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 Table of Contents  
CASE REPORT
Year : 2020  |  Volume : 6  |  Issue : 3  |  Page : 296-298

Unicuspid Aortic Valve with Bilateral Superior Vena Cavae, Abnormal Coronary Anatomy, and Right Ventricular Clot: A Rare Case Report


1 Department of CTVS, All India Institute of Medical Sciences, Rishikesh, Uttarakhand, India
2 Department of CTVS, All India Institute of Medical Sciences, New Delhi, India

Date of Submission20-May-2020
Date of Decision22-Jul-2020
Date of Acceptance15-Sep-2020
Date of Web Publication23-Dec-2020

Correspondence Address:
Dr. Anish Gupta
98, Om Vihar, Phase-1A, Gali No 11, Uttam Nagar, New Delhi - 110 059
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jpcs.jpcs_53_20

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  Abstract 


A teenager presented with congestive heart failure due to severe aortic stenosis. She had also developed ventricular clots due to ventricular dysfunction, therefore, was taken up for emergency aortic valve replacement. To our surprise, she had bilateral superior vena cava, which has not been reported with unicuspid aortic valve and ventricular clots, to the best of our knowledge. She also had an abnormal location of coronary ostia making them susceptible to injury during routine aortotomy, but it was picked up during transesophageal echocardiography and a careful aortic incision was planned. This report emphasizes the importance of careful aortotomy in congenital aortic pathologies, where coronary ostia may be located in abnormal positions. The role of transesophageal echocardiography is paramount in this situation to find the location of coronary ostia before incising the aorta.

Keywords: Bilateral superior vena cava, clot, unicuspid aortic valve


How to cite this article:
Gupta A, Rajashekar P. Unicuspid Aortic Valve with Bilateral Superior Vena Cavae, Abnormal Coronary Anatomy, and Right Ventricular Clot: A Rare Case Report. J Pract Cardiovasc Sci 2020;6:296-8

How to cite this URL:
Gupta A, Rajashekar P. Unicuspid Aortic Valve with Bilateral Superior Vena Cavae, Abnormal Coronary Anatomy, and Right Ventricular Clot: A Rare Case Report. J Pract Cardiovasc Sci [serial online] 2020 [cited 2021 Apr 17];6:296-8. Available from: https://www.j-pcs.org/text.asp?2020/6/3/296/304526




  Introduction Top


The unicuspid aortic valve (UAV) is a rare congenital anomaly which can present with aortic valve stenosis or insufficiency either in the neonatal age group or later in adulthood. Its association with abnormally located coronary ostia and left superior vena cava has not been described in the literature to the best of our knowledge and has an important bearing on surgical planning during aortic valve replacement. This report will be helpful in careful surgical planning in such cases to prevent catastrophes and also emphasizes the role of transesophageal echocardiography in congenital anomalies.


  Case Report Top


A 13-year-old adolescent girl presented with progressively increasing dyspnea on exertion (New York Heart Association class 1–3), giddiness, and swelling of bilateral feet for 3 months. On examination, she had a heart rate of 110/min, regular rhythm, and blood pressure of 90/60 mm Hg. Although she was comfortable at rest had facial puffiness with pallor, ascites, and bilateral pitting pedal edema suggestive of congestive heart failure. On auscultation ejection, the systolic murmur was heard in the aortic area associated with thrill. Her total bilirubin was 4.3 mg/dl and was found to be having a hepatitis B infection. The chest radiograph showed cardiomegaly with bilateral mild pleural effusions. Ultrasonography of the abdomen revealed a nodular course liver with moderate ascites due to chronic liver disease. She was admitted for full workup and medical stabilization but developed tenofovir-induced tubular dysfunction during her hospital stay. On transthoracic echocardiography, cardiomegaly was present with global hypokinesia and moderate pericardial effusion giving cardiomyopathy like a picture. The aortic valve was looking unicuspid and was severely stenosed. Furthermore, she had biventricular dysfunction with right ventricular clot and mild mitral regurgitation [Figure 1]. There was no associated patent ductus arteriosus or coarctation and ascending aorta dimensions were normal for her age. She was planned for aortic valve replacement and clot removal. To our surprise, we found that the innominate vein was absent and left superior vena cava was present and this association with UAV has not been reported in the English medical literature previously, to the best of our knowledge. Her coronary ostia were also abnormally located as the right ostium was far leftward just adjacent to the left ostium [Figure 2]. Both the problems were accurately picked up during transoesophageal echocardiography which helped us in planning the cannulation and aortotomy appropriately. Routine cardiopulmonary bypass was instituted using 3-angled venous cannulae and 21 argyll aortic cannula and right superior pulmonary vein vent was inserted. All three vena cavae were looped and snugged. After putting aortic cross-clamp, root cardioplegia was given and oblique aortotomy was made carefully protecting the abnormally located ostia. The right ventricular clot was removed through the tricuspid valve, though it was stuck and hiding between trabeculations of the right ventricle. The left ventricle was also inspected transaortic and through interatrial septum, as doubt of clot was raised in trans thoracic imaging but it was free of any clot. The aortic valve was mildly calcified but thickened and fibrosed and a single leaflet with slit-like opening was present with a rudimentary raphe making it unicommissural type of UAV. The aortic valve was excised and replaced with 17 St Jude regent flexicuff mechanical heart valve prosthesis. The patient came off cardiopulmonary bypass well and discharged on the 5th postoperative day uneventfully.
Figure 1: Transoesophageal echo images showing left superior vena cava, the unicuspid aortic valve in the opened position, right ventricular clot. (Left to right).

