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Year : 2021  |  Volume : 5  |  Issue : 1  |  Page : 40-42

An Interesting Case of Mitral-Aortic Intervalvular Fibrosa Pseudoaneurysm

Department of Cardiology, Arneja Heart Institute, Nagpur, Maharashtra, India

Date of Submission04-Jan-2020
Date of Decision28-Mar-2020
Date of Acceptance26-Jul-2020
Date of Web Publication26-Mar-2021

Correspondence Address:
Dr. Abhay Thakre
Arneja Heart Institute, Nagpur, Maharashtra
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jiae.jiae_3_20

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Pseudoaneurysm of the mitral-aortic intervalvular fibrosa (MAIF) usually occurs as a rare complication of aortic valve endocarditis; however, it may also be idiopathic in etiology. We report a 45-year-old male presenting with acute-onset progressive dyspnea of 2 weeks duration. There was no history of fever. Symptoms were secondary to MAIF aneurysm rupturing into the left ventricular outflow tract causing a large shunt which was diagnosed on echocardiography. The MAIF aneurysm that is idiopathic in etiology is a relatively rare entity as compared to that because of infective endocarditis.

Keywords: Infective endocarditis, mitral aortic intervalvular fibrosa, pseudoaneurysm

How to cite this article:
Thakre A, Arneja J S, Sharma A. An Interesting Case of Mitral-Aortic Intervalvular Fibrosa Pseudoaneurysm. J Indian Acad Echocardiogr Cardiovasc Imaging 2021;5:40-2

How to cite this URL:
Thakre A, Arneja J S, Sharma A. An Interesting Case of Mitral-Aortic Intervalvular Fibrosa Pseudoaneurysm. J Indian Acad Echocardiogr Cardiovasc Imaging [serial online] 2021 [cited 2021 Jul 23];5:40-2. Available from: https://www.jiaecho.org/text.asp?2021/5/1/40/312221

  Introduction Top

Pseudoaneurysm of the mitral-aortic intervalvular fibrosa (MAIF) is an uncommon condition, consisting of an expansile cavity in the mitral-aortic junction communicating with the left ventricular outflow tract (LVOT). When intervalvular fibrosa pseudoaneurysm communicates to both the left ventricle (LV) and left atrium (LA), eccentric jet flow from the anterior to posterior mitral annulus may occur during systole and mimic mitral regurgitation due to perforation in the anterior leaflet. The most common cause is aortic valve endocarditis, particularly in prosthetic valves. However, rarely, it can be seen without evidence of infective endocarditis.

  Case Report Top

A 45-year-old male presented to our the out-patient department (OPD) with a history of progressively dyspnea of 2 weeks duration associated with orthopnea for 2 days. On examination, he had New York Heart Association class IV dyspnea. He had no history of prolonged fever, cough, or chest pain. On physical examination, his blood pressure was 90/40 mmHg, heart rate was 122/min. Clinical examination showed ejection systolic murmur (grade-5/6) at the apex and LV third heart sound. There were bilateral extensive crepts on lung examination. Other systemic examination was within the normal limits. There was no clinical evidence of acute rheumatic activity or infective endocarditis. The chest X-ray showed mild cardiomegaly with the signs of pulmonary edema. Routine blood investigations were within normal range. The procalcitonin level and antistreptolysin O titer was normal. ECG showed sinus tachycardia.

The patient deteriorated in OPD and was immediately shifted to intensive cardiac care unit (ICCU). He was in respiratory distress and was intubated and put on ventilatory support. Bedside two-dimensional (2D) transthoracic echocardiogram (TTE) window was very poor, and nothing conclusive could be made out. However, significant pericardial effusion or severe LV dysfunction was ruled out.

Bedside transesophageal echocardiography (TEE) was hence done. There was a large expansile pseudoaneurym seen in MAIF [Video 1],[Video 2],[Video 3],[Video 4]. The aortic valve was densely calcified and severely stenosed. Color Doppler examination showed a turbulent flow from LVOT into the psedoaneurysm through a narrow neck [Video 2], [Video 3] and [Video 5]. There was moderate aortic regurgitation. The patient also had mild mitral valvular regurgitation. However, there was no communication between the pseudoaneurysm cavity and the LA. Real-time three-dimensional (3D) echocardiography was also done which showed the rent in the MAIF and the blood flow through it in the pulsatile pseudoaneurysm [Video 5],[Video 6],[Video 7],[Video 8]. Cardiac systolic functions were normal. The mitral and tricuspid valves were normal, and there were no vegetations over mitral or aortic valves and no annular abscess.

Video 1: Expansile pseudoaneurysm of mitral aortic intervalvular fibrosa.

Video 2: Mid-esophageal long-axis view showing color flow through rent in mitral aortic intervalvular fibrosa.

Video 3: Mid-esophageal five-chamber view showing color flow through rent in mitral aortic intervalvular fibrosa.

Video 4: Three-dimensional transesophageal echocardiography showing pulsatile pseudoaneurysm.

Video 5: Rent in the mitral aortic intervalvular fibrosa on three-dimensional transesophageal echocardiography.

Video 6: Rent in the mitral aortic intervalvular fibrosa along with the aneurysm flap.

Video 7: Color flow as visualized in three-dimensional echocardiography.

Video 8: Pseudoaneurysm flap visualized on three-dimensional transesophageal echocardiography.

