Unicuspid Aortic Valve: A Rare Congenital Anomaly

Abstract

Unicuspid aortic valve (UAV) is a rare congenital aortic valve anomaly. It has two 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 (BAV) or tricuspid aortic valve (TAV). 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, non-invasive diagnostic modalities and therapeutic approaches.

Diagnosis of unicuspid aortic valve after loss of consciousness during cesarean section: A case report

“…A major concern results from raphes or leaflet calcification. Recently, three‐dimensional (3D) echocardiography has become increasingly important in their diagnosis 8 . UAV can be further classified into acommissural UAV and unicommissural UAV based on the morphology of the aortic valve 9 This case was categorized as unicommissural UAV, which has a relatively large orifice area compared to acommissural UAV, and is associated with the appearance of symptoms in adults 9 Furthermore, the aortic valve in our patient was an overlong valve with a soft root and stiff tip.…”

“…Recently, three-dimensional (3D) echocardiography has become increasingly important in their diagnosis. 8 UAV can be further classified into acommissural UAV and unicommissural UAV based on the morphology of the aortic valve 9 This case was categorized as unicommissural UAV, which has a relatively large orifice area compared to acommissural UAV, and is associated with the appearance of symptoms in adults 9 Furthermore, the aortic valve in our patient was an overlong valve with a soft root and stiff tip. Therefore, it was incidentally detected as a mass near the right coronary apex of the aortic valve during the chest CT scan performed during the cesarean section postoperative analysis.…”

Bicuspid Aortic Valve in Children and Adolescents: A Comprehensive Review

“…A differential diagnosis should be made between BAV and unicuspid aortic valve (UAV). UAV is a rare condition which accounts for 4–5% of cases undergoing valve surgery for AS and is a frequent cause of intervention in younger patients (<25 years) [ 24 , 25 ]. In paediatric patients, UAV may often be diagnosed by TTE looking at the parasternal short axis view of the aortic valve due to the usually good-quality images and lack of valve calcification ( Figure 2 ); however, three-dimensional echocardiography has potential advantages over conventional TTE in confirming the diagnosis as well as in the monitoring of interventional procedures [ 24 ].…”

“…A differential diagnosis should be made between BAV and unicuspid aort (UAV). UAV is a rare condition which accounts for 4-5% of cases undergoing va gery for AS and is a frequent cause of intervention in younger patients (<25 years In paediatric patients, UAV may often be diagnosed by TTE looking at the par short axis view of the aortic valve due to the usually good-quality images and lack calcification (Figure 2); however, three-dimensional echocardiography has pote vantages over conventional TTE in confirming the diagnosis as well as in the mo of interventional procedures [24]. The ascending aorta is evaluated in parasternal long axis view at four differe (aortic annulus, sinuses of Valsalva, sino-tubular junction and proximal ascendin Due to the usually favorable acoustic window in children, TTE is suitable for a The ascending aorta is evaluated in parasternal long axis view at four different levels (aortic annulus, sinuses of Valsalva, sino-tubular junction and proximal ascending aorta).…”

Unicuspid aortic valve: More data and more doubts in the light of six years of follow-up observation

“…An unicuspid aortic valve is a rare congenital heart defect with an incidence is 0.02% [1,2]. The optimal time for cardiosurgical treatment in young adults with congenital aortic valve disease may be a matter of controversy [3,4]. In this population, left ventricle (LV) remodeling is an ongoing process from organogenesis onwards [5], and its degree may not match the severity of the defect.…”