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Aortic insufficiency in a patient with a quadricuspid aortic valve and abnormal

left coronary ostium

Article in Journal of the Practice of Cardiovascular Sciences · January 2016

DOI: 10.4103/2395-5414.183000

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4 authors, including:

Anish Gupta Abhishek Anand

All India Institute of Medical Sciences Rishikesh Guru Gobind Singh Indraprastha University
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Case Report

Aortic Insufficiency in a Patient with a Quadricuspid Aortic

Valve and Abnormal Left Coronary Ostium
Anish Gupta, Sandeep Chauhan1, Abhishek Anand, Akshay Kumar Bisoi
Departments of Cardiothoracic Vascular Surgery and 1Cardiac Anaesthesia, All India Institute of Medical Sciences, New Delhi, India

Abstract
A  64‑year‑old female had symptomatic severe aortic insufficiency and was taken up for aortic valve replacement. The patient was found to
have a quadricuspid aortic valve and abnormally located early bifurcated left coronary ostium which were very near to the commissures.
There is a risk of damaging the coronary ostia while excising the valve or the prosthetic valve can obstruct the abnormally located ostia.
Intraoperative transesophageal echocardiography can help in making an accurate intraoperative diagnosis and deciding aortotomy incision
and valve excision. This patient underwent successful aortic valve replacement taking great care to save the abnormally located left coronary
ostium.

Key words: Abnormal left coronary ostium, aortic insufficiency, quadricuspid aortic valve

Introduction and successfully replaced with aortic bioprosthesis (EPIC St.

Jude Minn., USA) taking great care to save the abnormally
A quadricuspid aortic valve is a rare congenital anomaly which
located coronary ostia. The patient recovered well in the
can lead to aortic insufficiency or aortic stenosis. If the patient
postoperative period without any complications.
needs aortic valve replacement, the rare association with the
abnormal location of coronary ostia should be kept in mind to
avoid prosthetic valve induced obstruction of coronary ostia. Discussion
Quadricuspid aortic valve is a rare congenital anomaly
Case Report with the first case report published in 1862 by Balington
and more than 200 cases have been reported till date.[1] The
A 64‑year‑old female who had breathlessness and palpitations
prevalence of quadricuspid aortic valve according to historical
of progressing severity for about 5 years was diagnosed with
autopsy reports is around 0.008% in a report by Simonds.[2]
symptomatic severe aortic regurgitation on transthoracic
Quadricuspid aortic valves are mostly discovered incidentally
echocardiography which showed thickened tricuspid aortic
during echocardiography, autopsy or during surgery but
valve with a 7 mm vena contracta of a central jet. She was
an accurate preoperative diagnosis is important because
found to have a degenerative aortic valve and was referred
though a quadricuspid aortic valve occurs as an isolated
for aortic valve replacement. Intraoperative transesophageal
anomaly, but various associated anomalies have been seen
echocardiography (TEE) surprisingly showed a quadricuspid
including atrial septal defect, pulmonary stenosis, ventricular
aortic valve [Figure 1] with malcoapting leaflets and a central
regurgitant jet. This was confirmed intraoperatively when a
Address for correspondence: Dr. Anish Gupta,
thickened degenerative quadricuspid aortic valve was found 98, Om Vihar, Phase‑1A, Shiv Shankar Road,
with four equal sized cusps [Figure 2]. There were also two left Uttam Nagar, New Delhi ‑ 110 059, India.
coronary ostia due to the early bifurcation of the left coronary E‑Mail: drash_06@yahoo.co.in
artery, and both the left coronary ostia were located very close
to the commissures [Figure 3]. The aortic valve was excised
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DOI: How to cite this article: Gupta A, Chauhan S, Anand A, Bisoi AK. Aortic
10.4103/2395-5414.183000 insufficiency in a patient with a quadricuspid aortic valve and abnormal left
coronary ostium. J Pract Cardiovasc Sci 2016;2:61-2.

© 2016 Journal of the Practice of Cardiovascular Sciences | Published by Wolters Kluwer - Medknow 61
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Gupta, et al.: Quadricuspid Valve

seven types out of which Type A and B are the most common.
Our patient had Type A quadricuspid valve according to this
classification.
Type A: Four equal cusps
Type B: Three equal cusps and one smaller cusp
Type C: Two equal larger cusps and two equal smaller
cusps
Type D: One large, two intermediate and one smaller cusp
Type E: Three equal cusps and one larger cusp
Type F: Two equal larger cusps and two inequal smaller
cusps
Type G: Four inequal cusps.
In a review article by Yamagishi et al.,[8] 192 cases were
Figure 1: Trans‑esophageal echocardiography image showing reviewed till December 2004 and mean age of patients was
quadricuspid aortic valve. found to be 51 years with a female preponderance. About
60% of patients had aortic regurgitation at the time of
presentation while aortic stenosis was seen in 8% of patients
and 7% of patients had a normal functioning valve.

Conclusion
A quadricuspid aortic valve is a rare congenital anomaly
which can lead to aortic valve problems. If the patient needs
aortic valve replacement, abnormal location of coronary ostia
should be kept in mind. In this patient, there there were two left
coronary ostia and they were close to the commissures. Surgery
with successful with implantation of a aortic bioprosthesis,
with no damage to the coronary ostia.
Financial support and sponsorship
Nil.
Figure 2: Excised quadricuspid aortic valve. Conflicts of interest
There are no conflicts of interest.

References
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quadricuspid aortic valve with displacement of the left coronary orifice.
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62 Journal of the Practice of Cardiovascular Sciences ¦ January-April 2016 ¦ Volume 2 ¦ Issue 1

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