Concomitant cardiovascular malformations in isolated bicuspid aortic valve disease: a retrospective cross-sectional study and meta-analysis
Open accessAbstract
Background: Congenital bicuspid aortic valve affects up to 2% of the general population. It occurs in complex congenital heart defects or in syndromes such as Turner, Marfan, or Loeys-Dietz. However, the majority of bicuspid aortic valves are considered to manifest as isolated malformations. Methods: We aimed to assess retrospectively associated cardiovascular malformations in 200 individuals with bicuspid aortic valve considered to occur as an isolated manifestation. All individuals underwent transthoracic echocardiography, 164 thoracoabdominal tomographic imaging, and 84 coronary artery imaging. In addition, we also performed a meta-analysis of data from the literature to assess the occurrence of associate malformations. Results: In our retrospective cross-sectional study collective, the mean age was 45±15 years, 154 (77%) individuals were male. Anatomy of bicuspid aortic valve according to Schaefer was type 1 in 142 (71%), type 2 in 35 (18%), type 3 in 2 (1%), unicuspid in 6 (3%), and unclassified in 15 (8%) individuals. Coarctation of the aorta had 4.2% of individuals, 3.6% had coronary anomalies. No individual had a patent ductus arteriosus, 0.5% had atrial and ventricular septal defect each, 1.5% mitral valve prolapse. No individual had a tricuspid valve prolapse. Our meta-analysis identified in cohorts with isolated bicuspid aortic valve 11.8% (95% CI: 7.7-16.0%) individuals with aortic coarctation, 3.7% (95% CI: 1.2-6.1%) with coronary anomalies, 3.3% (95% CI: 0.0-6.7%) with patent ductus arteriosus, 5.9% (95% CI: 1.3-10.5%) with ventricular septal defect and 1.6% (95% CI: 1.1-2.1%) with mitral valve prolapse. Conclusions: Individuals with isolated bicuspid aortic valve may exhibit a variety of associated cardiovascular malformations and therefore screening for associated malformations may be warranted.
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BAV is frequently associated with thoracic aortic dilation, coronary anomalies, coarctation, and other left-sided congenital lesions.
“BAV is frequently associated with thoracic aortic dilation, coronary anomalies, coarctation, and other left-sided congenital lesions,406 which should be actively screened for if BAV is identified.”
From Clinical practice guidelines for the care of girls and women with Turner syndrome. (2024) · cites this paper as [406]
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Clinical practice guidelines for the care of girls and women with Turner syndrome.
Gravholt, C. H., Andersen, N. H., Christin-Maitre, S., Davis, S. M., Duijnhouwer, A., Gawlik, A., Maciel-Guerra, A. T., Gutmark-Little, I., Fleischer, K., Hong, D., et al. (2024) · European Journal of Endocrinology · doi:10.1093/ejendo/lvae050 · Cited by 224 · see citing works
- Authors
- Szöcs, K., Toprak, B., Schön, G., Rybczynski, M., Brinken, T., Mahlmann, A., Girdauskas, E., Blankenberg, S., & von Kodolitsch, Y.
- Affiliations
- Universität Hamburg (DE)
- University Medical Center Hamburg-Eppendorf (DE)
- St. Josefs Hospital (DE)
- ORCID
- Venue
- Cardiovascular Diagnosis and Therapy
- Publisher
- AME Publishing Company
- Published
- 2022-08
- Volume
- 12
- Issue
- 4
- Pages
- 400-414
- ISSN
- 2223-3652 (ISSN-L)
- 2223-3660
- PMC
- PMC9412207
- OpenAlex
- W4291340455
- Cited by
- 10
- References
- 71
- Citations by year
- 2026: 4 · 2025: 4 · 2024: 1 · 2023: 1
- FWCI
- 0.97
- Type
- journal article
- Language
- en
- Open access
- diamond
- doi.org
- Licence
- cc-by-nc-nd
- Locations
- Cardiovascular Diagnosis and Therapy (publishedVersion, cc-by-nc-nd)
- PubMed Central (publishedVersion, cc-by-nc-nd)
- PubMed (publishedVersion)
- Topics
- Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Aortic Disease and Treatment Approaches
- Health Sciences › Medicine › Cardiology and Cardiovascular Medicine › Cardiac Valve Diseases and Treatments
- Health Sciences › Medicine › Epidemiology › Congenital Heart Disease Studies
- Keywords
- bicuspid aortic valve, coarctation of the aorta, coronary artery anomalies, patent ductus arteriosus, ventricular septal defect, mitral valve prolapse, congenital heart disease, Turner syndrome, Marfan syndrome, Loeys-Dietz syndrome
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