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Year : 2019  |  Volume : 3  |  Issue : 3  |  Page : 183-184

Rare Case of Unicuspid Unicommissural Aortic Valve Presenting in an Adult as Infective Endocarditis, Aortic Root Abscess, and Complete Heart Block

Department of Cardiology, Felix Healthcare, Noida, Uttar Pradesh, India

Date of Submission30-Jan-2019
Date of Decision05-Feb-2019
Date of Acceptance19-Feb-2019
Date of Web Publication18-Dec-2019

Correspondence Address:
Rahul Subhashrao Chalwade
A 301, Springs, Roadpali, Kalamboli, Sector 20, Navi Mumbai - 410 218, Maharashtra
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jiae.jiae_4_19

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Unicuspid aortic valve is a very rare congenital anomaly of aortic valve. Acommissural form usually presents at birth and unicommissural form presents in adulthood. They usually present as dyspnea, angina, and/or syncope. Mostly, it is detected on autopsy or pathological examination of surgically excised valve. Here, we present a rare case of unicuspid unicommissural aortic valve presenting with infective endocarditis with aortic root abscess and complete heart block. The patient was diagnosed on transthoracic echocardiography. Unfortunately, the patient succumbed to illness before surgery.

Keywords: Aortic root abscess, aortic valve anomalies, complete heart block, infective endocarditis complication, unicuspid aortic valve

How to cite this article:
Chalwade RS. Rare Case of Unicuspid Unicommissural Aortic Valve Presenting in an Adult as Infective Endocarditis, Aortic Root Abscess, and Complete Heart Block. J Indian Acad Echocardiogr Cardiovasc Imaging 2019;3:183-4

How to cite this URL:
Chalwade RS. Rare Case of Unicuspid Unicommissural Aortic Valve Presenting in an Adult as Infective Endocarditis, Aortic Root Abscess, and Complete Heart Block. J Indian Acad Echocardiogr Cardiovasc Imaging [serial online] 2019 [cited 2020 Oct 21];3:183-4. Available from: https://www.jiaecho.org/text.asp?2019/3/3/183/273296

  Introduction Top

Unicuspid aortic valve (UAV) is a very rare congenital anomaly of aortic valve. The reported prevalence of UAV is around 0.02%. Its clinical profile is variable.[1] It usually presents in infancy or childhood as severe aortic stenosis and/or aortic regurgitation. It sometimes presents in adulthood with aortic stenosis and/or aortic regurgitation, usually in unicommissural type.[2],[3] These patients are predisposed to valvular and aortic root complications. Here, we report a rare case of UAV with infective endocarditis (IE) complicated by aortic root abscess, complete heart block (CHB), and septic multiorgan failure.

  Clinical Presentation Top

A 53-year-old female patient presented to our emergency department with a history of dyspnea on exertion for 4 to 5 years, fever for 4 months, and syncopal episodes and increased breathlessness for 2 days. The patient was previously treated at a local hospital in a rural area. In the emergency department, the patient was febrile, tachypneic, dyspneic, and drowsy. Her heart rate was 32 beats/min and blood pressure was 80/30 mmHg. The patient clinically had bilateral crepitation and ejection systolic murmur in the aortic area. Electrocardiogram was suggestive of CHB. On blood investigation, the patient was found to be in multiorgan dysfunction. Her leukocyte count was raised, and there was marked renal and liver dysfunction. X-ray was suggestive of bilateral pulmonary edema. On two-dimensional (2D) echocardiography (echo), there was unicuspid unicommissural aortic valve with aortic root abscess. The patient underwent emergency temporary pacemaker implantation (TPI) and was advised emergency surgical intervention for valvular pathology, but she succumbed to the illness before any intervention.

