A rare pediatric cardiac anomaly: Quadricuspid aortic valve with aortic regurgitation

Congenital quadricuspid aortic valve (QAV) is a rare cardiac anomaly. Several different anatomical variations of a quadricuspid aortic valve have been described. Aortic regurgitation is the predominant valvular dysfunction associated with QAV and patients tend to present in their 5th or 6th decade of life. This anomaly is rarely picked up by transthoracic echocardiogram (TTE). A comprehensive transoesophageal echocardiography (TOE) study is more likely to diagnose it. We describe a very rare type of QAV – Type F in a 52-year-old lady who presented with symptoms of shortness of breath and pre-syncope. We include TOE images and intra-operative valve images.

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A case of quadricuspid aortic valve with severe aortic regurgitation diagnosed by transthoracic echocardiography

Choonpa Igaku ◽

10.3179/jjmu.38.461 ◽

2011 ◽

Vol 38 (4) ◽

pp. 461-464

Author(s):

Koutatsu NOMURA ◽

Yoshikazu YAZAKI ◽

Masako MIYASHITA ◽

Sachiko OOTSUKI ◽

Yutaka KUMAGAI ◽

...

Keyword(s):

Aortic Valve ◽

Aortic Regurgitation ◽

Transthoracic Echocardiography ◽

Quadricuspid Aortic Valve ◽

Severe Aortic Regurgitation

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Quadricuspid aortic valve associated with severe aortic regurgitation.

Japanese Circulation Journal ◽

10.1253/jcj.49.190 ◽

1985 ◽

Vol 49 (2) ◽

pp. 190-191 ◽

Cited By ~ 6

Author(s):

MASAHIKO MATSUMOTO ◽

SHIGEHIKO MIKI ◽

KENJI KUSUHARA ◽

YUICHI UEDA ◽

YUTAKA OHKITA ◽

...

Keyword(s):

Aortic Valve ◽

Aortic Regurgitation ◽

Quadricuspid Aortic Valve ◽

Severe Aortic Regurgitation

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Quadricuspid Aortic Valve with Moderate Aortic Regurgitation Demonstrated by 3-dimensional Transoesophageal Echocardiography

Turk Kardiyoloji Dernegi Arsivi-Archives of the Turkish Society of Cardiology ◽

10.5543/tkda.2016.04987 ◽

2016 ◽

Author(s):

Muhittin Demirel

Keyword(s):

Aortic Valve ◽

Aortic Regurgitation ◽

Transoesophageal Echocardiography ◽

Quadricuspid Aortic Valve ◽

3 Dimensional

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Common Complication in an Uncommon Disease: Presence of Aortic Regurgitation in Patient with Quadricuspid Aortic Valve

International Journal of Clinical Medicine ◽

10.4236/ijcm.2016.73024 ◽

2016 ◽

Vol 07 (03) ◽

pp. 231-235

Author(s):

Adikesava Naidu Otikunta ◽

Y. V. Subba Reddy ◽

Praneeth Polamuri ◽

Ravi Srinivas

Keyword(s):

Aortic Valve ◽

Aortic Regurgitation ◽

Quadricuspid Aortic Valve ◽

Common Complication ◽

Uncommon Disease

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Intraoperative diagnosis of a quadricuspid aortic valve

Asian Cardiovascular and Thoracic Annals ◽

10.1177/0218492317692467 ◽

2017 ◽

Vol 26 (9) ◽

pp. 704-706

Author(s):

Hassan Tatari ◽

Maziar Gholampour Dehaki ◽

Gholamreza Omrani ◽

Hafez Ghaheri ◽

Alwaleed Al-Dairy ◽

...

Keyword(s):

Aortic Valve ◽

Aortic Regurgitation ◽

Septal Defect ◽

Incidental Finding ◽

Aortic Valve Repair ◽

Quadricuspid Aortic Valve ◽

Valve Regurgitation ◽

Valve Repair ◽

Ventricular Septal Defect Closure

Quadricuspid aortic valve is a rare anomaly, and most patients require surgery for aortic regurgitation in the 5th or 6th decades of life; only a few cases of aortic valve repair in childhood have been reported. A 3-year-old boy was scheduled for ventricular septal defect closure and aortic valve repair. Quadricuspid aortic valve was an incidental finding at operation; it was repaired by joining the left anterior and right anterior cusps. At the 9-month follow-up, the patient had no more than mild aortic regurgitation. We emphasize the importance of detecting this anomaly, especially in children with aortic valve regurgitation.

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Quadricuspid aortic valve: a case report and review of literature

Future Cardiology ◽

10.2217/fca-2020-0135 ◽

2021 ◽

Author(s):

Usman Ghani Piracha ◽

Gurukripa N. Kowlgi ◽

Walter Paulsen ◽

Mohammad Khalid Mojadidi ◽

Nimesh Patel

Keyword(s):

Case Report ◽

Aortic Valve ◽

Aortic Valve Replacement ◽

Functional Status ◽

Aortic Regurgitation ◽

Valve Replacement ◽

Clinical Presentation ◽

Quadricuspid Aortic Valve ◽

Review Of Literature ◽

Cardiac Defect

Quadricuspid aortic valve, a rare congenital cardiac defect, manifests most commonly as aortic regurgitation. Clinical presentation mainly depends on the functional status of the aortic valve, myocardium and associated cardiovascular abnormalities. Aortic valve replacement or repair is usually warranted in the 5th or 6th decade.

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