|Year : 2016 | Volume
| Issue : 1 | Page : 61-62
Aortic insufficiency in a patient with a quadricuspid aortic valve and abnormal left coronary ostium
Anish Gupta1, Sandeep Chauhan2, Abhishek Anand1, Akshay Kumar Bisoi1
1 Department of Cardiothoracic Vascular Surgery, All Institute of Medical Sciences, New Delhi, India
2 Department of Cardiac Anaesthesia, All Institute of Medical Sciences, New Delhi, India
|Date of Web Publication||26-May-2016|
98, Om Vihar, Phase-1A, Shiv Shankar Road, Uttam Nagar, New. Delhi - 110 059
Source of Support: None, Conflict of Interest: None
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.
Keywords: Abnormal left coronary ostium, aortic insufficiency, quadricuspid aortic valve
|How to cite this article:|
Gupta A, Chauhan S, Anand A, Bisoi AK. Aortic insufficiency in a patient with a quadricuspid aortic valve and abnormal left coronary ostium. J Pract Cardiovasc Sci 2016;2:61-2
|How to cite this URL:|
Gupta A, Chauhan S, Anand A, Bisoi AK. Aortic insufficiency in a patient with a quadricuspid aortic valve and abnormal left coronary ostium. J Pract Cardiovasc Sci [serial online] 2016 [cited 2021 Oct 16];2:61-2. Available from: https://www.j-pcs.org/text.asp?2016/2/1/61/183000
| Introduction|| |
A quadricuspid aortic valve is a rare congenital anomaly which can lead to aortic insufficiency or aortic stenosis. If the patient 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.
| Case Report|| |
A 64-year-old female who had breathlessness and palpitations of progressing severity for about 5 years was diagnosed with symptomatic severe aortic regurgitation on transthoracic echocardiography which showed thickened tricuspid aortic valve with a 7 mm vena contracta of a central jet. She was found to have a degenerative aortic valve and was referred for aortic valve replacement. Intraoperative transesophageal echocardiography (TEE) surprisingly showed a quadricuspid aortic valve [Figure 1] with malcoapting leaflets and a central regurgitant jet. This was confirmed intraoperatively when a thickened degenerative quadricuspid aortic valve was found with four equal sized cusps [Figure 2]. There were also two left coronary ostia due to the early bifurcation of the left coronary artery, and both the left coronary ostia were located very close to the commissures [Figure 3]. The aortic valve was excised and successfully replaced with aortic bioprosthesis (EPIC St. Jude Minn., USA) taking great care to save the abnormally located coronary ostia. The patient recovered well in the postoperative period without any complications.
|Figure 1: Trans-esophageal echocardiography image showing quadricuspid aortic valve.|
Click here to view
| Discussion|| |
Quadricuspid aortic valve is a rare congenital anomaly with the first case report published in 1862 by Balington and more than 200 cases have been reported till date. The prevalence of quadricuspid aortic valve according to historical autopsy reports is around 0.008% in a report by Simonds. Quadricuspid aortic valves are mostly discovered incidentally during echocardiography, autopsy or during surgery but an accurate preoperative diagnosis is important because though a quadricuspid aortic valve occurs as an isolated anomaly, but various associated anomalies have been seen including atrial septal defect, pulmonary stenosis, ventricular septal defect, hypertrophic obstructive cardiomyopathy  and aortic aneurysm. One of such anomalies associated with a quadricuspid aortic valve is an abnormally located coronary ostia  and there is a case report of obstruction of low-lying coronary ostia by the prosthetic aortic valve. Surgeons should, therefore, be careful while implanting the aortic prosthetic valve in such a condition. TEE can be of great help in making an accurate intraoperative diagnosis, deciding aortotomy incision and for valve excision. Hurwitz and Roberts  have classified quadricuspid aortic valves into 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., 192 cases were reviewed till December 2004 and mean age of patients was 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
Conflicts of interest
There are no conflicts of interest.
| References|| |
Robicsek F, Sanger PW, Daugherty HK, Montgomery CC. Congenital quadricuspid aortic valve with displacement of the left coronary orifice. Coll Works Cardiopulm Dis 1968;14:87-90.
Simonds JP. Congenital malformation of the aortic and pulmonary valves. Am J Med Sci 1923;166:584-95.
Hojo H, Yokote Y, Kyo S. A case of aortic regurgitation due to quadricuspid aortic valve with ventricular septal defect and double chamber right ventricle. J Jpn Coll Surg 2003;28:874-8.
Janssens U, Klues HG, Hanrath P. Congenital quadricuspid aortic valve anomaly associated with hypertrophic non-obstructive cardiomyopathy: A case report and review of the literature. Heart 1997;78:83-7.
Naito K, Ohteki H, Yunoki J, Hisajima K, Sato H, Narita Y. Aortic valve repair for quadricuspid aortic valve associated with aortic regurgitation and ascending aortic aneurysm. J Thorac Cardiovasc Surg 2004;128:759-60.
Robicsek F, Sanger PW, Daugherty HK, Montgomery CC. Congenital quadricuspid aortic valve with displacement of the left coronary orifrice. Am J Cardiol 1969;23:288-90.
Hurwitz LE, Roberts WC. Quadricuspid semilunar valve. Am J Cardiol 1973;31:623-6.
Yamagishi Y, Yuda S, Tsuchihashi K, Saitoh S, Miura T, Ura N, et al
. Quadricuspid aortic valve associated with aortic stenosis and regurgitation: Report of a case and a review of the literature. J Med Ultrasonics 2007;34:197-200.
[Figure 1], [Figure 2], [Figure 3]