{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,14]],"date-time":"2026-09-14T11:07:59Z","timestamp":1789384079617,"version":"build-2803163510"},"reference-count":142,"publisher":"MDPI AG","issue":"5","license":[{"start":{"date-parts":[[2026,9,13]],"date-time":"2026-09-13T00:00:00Z","timestamp":1789257600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Med. Sci.","Medical Sciences"],"abstract":"<jats:p>Background: Bicuspid aortic valve disease (BAVD) is the most frequent congenital heart disease, occurring either as an isolated lesion or in association with other congenital cardiovascular malformations, with variable patterns of progression and risk of valvular and vascular complications. Studies addressing pediatric bicuspid aortopathy are still in their infancy. This narrative review aims to provide a comprehensive perspective on the current scientific evidence regarding BAVD-associated aortopathy in pediatric patients, underscoring multiple challenges in initial diagnosis, long-term surveillance, and therapeutic decision-making, and focusing on the potential roles of circulating biomarkers and advanced multimodal imaging tools that may improve individualized risk stratification. Methods: Despite the narrative design of this review, a structured search of the current available literature was performed to identify studies addressing pediatric BAV, associated aortopathy, biomarkers, vascular remodeling, and multimodal imaging. Priority was given to pediatric cohorts, longitudinal studies, consensus documents, and contemporary guidelines. The search was conducted in the online databases PubMed\/Medline and Web of Science for English-language original articles published in the last 10 years, up to May 2026. We used the following main terms: \u201cBicuspid Aortic Valve Disease\u201d [MeSH], \u201cAorta\u201d [MeSH], \u201cInfant\u201d [MeSH], \u201cChild\u201d [MeSH], \u201cAdolescent\u201d [MeSH], combined by Boolean operators with secondary keywords: \u201cpathogenesis\u201d, \u201cmechanism\u201d, \u201cprogression\u201d, \u201cmultimodal imaging\u201d, \u201cechocardiography\u201d, \u201ccardiovascular magnetic resonance imaging\u201d, \u201ccomputed tomography\u201d, \u201ccirculating biomarkers\u201d. The retrieved studies were screened for eligibility using previously established inclusion and exclusion criteria. A total of 63 studies were included in the analysis for this narrative review. Results: Two main theories underpin the etiopathogenesis of aortopathy associated with the bicuspid aortic valve, positing that genetic factors predispose the aortic wall to remodeling in an abnormal hemodynamic environment. Embryological development and dysregulation of molecular and cellular structures are also intertwined during the formation and progression of the aortic valve with two semilunar cusps, resulting in consequent alterations in the aortic wall\u2019s architectural organization. Advances in molecular studies have highlighted circulating biomarkers with potential utility in predicting aortopathy, as they are involved in extracellular matrix remodeling, endothelial dysfunction, and aortic valve calcification, including matrix metalloproteinases (MMPs) and their tissue inhibitors (TIMPs), transforming growth factor-\u03b2 (TGF-\u03b2), and microRNAs. In addition, multimodal imaging techniques have emerged as essential tools for assessing the morphology and function of cardiovascular structures, particularly aortic biomechanical properties and hemodynamic abnormalities, at the time of initial diagnosis and during regular monitoring. Taken together, blood biomarkers and imaging parameters of aortic remodeling and flow disturbances might gain increasing prognostic value in pediatric BAVD-associated aortopathy. However, larger longitudinal studies are required for clinical validation beyond research settings. Although guidelines on the management of BAVD are available for adults, they are not entirely applicable to children, who are undergoing continuous somatic growth that affects diagnostic possibilities and therapeutic options. Conclusions: Despite growing literature in the realm of BAVD and related conditions, the management of pediatric patients remains challenging in daily clinical practice, as adult guidelines cannot be completely applied to children. Given the heterogeneity and complexity of pathogenic mechanisms, clinical presentations, natural history, and outcomes, future research is warranted to explore the progression profiles of valvular and vascular disorders associated with BAV in children and to achieve an optimal approach to pediatric bicuspid aortopathy.<\/jats:p>","DOI":"10.3390\/medsci14050567","type":"journal-article","created":{"date-parts":[[2026,9,14]],"date-time":"2026-09-14T10:19:41Z","timestamp":1789381181000},"page":"567","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Bicuspid Aortic Valve Disease-Associated Aortopathy in Pediatric Subjects\u2014From Traditional Assessment to Current Advances and Future Perspectives: A Narrative Review"],"prefix":"10.3390","volume":"14","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-2826-3112","authenticated-orcid":false,"given":"Oana Iulia","family":"Man","sequence":"first","affiliation":[{"name":"Department of Internal Medicine, Iuliu Ha\u021bieganu University of Medicine and Pharmacy, 400012 Cluj-Napoca, Romania"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lucia","family":"Agoston-Coldea","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, Iuliu Ha\u021bieganu University of Medicine and Pharmacy, 400012 Cluj-Napoca, Romania"},{"name":"2nd Department of Internal Medicine Clinic, Emergency County Hospital, 400006 Cluj-Napoca, Romania"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7649-5308","authenticated-orcid":false,"given":"Cecilia","family":"Lazea","sequence":"additional","affiliation":[{"name":"Department 8, Mother and Child, Iuliu Ha\u021bieganu University of Medicine and Pharmacy, 400370 Cluj-Napoca, Romania"},{"name":"1st Pediatrics Clinic, Emergency Pediatric Clinical Hospital, 400370 Cluj-Napoca, Romania"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2026,9,13]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","unstructured":"Katsaros, O., Ktenopoulos, N., Korovesis, T., Benetos, G., Apostolos, A., Koliastasis, L., Sagris, M., Milaras, N., Latsios, G., and Synetos, A. 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