An Exceedingly Rare Case of Concomitant Quadricuspid Aortic Valve and Atrial Myxoma

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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Rare case of quadricuspid aortic valve

Acta chirurgica iugoslavica ◽

10.2298/aci0902097m ◽

2009 ◽

Vol 56 (2) ◽

pp. 97-99

Author(s):

S. Micovic ◽

B.P. Djukanovic ◽

P.S. Milojevic ◽

D. Nezic ◽

M. Jovic ◽

...

Keyword(s):

Congenital Anomaly ◽

Aortic Valve ◽

Aortic Valve Replacement ◽

Valve Replacement ◽

Rare Case ◽

Pulmonary Valve ◽

Quadricuspid Aortic Valve ◽

Valve Regurgitation ◽

Unequal Distribution ◽

Rare Congenital Anomaly

Quadricuspid aortic valve is rare congenital anomaly. There are only 197 cases published in literature so far. That includes clinical and autopsy reports. This congenital anomaly occurs more often in pulmonary valve but function stays normal in 10 of 11es. On the other side, in aortic position valve is malfunctioning in 50% of cases. Valve regurgitation is more likely to occur than stenosis In this kind of malformation, valves are prone to early dysfunction and endocarditis due to different valve architecture and unequal distribution of mechanical stress along valve cusps. Aortic valve replacement is indicated in younger population of these patients. This is a case report of rare congenital anomaly of big blood vessels - aortic valve with four cusps. This anomaly produced significant aortic regurgitation so this patient was indicated for aortic valve replacement.

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A rare case of infective endocarditis in a quadricuspid aortic valve caused by Gemella sanguinis

Discussion of Clinical Cases ◽

10.5430/dcc.v7n3p17 ◽

2021 ◽

Vol 7 (3) ◽

pp. 17

Author(s):

Hyun Ji ◽

Shravya Vinnakota ◽

Walter R. Wilson ◽

Vuyisile T. Nkomo

Keyword(s):

Infective Endocarditis ◽

Aortic Valve ◽

Aortic Valve Replacement ◽

Valve Replacement ◽

Rare Case ◽

English Literature ◽

Quadricuspid Aortic Valve ◽

Rare Occurrence ◽

Transesophageal Echocardiogram ◽

Intravenous Antibiotics

Infective endocarditis (IE) in patients with a quadricusp aortic valve (QAV) is a rare occurrence that has only been described nine times in English literature. Similar in rarity, IE by Gemella sanguinis has only been described ten times. It has never been described in a patient with QAV. We describe a case of Gemella sanguinis infective endocarditis of a quadricusp aortic valve in a patient in his 4th decade of life. His QAV was detected on pre-operative transesophageal echocardiogram and confirmed intra-operatively. The patient was managed with bioprosthetic aortic valve replacement and a 6-week course of intravenous antibiotics.

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A rare case of quadricuspid aortic valve with moderate aortic regurgitation with left ventricular non compaction presenting with ventricular tachycardia

IHJ Cardiovascular Case Reports (CVCR) ◽

10.1016/j.ihjccr.2018.03.006 ◽

2018 ◽

Vol 2 (2) ◽

pp. 127-130

Author(s):

Arnab Ghosh Chaudhury ◽

B. Prabhavathi ◽

C.N. Manjunath

Keyword(s):

Ventricular Tachycardia ◽

Aortic Valve ◽

Aortic Regurgitation ◽

Rare Case ◽

Left Ventricular ◽

Quadricuspid Aortic Valve

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WCC 2017-C-109: Congenital Quadricuspid Aortic Valve Associated with Severe Aortic Regurgitation: A Rare Case Report

Indian Journal of Cardiovascular Disease in Women WINCARS ◽

10.1055/s-0038-1656445 ◽

2017 ◽

Vol 02 (S 01) ◽

pp. S13-S15

Author(s):

Pavaneel Bhandary ◽

Palanki Satyagopal ◽

Muppiri Kumar ◽

Ravinuthala Kumar

Keyword(s):

Case Report ◽

Aortic Valve ◽

Aortic Regurgitation ◽

Male Patient ◽

Rare Case ◽

Aortic Insufficiency ◽

Quadricuspid Aortic Valve ◽

Severe Aortic Regurgitation ◽

Rare Case Report

AbstractQuadricuspid aortic valve is a very rare congenital valvular anomaly. Most of these cases present with aortic insufficiency. We present a 38 year old male patient with aquadricuspid aortic valve with severe aortic regurgitation.

