Unicuspid aortic valve: Case series and review

Suraci, Nicholas; Presti, Saberio Lo; D'Mello, Jayanand; Xydas, Steve; Mihos, Christos G.

doi:10.1111/echo.14885

Cited by 4 publications

(2 citation statements)

References 11 publications

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“…No caso exposto, realizou-se a cirurgia do tipo Bentall em que o aneurisma aórtico é substituído por um enxerto de Dacron, a válvula aórtica é substituída por uma válvula protética e as artérias coronárias são recolocadas no enxerto aórtico4. As diretrizes americanas não especificam o tratamento da estenose da valva aórtica unicúspide, no entanto, há um consenso de que as intervenções percutâneas são proibitivas (3).…”

Section: Relato De Casounclassified

“…Já o tipo unicomissural é mais comum, sendo observado em 4.5% dos adultos submetidos à troca valvar aórtica isolada (1). Normalmente, esses indivíduos permanecem assintomáticos até a quinta década (3). Esta anomalia valvar sugere ter base genética, com dados epidemiológicos relacionados com coarctação da aorta e estenose da valva aórtica (4).…”

Section: Introductionunclassified

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Achado raro de válvula unicúspide unicomissural em um paciente jovem

Gabriela Pimenta dos Santos,

Ferreira de Lima Duarte,

Akio Nishijuka

et al. 2024

J onscience

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A valva aórtica unicúspide (VAU) é uma doença rara em adultos, encontrada em 0,02% da população americana, e normalmente cursa de forma assintomática até a quinta década de vida. Esta anomalia valvar sugere ter base genética, com forte associação com coarctação da aorta, estenose e insuficiência da valva aórtica. O presente caso é sobre um paciente do sexo masculino, 28 anos, com relato de cardiopatia desde a infância sem acompanhamento. Iniciou quadro de dispneia aos moderados esforços aos 27 anos. Apresentou síndrome edemigênica, dor pleurítica, sendo internado com diagnóstico de tromboembolismo venoso. Ecocardiograma transtorácico (ETT) evidenciou VAU com estenose moderada e regurgitação grave. Angiotomografia da aorta revelou dilatação aneurismática. Foi submetido a cirurgia de Bentall. O diagnóstico pré-operatório de VAU é desafiador com o uso de ETT e frequentemente resulta em diagnósticos errôneos. Com relação ao tratamento, as diretrizes americanas não especificam o tratamento da estenose da VAU. O mais recomendado é o cirúrgico e, para isso, existem alguns procedimentos que devem ser analisados e escolhidos de acordo com a individualidade de cada paciente.

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Section: Relato De Casounclassified

Section: Introductionunclassified

Achado raro de válvula unicúspide unicomissural em um paciente jovem

Gabriela Pimenta dos Santos,

Ferreira de Lima Duarte,

Akio Nishijuka

et al. 2024

J onscience

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Coronary artery dilation associated with bicuspid and unicuspid aortic valve disease in children: a series of 17 patients

et al. 2023

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Introduction: Coronary artery dilation associated with bicuspid/unicuspid aortic valves is described in adults with limited data in children. We aimed to describe the clinical course of children with bicuspid/unicuspid aortic valves and coronary dilation including coronary Z-score changes over time, association of coronary changes with aortic valve anatomy/function, and complications. Materials and methods: Institutional databases were searched for children ≤18 years with both bicuspid/unicuspid aortic valves and coronary dilation (1/2006-6/2021). Kawasaki disease and isolated supra-/subvalvar aortic stenosis were excluded. Statistics were descriptive with associations measured by Fisher’s exact test and overlapping 83.7% confidence intervals. Results: Of 17 children, bicuspid/unicuspid aortic valve was diagnosed at birth in 14 (82%). Median age at coronary dilation diagnosis was 6.4 years (range: 0-17.0). Aortic stenosis was present in 14 (82%) [2 (14%) moderate, 8 (57%) severe]; 10 (59%) had aortic regurgitation; 8 (47%) had aortic dilation. The right coronary was dilated in 15 (88%), left main in 6 (35%), and left anterior descending in 1 (6%) with no relationship between leaflet fusion pattern or severity of aortic regurgitation/stenosis on coronary Z-score. Follow-up evaluations were available for 11 (mean 9.3 years, range 1.1–14.8) with coronary Z-scores increasing in 9/11 (82%). Aspirin was used in 10 (59%). There were no deaths or coronary artery thrombosis. Discussion: In children with bicuspid/unicuspid aortic valves and coronary dilation, the right coronary artery was most frequently involved. Coronary dilation was observed in early childhood and frequently progressed. Antiplatelet medication use was inconsistent, but no child died nor developed thrombosis.

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A rare congenital anomaly: unicuspid unicommissural aortic valve associated with stenosis and ascending aorta dilatation

Ku,

Lv,

2024

Acta Cardiologica

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Unicuspid aortic valve: Case series and review

Cited by 4 publications

References 11 publications

Achado raro de válvula unicúspide unicomissural em um paciente jovem

Achado raro de válvula unicúspide unicomissural em um paciente jovem

Coronary artery dilation associated with bicuspid and unicuspid aortic valve disease in children: a series of 17 patients

A rare congenital anomaly: unicuspid unicommissural aortic valve associated with stenosis and ascending aorta dilatation

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