Unicuspid Aortic Valve: A Rare Congenital Anomaly

Cardiology. 2022;147(2):207-215. doi: 10.1159/000521623. Epub 2021 Dec 29.


Unicuspid aortic valve (UAV) is a rare congenital aortic valve anomaly. It has 2 subtypes and often is associated with aortic valve dysfunction and corresponding clinical presentations. Echocardiography is the first choice of diagnostic method for UAV. Three-dimensional echocardiography has played an increasingly important role in diagnosis, intraprocedural guidance, and post-procedural assessment in recent years. There remain challenges in distinguishing UAV from bicuspid aortic valve or tricuspid aortic valve. Misdiagnosis is most resulted from raphes or leaflet calcifications. Multi-modality imaging has obvious advantages over single-modality imaging and is crucial for UAV diagnosis. Accurate identification of aortic valve morphology is important to choose the best treatment. Aortic balloon valvoplasty, surgical valvotomy, commissurotomy, Ross procedure, surgical valve repair, and surgical replacement are most common treatment modalities of UAV. In this review, we aim to discuss UAV including epidemiology, definition, classification, diagnostic importance and necessity, valvular function, clinical presentation, associated disorders, noninvasive diagnostic modalities, and therapeutic approaches.

Keywords: Aortic stenosis; Echocardiography; Unicuspid aortic valve.

Publication types

  • Review

MeSH terms

  • Aortic Valve / abnormalities
  • Aortic Valve / diagnostic imaging
  • Aortic Valve / surgery
  • Aortic Valve Stenosis*
  • Echocardiography / methods
  • Heart Defects, Congenital* / diagnosis
  • Heart Valve Diseases* / diagnostic imaging
  • Heart Valve Diseases* / surgery
  • Humans

Supplementary concepts

  • unicuspid aortic valve