2D echo of the patient was suggestive of severe left ventricular hypertrophy and all four chambers dilated, and there was unicuspid unicommissural aortic valve with commissure at 4 o'clock position [[Figure 1] and Videos 1-3]. The aortic valve was thickened with severe aortic stenosis and severe aortic regurgitation along with aortic root dilatation. There was vegetation over the aortic valve of size 1.4 cm × 1.2 cm with extension as aortic root abscess of size 1.9 cm × 1.2 cm on 9–12 o'clock position [[Figure 2] and Video 4]. The tricuspid valve showed moderate regurgitation with mild pulmonary hypertension. TPI lead was in situ.
Figure 1: Picture on the left showing unicuspid aortic valve in closed position with unicommisural attachment to annulus at 4'o clock position. Picture on the right showing valve in open position with circular narrowed valve opening with eccentric location, with partial attachment raphe seen

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Figure 2: Picture on the left showing vegetation over leaflet and picture on the right showing root abscess extending to interventricular septum and compressing

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  Discussion Top

Developmental abnormalities of aortic valve cusps are seen commonly. Most commonly seen is bicuspid (0.9%–1.3%) followed by rare abnormalities as unicuspid (0.02%), quadricuspid (0.008%–0.043%), and pentacuspid aortic valves. UAV is rare and can be confused with bicuspid valve. It was first reported by Edwards in 1958.[4] UAV is more common in males as compared to females with a gender ratio of 4:1 (M:F).[5]

There are two types of UAV described.[2] Acommisural and unicommisural forms depending on the attachment to the aortic root. Acommisural form has no root attachment and only small central opening. Unicommisural form has one lateral attachment to root with eccentric slit-like opening. Unicommisural form has larger effective orifice area.

Clinical presentation usually differs depending on type. Acommisural form usually presents in infancy only with left ventricular failure secondary to aortic stenosis. Unicommisural form is usually asymptomatic in childhood which can present clinically in third to fifth decades. The most common symptoms are dyspnea, angina, and syncope. Valve usually manifests as isolated aortic stenosis followed by mixed aortic stenosis/regurgitation and isolated aortic regurgitation.

UAV is associated with many complications such as aortic aneurysm, aortic dissection, heart failure, conduction abnormalities, and IE. IE rarely may get complicated by root abscess. This root abscess is located at aortic valve and sometimes extends to interventricular septum interfering with the conduction system, which leads to conduction abnormalities as CHB.

Prognosis of patients with IE complicated by abscess and sepsis with multiorgan dysfunction is very poor. In such patients, ideal management would be emergency aortic valve + root replacement, long-term antibiotics, and hope for the best. Even with such aggressive management, still outcome will be poor in such complicated patients.

Our reported case is unusual in many aspects as the patient was female in her fifth decade. It is less common in females that too presenting in adulthood. These patients are prone to many complications related to valve and aorta, but acute presentation like our patient as IE complicated by aortic root abscess leading to CHB is not reported before.

  Conclusion Top

Our unusual case suggests that, even though rare, UAV can present in adulthood and if symptoms neglected (as in remote areas), patients may land up in rare complications as our patient. Sometimes, rare anomalies (UAV) may get rare sequele (IE) which may progress to rare complication (root abscess with CHB)!

Declaration of patient consent

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

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Conflicts of interest

There are no conflicts of interest.

  References Top

Novaro GM, Mishra M, Griffin BP. Incidence and echocardiographic features of congenital unicuspid aortic valve in an adult population. J Heart Valve Dis 2003;12:674-8.  Back to cited text no. 1
Anderson RH. Understanding the structure of the unicuspid and unicommissural aortic valve. J Heart Valve Dis 2003;12:670-3.  Back to cited text no. 2
Roberts WC, Ko JM. Frequency by decades of unicuspid, bicuspid, and tricuspid aortic valves in adults having isolated aortic valve replacement for aortic stenosis, with or without associated aortic regurgitation. Circulation 2005;111:920-5.  Back to cited text no. 3
Edwards JE. Pathologic aspects of cardiac valvular insufficiencies. AMA Arch Surg 1958;77:634-49.  Back to cited text no. 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. 5


  [Figure 1], [Figure 2]


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