patients, we excluded patients who previously underwent open heart surgery (OHS) or who had OHS within 6 months of referral.
RESULTS
A total of 170 patients (117 male [69%], age 50 ± 13 years) were enrolled. More than 70% (n = 124, 73%) were incidentally diagnosed with BAV during routine health examinations or preoperative screening for non-cardiac surgery. At the time of enrollment, moderate to severe aortic stenosis (AS) or regurgitation (AR) was present in 77 patients (45%) and 98 (58%) showed aortic dilation: 42 (25%) had non-significant valvular dysfunction without aortic dilation. During a median follow-up of 4 years, AS severity increased significantly (p < 0.001), while there was no significant change in AR severity (p = 0.361). A total of 27 patients (16%) underwent OHS, including isolated aortic valve (AV) surgery (n = 11, 41%), AV with combined aortic surgery (n = 12, 44%), and isolated aortic surgery (n = 4, 15%): no patient developed aortic dissection. Moderate to severe AS (hazard ratio [HR] 4.61; 95% confidence interval [CI] 1.83-11.62; p = 0.001), NYHA class ≥ 2 (HR 2.53; 95% CI 1.01-6.35; p = 0.048) and aortic dilation (HR 2.13; 95% CI 0.87-5.21; p = 0.099) were associated with surgical events.
CONCLUSIONS
Progression patterns of valvular dysfunction and impacts of BAV phenotype on OHS should be explored in future studies with longer follow-up durations.

Keyword

Bicuspid aortic valve; Aortic valve function; Clinical outcome

MeSH Terms

Aortic Valve Stenosis
Aortic Valve*
Bicuspid*
Cohort Studies*
Follow-Up Studies
Heart Diseases
Humans
Male
Mass Screening
Phenotype
Prospective Studies
Referral and Consultation
Tertiary Care Centers*
Thoracic Surgery

Figure

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  Figure 1 Surgical event-free survival rates (A) and impact of severity of valvular dysfunction or aortic dilation at the time of the initial diagnosis (B-D). Ao: aortic, AR: aortic regurgitation, AS: aortic stenosis, AV: aortic valve.

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  Figure 2 Comparison of total surgical events according to BAV phenotype classified with the conventional (A) and dichotomous (B) methods. BAV: bicuspid aortic valve, CCF: coronary cusp fusion, MCF: mixed cusp fusion.

• 

  Figure 3 Changes of aortic valve function during follow-up (n = 129, median 3.9 years [IQR, 2.7-7.6]). AR: aortic regurgitation, AS: aortic stenosis, IQR: interquartile range.

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