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Figure 2: Intraoperative image of the left superior vena cava, coronary anatomy, excised and unicuspid aortic valve. (Left to right).

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  Discussion Top


The aortic valve develops from three embryonic tubercles which form a tricuspid semilunar valve but it can be abnormally formed into two, four, or sometimes single cusp if all three tubercles are fused. Very rarely, five or more cusps have been reported in the literature.[1] These abnormalities of the aortic valve are usually accompanied by some form of aortopathy or connective tissue disorders such as Marfan's syndrome. The bicuspid aortic valve is the most common congenital cardiovascular anomaly which is seen in 1%–2% of the population. UAV is slightly more common than the quadricuspid aortic valve, but it is still a rare malformation with an incidence of 0.02% in adults and has a male preponderance (male-to-female ratio 4:1). UAV was first reported by Edwards as early as 1958[2] and various associations with the aortic aneurysm, aortic dissection, patent ductus arteriosus, and coarctation of the aorta have been reported since then.[3],[4] However, to the best of our knowledge, it is the first case of UAV with left superior vena cava and right ventricular clot, although clot would have developed as a sequel to ventricular dysfunction. UAV has been classified into two types acommissural and unicommissural. Acommissural variety has no commissure and an only a central opening is present for a forward flow which can be circular or oval in shape and therefore, the patient is very symptomatic and presents early in infancy or childhood with congestive heart failure. On the other hand, unicommissural variety has a slit-like orifice reaching till annulus and symptoms are late in the form of progressing dyspnea or angina on exertion usually in the third decade of life.


  Conclusion Top


We want to re-emphasize the need for careful aortotomy during the surgery for congenital aortic pathologies where coronary ostia may be located in an abnormal position making it susceptible to injury. The role of transesophageal echocardiography is paramount in this situation to find the location of coronary ostia before incising the aorta. The presence of the left superior vena cava with UAV is a new finding which can affect perfusion planning during surgery.

Ethical clearance

As per institute policy, not required for case reports.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given her consent for her images and other clinical information to be reported in the journal. The patient understands that her name and initials will not be published and due efforts will be made to conceal her identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Kamata S, Sakagoshi N Ohata T, Sasako Y. Successful surgical treatment of pentacuspid aortic valve with severe aortic regurgitation. Jpn J Cardiovasc Surg 2008;37:53-5.  Back to cited text no. 1
    
2.
Edwards JE. Pathologic aspects of cardiac valvular insufficiencies. AMA Arch Surg 1958;77:634-49.  Back to cited text no. 2
    
3.
Kang SD, Seol SH, Park BM, Kim DK, Kim KH, Kim DI, et al. Incidental diagnosis of the unicuspid aortic valve with ascending aortic aneurysm in an asymptomatic adult. J Cardiovasc Ultrasound 2011;19:102-4.  Back to cited text no. 3
    
4.
Mookadam F, Thota VR, Garcia-Lopez AM, Emani UR, Alharthi MS, Zamorano J, et al. Unicuspid aortic valve in adults: A systematic review. J Heart Valve Dis 2010;19:79-85.  Back to cited text no. 4
    


    Figures

  [Figure 1], [Figure 2]



 

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