Quadricuspid Aortic Valve, Single Coronary Artery, Solitary Kidney and Oblique Facial Cleft. A Unique Constellation of Congenital Abnormalities: Case Report and Review of the Literature

Mehisen, Rabah Al; Essely, Ramy El

doi:10.4172/2157-7412.1000291

Cited by 4 publications

(4 citation statements)

References 35 publications

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“…Rare craniofacial clefts may be associated with local (head/neck) and systemic anomalies, including anophthalmia, choanal atresia, absence of the lacrimal system, maxillary hypoplasia, polydontia, and cardiovascular (such as quadricuspid aortic valve and single coronary artery) and renal defects (e.g. solitary kidney) [12,16]. Ocular abnormalities were the most common anomalies in the present study, and this was similar to previous reports [8,12].…”

Section: Discussionsupporting

confidence: 91%

Rare primary craniofacial clefts: pattern, challenges, and management in a Nigerian population

Agbara¹,

Akintububo²,

Fomete³

et al. 2020

jos

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Introduction: There is inconsistent clinical presentation of rare orofacial clefts, and its low number has resulted in most studies on rare craniofacial clefts to be retrospective in nature, with significant amount published as case reports. Objectives: To study the pattern, challenges, and management of rare craniofacial clefts in a Nigerian population. Material and methods: This is a retrospective observational study of patients with rare facial clefts, who visited private health institutions between January 2009 and August 2018. Data retrieved was analyzed using statistical package for social sciences (SPSS), version 16 (SPSS Inc., Chicago, IL, USA). Results: Of the 899 patients who presented with orofacial clefts within the reviewed period, a total of 20 patients had rare facial clefts, with typical to rare craniofacial cleft ratio of 46.3 : 1. The prevalence rate of rare craniofacial cleft was 22.2 per 1,000 cases. Age of patients ranged from 0.04 to 37.00 years, with median age of 3.50 ± 36.96 years. There were 10 males and 10 females, with male to female ratio of 1 : 1. Tessier number 0 had the highest frequency (n = 9, 36.0%), followed by Tessier number 7 (n = 3, 12.0%) and 2 (n = 3, 12.0%). Repair was achieved using local tissue advancement and direct closure techniques under general anesthesia in 10 patients and local anesthesia in 6 patients. Coloboma of the eyelid was the most common abnormality noted. Conclusions: The prevalence of rare craniofacial clefts is low in this environment and Tessier 0 cleft is the commonest type seen. Treatment is generally challenging due to inadequate manpower.

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Section: Discussionsupporting

confidence: 91%

Rare primary craniofacial clefts: pattern, challenges, and management in a Nigerian population

Agbara¹,

Akintububo²,

Fomete³

et al. 2020

jos

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“…Por lo general, se encuentra accidentalmente en la autopsia o incidentalmente en la cirugía por valvulopatía aórtica. Aparece como una malformación asintomática aislada sin evidencia de alteraciones hemodinámicas u otro defecto cardiaco asociado, pero también se ha informado que coexiste con otras malformaciones como anomalías coronarias (únicamente encontramos 3 casos reportados en la literatura donde coexisten arteria coronaria única y válvula aórtica cuadricúspide), estenosis de la válvula pulmonar, defecto septal ventricular, miocardiopatía no obstructiva y dilatación aneurismática de la aorta ascendente 7,8 .…”

Section: Discussionunclassified

Arteria coronaria única con origen en seno de Valsalva derecho y válvula aórtica cuadricúspide en un paciente con infarto agudo de miocardio.

López

Medina

Rojas

et al. 2022

Rev Ecocar Pract (RETIC)

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La arteria coronaria única es una anomalía congénita en la que el árbol coronario nace de un solo ostium, pudiendo asociarse a otras malformaciones cardíacas. Puede ser un hallazgo incidental, sin embargo, en pacientes jóvenes, o en casos de cursar con un trayecto interarterial, puede ser causa de angina, infarto agudo del miocardio e incluso muerte súbita, siendo de vital importancia su oportuna identificación. Se presenta el caso de un paciente el cual presentó dolor precordial, diagnosticando infarto agudo del miocardio sin elevación del ST, identificándose en angiografía y angiotoTC coronaria esta inusual anomalía.

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“…Bicuspid (approximately 2% of the population) aortic valves are the most prevalent aortic anomaly [ 12 ], followed by unicuspid (0.02% [ 13 ]) then QAVs [ 14 ], although the borderline scarcity between unicuspid and QAVs generate conflicting data to said rates of occurrences [ 15 ]. There have only been eight reported cases of quinticuspid, also referred to as pentacuspid aortic valves as of 2016 [ 16 ]. Quadricuspid pulmonary valves are nine times more prevalent than QAVs, with a comparable minor male predominance [ 17 ].…”

Section: Incidencesmentioning

confidence: 99%

Quadricuspid Aortic Valve: An Introduction for Clinicians

Saith

Murthy

2022

Cardiol Res

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Quadricuspid aortic valve (QAV) is a congenital heart anomaly in which the aortic valve has four cusps of various size possibilities, as opposed to the three symmetrical cusps generally observed. This cardiac valvular abnormality is rarely identified, with an estimated incidence rate of 0.013% to 0.043%, although recent technological advancements in diagnostics have contributed to an increase in detection. Historically, it had been typically encountered during open heart surgery or postmortem; however, it is presently diagnosed primarily via ultrasound echocardiography, and could go undetected unless specifically considered. It was first reported by Babington in 1847, and since then approximately 300 cases have been published. This condition is sporadically associated with additional congenital cardiovascular defects, with coronary artery irregularities being the most common. In more than half of published QAV incidences it has led to the progressive development of aortic regurgitation (AR) usually sans aortic stenosis, particularly amongst elderly patients, often requiring surgical intervention after 50 years of age. A fifth of total instances, but two-thirds of instances with AR, warrant surgery seldom amidst complications, with reconstructive tricuspidization preferred over valve replacement.

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Quadricuspid Aortic Valve, Single Coronary Artery, Solitary Kidney and Oblique Facial Cleft. A Unique Constellation of Congenital Abnormalities: Case Report and Review of the Literature

Cited by 4 publications

References 35 publications

Rare primary craniofacial clefts: pattern, challenges, and management in a Nigerian population

Rare primary craniofacial clefts: pattern, challenges, and management in a Nigerian population

Arteria coronaria única con origen en seno de Valsalva derecho y válvula aórtica cuadricúspide en un paciente con infarto agudo de miocardio.

Quadricuspid Aortic Valve: An Introduction for Clinicians

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