Focus on a rare clinical entity: unicuspid aortic valve disease

Naito, Shokichi; Sequeira-Gross, Tatiana; Petersen, Johannes; Holst, Theresa; Reichenspurner, Hermann; Girdauskas, Evaldas

doi:10.1080/14779072.2020.1811685

Cited by 12 publications

(12 citation statements)

References 39 publications

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“…Because of the earlier presentation and increased likelihood of more severe stenosis, unicuspid aortic valves manifest with an increased incidence of ascending aorta dilatation, 7 and aortic complications than the bicuspid and quadricuspid aortic valves. 2,25 In accordance with the literature, aortic dilatation was present in the patient with the unicuspid aortic valve, while aortic measurements were normal in the patient with the quadricuspid aortic valve.…”

Section: Discussionsupporting

confidence: 85%

“…Adopting the current recommendations of the bicuspid aortic valve guideline for E. Aslan these patients would be appropriate in the close management and monitoring of both aortic valve and aortic complications. 25 While evaluating these patients, it is necessary to clearly demonstrate the coronary arteries, especially for patients who will undergo surgery, as well as other possible intracardiac pathologies.…”

Section: Discussionmentioning

confidence: 99%

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Unicuspid and quadricuspid aortic valves: two case reports and literature review

Aslan

2021

Cardiol Young

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There are three cusps in a normally developed aortic valve. Abnormal excavation or fusion, during the embryological development of the aortic valve, results in a varying number of cusps. Bicuspid aortic valve is the most common, but more rarely, unicuspid and quadricuspid aortic valves can be seen. Here, a case of a 16-year-old male with a unicommissural unicuspid aortic valve and a case of a 13-year-old female with a quadricuspid aortic valve were reported.

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Section: Discussionsupporting

confidence: 85%

Section: Discussionmentioning

confidence: 99%

Unicuspid and quadricuspid aortic valves: two case reports and literature review

Aslan

2021

Cardiol Young

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“…In addition, the prevalence, age at presentation and prognosis of unicuspid and BAV, are not equivalent ( 13 , 14 ). Furthermore, the anatomic definition of unicuspid aortic valve (one cusp with or without a commissure: unicommissural or acommissural) ( 15 , 16 ) is very different from that of BAV (two cusps, two commissures); therefore, the Sievers classification includes only one (unicommissural) of the two types of unicuspid aortic valves as if it were a subtype of BAV, which is not correct.…”

Section: Limitations Of the Sievers Classificationmentioning

confidence: 99%

Speaking a common language: the international consensus on bicuspid aortic valve nomenclature and classification

Michelena¹

2022

Ann Cardiothorac Surg

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The congenital bicuspid aortic valve (BAV) condition is a valvulo-aortopathy with heterogeneous phenotypic expressions and clinical outcomes. A multitude of pre-existing classification systems, some extensive and some succinct, utilize combinations of numbers and/or letters to describe the condition. These diverse nomenclature systems are used according to the random preference of clinicians and researchers, generating confusion in clinical practice and research alike, effectively creating a barrier of communication at multiple levels. Based on imaging, pathology, surgery and clinical history evidence-based principles, and in utilizing the English language, the international nomenclature and classification consensus on the congenital BAV and its aortopathy offers a simple nosologic and phenotypic format that covers all possible phenotypes and clinical presentations of the bicuspid valvulo-aortopathy. From the nosology perspective, the valvuloaortopathy has three major clinical presentations: (I) typical valvulo-aortopathy; (II) complex valvuloaortopathy; and (III) undiagnosed or uncomplicated valvulo-aortopathy. From the valvular perspective, the congenital BAV has three major phenotypic expressions: (I) the fused BAV; (II) the 2-sinus BAV; and (III) the partial fusion (forme fruste) BAV. From the aortopathy perspective, the condition has three major phenotypic expressions: (I) ascending phenotype; (II) root phenotype; and (III) extended phenotypes. The international consensus is intended for universal use by Clinicians (pediatric and adult), echocardiography sonographers and physicians, cardiovascular advanced-imaging specialists, interventional cardiologists, cardiovascular surgeons, pathologists, geneticists and researchers encompassing clinical and basic research areas. When new landmark research is available, this international consensus may be subject to change in accordance with evidence-based data.

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“…21 In addition, the prevalence, age at presentation and prognosis of unicuspid and BAV are not equivalent. 22,23 Furthermore, the definition of unicuspid aortic valve (1 cusp with or without a commissure: unicommissural or acommissural) 24,25 is very different from that of BAV (2 cusps, 2 commissures); therefore, the Sievers classification includes only 1 (unicommissural) of the 2 types of unicuspid aortic valves as if it were a subtype of BAV, which is confusing. The use of one or another of the many classifications for research varies according to authors and institutions, and there are specific terminologies that lead to confusion such as the "true" BAV 2 ; does it mean that the others are false BAV?…”

Section: Why Have a Standard Nomenclature And Classification Consensu...mentioning

confidence: 99%

Summary: International Consensus Statement on Nomenclature and Classification of the Congenital Bicuspid Aortic Valve and Its Aortopathy, for Clinical, Surgical, Interventional and Research Purposes

Michelena

Corte

Evangelista

et al. 2021

The Annals of Thoracic Surgery

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Focus on a rare clinical entity: unicuspid aortic valve disease

Cited by 12 publications

References 39 publications

Unicuspid and quadricuspid aortic valves: two case reports and literature review

Unicuspid and quadricuspid aortic valves: two case reports and literature review

Speaking a common language: the international consensus on bicuspid aortic valve nomenclature and classification

Summary: International Consensus Statement on Nomenclature and Classification of the Congenital Bicuspid Aortic Valve and Its Aortopathy, for Clinical, Surgical, Interventional and Research Purposes

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