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A rare case of pseudo-quadricuspid aortic valve repair

JTCVS Techniques ◽

10.1016/j.xjtc.2021.10.030 ◽

2021 ◽

Author(s):

Maureen Klepper ◽

Jama Jahanyar ◽

Gaby Aphram ◽

Laurent de Kerchove ◽

Gebrine el Khoury

Keyword(s):

Aortic Valve ◽

Rare Case ◽

Aortic Valve Repair ◽

Quadricuspid Aortic Valve ◽

Valve Repair

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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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Aortic Aneurysm and Dissection in Pregnancy: Report of a Rare Case in a Marfan with Bicuspid Aortic Valve

Indian Journal of Cardiovascular Disease in Women WINCARS ◽

10.1055/s-0040-1722793 ◽

2020 ◽

Vol 5 (04) ◽

pp. 331-334

Author(s):

Paolo Masiello ◽

Rocco Leone ◽

Rossella Maria Benvenga ◽

Severino Iesu

Keyword(s):

Aortic Valve ◽

Aortic Dissection ◽

Aortic Arch ◽

Bicuspid Aortic Valve ◽

Rare Case ◽

Unplanned Pregnancy ◽

Type A ◽

Cardiac Biomarkers ◽

High Morbidity ◽

Type A Aortic Dissection

Abstract Background Type A aortic dissection is an emergency with high morbidity and mortality when surgery is not performed. Few cases are described in the literature about aortic dissection during pregnancy. A correlation between pregnancy and aortic dissection is mainly reported in patients with family history and connective tissue disorders, such as Marfan’s syndrome (MS), Loeys–Dietz’s syndrome, and Ehlers–Danlos’s syndromes, and patients with bicuspid aortic valve (BAV); exceptional cases are also described in patients without risk factors. Case presentation A 22-year-old young woman with MS, ascending aorta dilation, and BAV became pregnant. During labor, she experienced a short-term chest pain with spontaneous resolution. The electrocardiogram (ECG) and cardiac biomarkers were negative for acute coronary artery disease, but no transthoracic echocardiogram (TTE) was performed. A caesarean section was performed without complications. After 1 month, a routine TTE showed a chronic ascending aortic dissection involving the aortic arch and supra-aortic vessels. Due to a normally functioning aortic valve, the David operation was performed (sparing aortic valve) with the replacement of the aortic arch and supra-aortic vessels. Conclusions Aortic dissection is a rare cardiovascular complication that can occur during pregnancy and is associated with very high-risk mortality. We have reported a rare case of undiagnosed type A aortic dissection involving the aortic arch during unplanned pregnancy in patients with BAV and MS, subsequently treated with the David surgery and replacement of ascending aortic arch and supra-aortic vessels. A closer clinical and instrumental follow-up is necessary in this particular group of patients at risk. Awareness of all physicians involved is mandatory.

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Quadricuspid Aortic Valve Repair Facilitated by Geometric Ring Annuloplasty

Innovations Technology and Techniques in Cardiothoracic and Vascular Surgery ◽

10.1177/15569845211003095 ◽

2021 ◽

pp. 155698452110030

Author(s):

Joseph A. McGuire ◽

Heather K. Hayanga ◽

Jeremiah W. Hayanga ◽

Daniel Sloyer ◽

Matthew Ellison ◽

...

Keyword(s):

Aortic Valve ◽

Patient Outcomes ◽

Systematic Approach ◽

Aortic Insufficiency ◽

Aortic Valve Repair ◽

Autologous Reconstruction ◽

Quadricuspid Aortic Valve ◽

Valve Repair ◽

Ring Annuloplasty ◽

Annular Geometry

Quadricuspid aortic valve (QAV) is a rare congenital anomaly often associated with aortic insufficiency. The exact anatomy of QAV is variable, and most cases have undergone aortic valve replacement. With the recognition that aortic valve repair achieves superior patient outcomes as compared to replacement, a systematic approach to autologous reconstruction of QAV is needed. This article reports 2 cases having successful repair utilizing geometric aortic annuloplasty rings, and describes a proposed scheme for repairing most QAV defects, based on relative leaflet and commissural characteristics. Using either tri-leaflet or bicuspid ring annuloplasty, the normal sub-commissural triangles can be remodeled into a 120° or 180° configuration, respectively, and then the leaflets can be sutured and plicated to fit annular geometry. With this approach, most quadricuspid valves potentially could undergo autologous reconstruction.

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