New Insights Into Unicuspid Aortic Valve Disease in Adults: Not Just a Subtype of Bicuspid Aortic Valves

Noly, Pierre‐Emmanuel; Basmadjian, Lauren; Bouhout, Ismail; Le, Van Hoai Viet; Poirier, Nancy; El-Hamamsy, Ismaı̈l

doi:10.1016/j.cjca.2015.10.028

Cited by 40 publications

(45 citation statements)

References 23 publications

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“…If the aortic root or ascending aorta is involved, surgical treatment is usually required. A recent study reported that UAV was specifically accompanied by dilatation at the aortic annulus level, 12) and our patient also showed dilatation, with the ascending aorta of 50 mm and the aortic annulus of 32 mm.…”

Section: Discussionsupporting

confidence: 73%

“…However, given that the prevalence of an UAV is about 0.02% in the adult population, the actual incidence identified by the using echocardiography as a pre-operative diagnostic tool seems to be an underestimate. Of the 231 cases in a systematic review in 2010, only 10% were diagnosed before surgery, 1) and Noly et al 12) showed that only 14% of UAV was diagnosed by the preoperative echocardiography. Other diagnostic tools for an UAV include cardiac CT or cardiac magnetic resonance imaging.…”

Section: Discussionmentioning

confidence: 99%

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Severe Aortic Stenosis Associated with Unicommissural Unicuspid Aortic Valve in a Middle Aged Male

Kwon

Park

Kim

et al. 2016

J Cardiovasc Ultrasound

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Unicuspid aortic valve (UAV) is an extremely rare form of congenital aortic valvular abnormality. Although UAV shows similar clinical characteristics to bicuspid aortic valve, the clinical symptoms develop at earlier age and progress at a faster pace in UAV. In this report, we are presenting a 42-year-old male with severe aortic stenosis associated with unicommissural UAV. The patients underwent a successful Bentall operation.

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Section: Discussionsupporting

confidence: 73%

Section: Discussionmentioning

confidence: 99%

Severe Aortic Stenosis Associated with Unicommissural Unicuspid Aortic Valve in a Middle Aged Male

Kwon

Park

Kim

et al. 2016

J Cardiovasc Ultrasound

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show abstract

“…[12][13][14] Especially in young patients unicuspid valves are more frequent than generally believed, simply because the echocardiographic characteristics are often missed. [15][16][17] Quadricuspid valves are frequent in truncus arteriosus and rare in normal cardiac anatomy 18 ; on the other hand, repair approaches are particularly important in truncus, which usually requires surgery at neonatal or infant stage.…”

Section: Know the Geometry Of The Aortic Valve (And Its Morphologic Smentioning

confidence: 99%

The 10 Commandments for Aortic Valve Repair

Schäfers

2019

Innovations�(Phila)

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“…Even though the patient was diagnosed with aortic stenosis shortly after birth, the diagnosis of UAV was not made until open heart surgery was performed 17 years later. This is common, indeed the majority of UAVs are diagnosed peri-operatively [ 2 , 6 ]. Transesophageal echocardiography (TEE) is the gold standard for diagnosis of UAV with a sensitivity and specificity of 75 and 86% respectively [ 7 ].…”

Section: Discussionmentioning

confidence: 99%

Congenital aortic stenosis due to unicuspid unicommissural aortic valve: a case report

Ingason

Sigfússon

Torfason

2018

J Cardiothorac Surg

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BackgroundUnicuspid unicommissural aortic valve is an extremely rare congenital anomaly that usually presents in adulthood but can rarely present in infancy. We report a 17-year-old patient with congenital aortic stenosis secondary to unicuspid unicommissural aortic valve that was successfully treated with aortic valve replacement.Case presentationThe patient was diagnosed with aortic stenosis after a murmur was heard in the newborn nursery and subsequently underwent aortic balloon valvuloplasty 6 weeks after birth. He had been regularly followed up since and underwent numerous cardiac catheterizations, including another aortic balloon valvuloplasty at age 13. During follow-up at age 17, the patient presented with symptomatic severe aortic stenosis and mild left ventricular hypertrophy. Aortic valve replacement was planned since the patient was nearly adult-sized and to reduce the risk of cardiac decompensation. During the operation an unicuspid unicommissural aortic valve was revealed. The patient recovered well post-operatively. He was discharged 5 days after the surgery in good condition and was completely symptom-free at follow-up 6 weeks later.ConclusionsUnicuspid aortic valve is a rare congenital anomaly that can cause congenital aortic stenosis. It is seldom diagnosed pre-operatively but should be suspected in infants presenting with aortic stenosis.

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New Insights Into Unicuspid Aortic Valve Disease in Adults: Not Just a Subtype of Bicuspid Aortic Valves

Cited by 40 publications

References 23 publications

Severe Aortic Stenosis Associated with Unicommissural Unicuspid Aortic Valve in a Middle Aged Male

Severe Aortic Stenosis Associated with Unicommissural Unicuspid Aortic Valve in a Middle Aged Male

The 10 Commandments for Aortic Valve Repair

Congenital aortic stenosis due to unicuspid unicommissural aortic valve: a case report

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