The serial blood cultures were negative. The patient was treated in the ICCU, and after stabilization, he underwent aortic valve replacement (bileaflet metallic prosthetic valve) with the repair of pseudoaneurysm. The rent seen on 3D TEE could be visualized well intraoperatively [Figure 1]. Intraoperatively, the aortic valve was densely calcified, bicuspid, and was severely stenosed. Intraoperative transesophageal echocardiogram showed successful closure of MAIF shunt and pseudoaneurysm repair with normally functioning bileaflet metallic prosthetic valve in aortic position [Video 9] and [Video 10]. The patient had uneventful postoperative course and was discharged in the stable condition.
Figure 1: Intraoperative picture showing rent in mitral-aortic intervalvular fibrosa

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Video 9: Intraoperative transesophageal echocardiography with color Doppler after aortic valve replacement and pseudoaneurysm repair.

Video 10: Intraoperative transesophageal echocardiography with X-plane imaging after aortic valve replacement and pseudoaneurysm repair.

  Discussion Top

MAIF is the junction between the left half of the noncoronary cusp and the adjacent third of the left coronary cusp of the aortic valve and the anterior mitral leaflet. It is a relatively avascular tissue which makes it vulnerable to get affected. Pseudoaneurysm of the MAIF is a rare but potentially life-threatening complication of aortic valve endocarditis, aortic valve surgery, or chest trauma. Aortic valve endocarditis is usually the main reason that predisposes the MAIF to perforate and form a pseudoaneurysm. Both direct extension of the infection from the aortic wall and the aortic jet striking the subaortic structures and anterior mitral leaflet may damage and infect the MAIF.[1],[2],[3],[4]

Rupture of an MAIF pseudoaneurysm may give rise either to a pericardial tamponade, an eccentric jet of mitral regurgitation, or a direct communication between the LVOT and the LA. In some instances, the pseudoaneurysm remains intact and progressive enlargement may compress the coronary arteries causing symptomatic coronary obstruction.[5],[6] These patients present with angina, mimicking obstructive coronary artery disease. These false aneurysms are prone to rupture, embolize, or even cause further destruction of the aortic or mitral valve apparatus. When the pseudoaneurysm communicates with the ascending aorta or LA, it may result in heart failure with a clinical picture similar to that of aortic or mitral regurgitation, respectively.

TEE is known to be superior to TTE in imaging the valves in detail and to rule out infective endocarditis and paravalvular abscess.[7],[8] TTE may show the pseudoaneurysm located behind the aortic root by the occurrence of systolic expansion and diastolic collapse seen by echocardiography. This should be differentiated from a typical aortic ring abscess which does not show this phenomenon. Color-flow imaging is helpful in confirming this phenomenon. Both TTE and TEE can detect a pseudoaneurysm of the MAIF with sensitivity rates of 43% and 90%, respectively. 3D echocardiography may provide excellent 3D anatomical information and may also help in guiding the appropriate surgical therapy.

The recommended treatment for a pseudoaneurysm of MAIF is surgery even in asymptomatic patients to prevent the development of complications.[1],[2] Resection and repair of the defect can be done with or without aortic valve replacement or homograft replacement of the aortic root. When there is coronary involvement, a concomitant coronary bypass surgery may be necessary. Surgery for this condition is technically complex, hence, the associated morbidity and mortality are not negligible. If the anatomy of the MAIF aneurysm is suitable, even percutaneous device closure of the aneurysm can be an option.

In our case, there was no active infective endocarditis of aortic or mitral valves. The aortic valve was stenosed, and hence, was replaced. The pseudoaneurysm was repaired with pledget suture.

  Conclusion Top

MAIF aneurysm even though commonly associated with aortic valve endocarditis may occur in isolation, i.e., idiopathic. 3D TEE is helpful in visualizing the defect in detail and guides in planning the surgical management.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Sudhakar S, Sewani A, Agrawal M, Uretsky BF. Pseudoaneurysm of the mitral-aortic intervalvular fibrosa (MAIVF): A comprehensive review. J Am Soc Echocardiogr 2010;23:1009-18.  Back to cited text no. 1
Xie M, Li Y, Cheng TO, Wang X, Lu Q, He L, et al. Pseudoaneurysm of the mitral-aortic intervalvular fibrosa. Int J Cardiol 2013;166:2-7.  Back to cited text no. 2
Apostolidou E, Beale C, Poppas A, Stockwell P. Pseudoaneurysm of the mitral-aortic intervalvular fibrosa: A case series with literature review. CASE (Phila) 2017;1:221-6.  Back to cited text no. 3
Afridi I, Apostolidou MA, Saad RM, Zoghbi WA. Pseudoaneurysms of the mitral-aortic intervalvular fibrosa: dynamic characterization using transesophageal echocardiographic and Doppler techniques. J Am Coll Cardiol 1995;25:137-45.  Back to cited text no. 4
Parashara DK, Jacobs LE, Kotler MN, Yazdanfar S, Spielman SR, Janzer SF, et al. Angina caused by systolic compression of the left coronary artery as a result of pseudoaneurysm of the mitral-aortic intervalvular fibrosa. Am Heart J 1995;129:417-21.  Back to cited text no. 5
Almeida J, Pinho P, Torres JP, Garcia JM, Maciel MJ, Lima CA, et al. Pseudoaneurysm of the mitral-aortic fibrosa: myocardial ischemia secondary to left coronary compression. J Am Soc Echocardiogr 2002;15:96-8.  Back to cited text no. 6
Çimen T, Doğan M, Kızıltepe U, Akyel A, Sunman H, Yeter E. