• Users Online: 167
  • Home
  • Print this page
  • Email this page
Home About us Editorial board Ahead of print Current issue Search Archives Submit article Instructions Subscribe Contacts Login 


 
 Table of Contents  
CASE REPORT
Year : 2016  |  Volume : 2  |  Issue : 1  |  Page : 61-62

Aortic insufficiency in a patient with a quadricuspid aortic valve and abnormal left coronary ostium


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 Publication26-May-2016

Correspondence Address:
Anish Gupta
98, Om Vihar, Phase-1A, Shiv Shankar Road, Uttam Nagar, New. Delhi - 110 059
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2395-5414.183000

Rights and Permissions
  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.

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 Top


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 Top


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
Figure 2: Excised quadricuspid aortic valve.

Click here to view
Figure 3: Pictorial representation of coronary anatomy in the patient.

Click here to view



  Discussion Top


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.[1] The prevalence of quadricuspid aortic valve according to historical autopsy reports is around 0.008% in a report by Simonds.[2] 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,[3] hypertrophic obstructive cardiomyopathy [4] and aortic aneurysm.[5] One of such anomalies associated with a quadricuspid aortic valve is an abnormally located coronary ostia [5] and there is a case report of obstruction of low-lying coronary ostia by the prosthetic aortic valve.[6] 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 [7] 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.,[8] 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 Top


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.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
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.  Back to cited text no. 1
[PUBMED]    
2.
Simonds JP. Congenital malformation of the aortic and pulmonary valves. Am J Med Sci 1923;166:584-95.  Back to cited text no. 2
    
3.
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.  Back to cited text no. 3
    
4.
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.  Back to cited text no. 4
    
5.
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.  Back to cited text no. 5
    
6.
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.  Back to cited text no. 6
[PUBMED]    
7.
Hurwitz LE, Roberts WC. Quadricuspid semilunar valve. Am J Cardiol 1973;31:623-6.  Back to cited text no. 7
[PUBMED]    
8.
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.  Back to cited text no. 8
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]


This article has been cited by
1 Quadricuspid aortic valve with a hidden left ostium: Case report and literature review
Filipe R. Soares,Carlos Branco,Gonçalo F. Coutinho,David Prieto,Manuel J. Antunes
Revista Portuguesa de Cardiologia (English Edition). 2021; 40(1): 63.e1
[Pubmed] | [DOI]
2 Quadricuspid aortic valve with a hidden left ostium: Case report and literature review
Filipe R. Soares,Carlos Branco,Gonçalo F. Coutinho,David Prieto,Manuel J. Antunes
Revista Portuguesa de Cardiologia. 2020;
[Pubmed] | [DOI]



 

Top
 
 
  Search
 
Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
Access Statistics
Email Alert *
Add to My List *
* Registration required (free)

 
  In this article
   Abstract
  Introduction
  Case Report
  Discussion
  Conclusion
   References
   Article Figures

 Article Access Statistics
    Viewed2221    
    Printed49    
    Emailed0    
    PDF Downloaded171    
    Comments [Add]    
    Cited by others 2    

Recommend this journal


[TAG2]
[TAG3]
[TAG4]