Unicuspid Aortic Valve: Transesophageal Echocardiographic Features

Tempe, Deepak K; Garg, Mukesh; Tomar, Akanksha; Dutta, Devesh; Dutta, Rahul; Singh, Aditya Kumar

doi:10.1053/j.jvca.2011.01.011

Cited by 8 publications

(12 citation statements)

References 9 publications

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“…A UAV is a rare congenital cardiac anomaly with an estimated incidence of 0.02% in the general population and 4–6% in patients presenting for isolated AV replacement (3, 10). UAV results when two of the commissures fail to develop between the three cusps that normally form the AV during valvulogenesis (11).…”

Section: Discussionmentioning

confidence: 99%

“…The former is the most common and creates a larger effective orifice area that allows better blood flow into adulthood. The latter is characterized by a central orifice and earlier clinical presentation from premature valvular stenosis during childhood (3, 12). …”

Section: Discussionmentioning

confidence: 99%

“…Echocardiographic characteristics of UAV include the visualization of a single AV leaflet with an eccentric orifice and single commissural attachment zone in unicommissural valves, whereas acommissural valves have a central orifice without a commissural attachment. Misdiagnosis of a UAV for bicuspid or tricuspid AV can result from visualization of a raphe or calcifications mimicking a raphe, which can resemble true commissures in diastole (3, 11, 15). …”

Section: Discussionmentioning

confidence: 99%

“…In spite of the high incidence of AV anomalies in TS patients, the predominant pathology remains bicuspid aortic stenosis (AS) (1, 2). Congenital unicuspid aortic valve (UAV) is a very rare anomaly associated with premature AS and accounts for approximately 5% of all isolated stenotic AV replacements (3–5). Transthoracic echocardiography (TTE) fails to clearly define AV morphology in 10–40% of patients, despite being the primary imaging modality for the diagnosis and assessment of AS severity (3, 6, 7).…”

Section: Introductionmentioning

confidence: 99%

“…Congenital unicuspid aortic valve (UAV) is a very rare anomaly associated with premature AS and accounts for approximately 5% of all isolated stenotic AV replacements (3–5). Transthoracic echocardiography (TTE) fails to clearly define AV morphology in 10–40% of patients, despite being the primary imaging modality for the diagnosis and assessment of AS severity (3, 6, 7). We present a case of intraoperative diagnosis of unicuspid AS by transesophageal echocardiography (TEE) in a patient with TS presenting for aortic valve replacement (AVR) with a preoperative diagnosis of bicuspid AS.…”

Section: Introductionmentioning

confidence: 99%

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Unicuspid Aortic Stenosis in a Patient with Turner Syndrome: A Case Report

Essandoh

Castellon-Larios

Zuleta-Alarcon

et al. 2014

Front. Cardiovasc. Med.

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Congenital aortic valve anomalies are the cause of premature aortic stenosis in pediatric and younger adult populations. Despite being very rare, unicuspid aortic valves account for approximately 5% of isolated aortic valve replacements. Patients with aortic stenosis, present with the same symptomatology independent of leaflet morphology. However, the presence of bicuspid and unicuspid aortic stenosis is associated with a higher incidence of aortopathy, especially in Turner syndrome patients. Turner syndrome, an X monosomy, is associated with aortic valve anomalies, aortopathy, and hypertension. These risk factors lead to a higher incidence of aortic dissection in this population. Patients with Turner syndrome and aortic stenosis that present for aortic valve replacement should therefore undergo extensive aortic imaging prior to surgery. Transthoracic echocardiography is the diagnostic tool of choice for valvular pathology, yet it can misdiagnose unicuspid aortic valves as bicuspid valves due to certain similarities on imaging. Transesophageal echocardiography is a better tool for distinguishing between the two valvular abnormalities, although diagnostic errors can still occur. We present a case of a 50-year-old female with history of Turner syndrome and bicuspid aortic stenosis presenting for aortic valve replacement and ascending aorta replacement. Intraoperative transesophageal echocardiography revealed a stenotic unicommissural unicuspid aortic valve with an eccentric orifice, which was missed on preoperative imaging. This case highlights the importance of intraoperative transesophageal echocardiography in confirming preoperative findings, diagnosing further cardiac pathology, and ensuring adequate surgical repair.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 3 more Smart Citations

Unicuspid Aortic Stenosis in a Patient with Turner Syndrome: A Case Report

Essandoh

Castellon-Larios

Zuleta-Alarcon

et al. 2014

Front. Cardiovasc. Med.

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Congenital unicuspid aortic valve stenosis in siblings

Kawahara

Suzuki

Takahara

et al. 2013

Gen Thorac Cardiovasc Surg

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We reported the case of unicuspid aortic valve in sibling, suggesting the familial incidence and genetic relation. A 41-year-old man (elder brother) with 79 mmHg of aortic valvular peak pressure gradient (peak PG) underwent aortic valve replacement with mechanical prosthetic valve (ATS 21 mm). The intraoperative finding showed the unicuspid aortic valve with one rudimentary commissure. A 37-year-old woman (sister) had been followed by echocardiography as bicuspid aortic valve since 10 years old. After 27 years, the peak PG had increased to 176 mmHg on preoperative echocardiography. The aortic valve replacement was performed. The bioprosthetic valve (MOSAIC 21 mm) was implanted supra-annularly because the patient required pregnancy. The intraoperative finding showed the unicuspid aortic valve with two rudimentary commissures and one opening of LCC-RCC commissure. The histopathological study of both patients confirmed as congenitally unicuspid aortic valve. In our best knowledge, this is a first report of unicuspid aortic valve in sibling.

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Intervention and Management of Congenital Left Heart Obstructive Lesions

Schimke

Majithia

Baumgärtner

et al. 2013

Curr Treat Options Cardio Med

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While congenital heart defects are typically diagnosed in early childhood, depending on the severity of the lesion, they may not be recognized until later childhood or in adulthood. Left heart pressure loading lesions, including mitral valve stenosis, subaortic stenosis, valvular aortic stenosis, supravalvular aortic stenosis, and coarctation of the aorta, may be found as an isolated lesion or as a constellation together known as Shone syndrome. This review will highlight the literature published regarding the diagnosis, intervention, and long-term management of left heart pressure loading lesions.

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Unicuspid Aortic Valve: Transesophageal Echocardiographic Features

Cited by 8 publications

References 9 publications

Unicuspid Aortic Stenosis in a Patient with Turner Syndrome: A Case Report

Unicuspid Aortic Stenosis in a Patient with Turner Syndrome: A Case Report

Congenital unicuspid aortic valve stenosis in siblings

Intervention and Management of Congenital Left Heart Obstructive Lesions

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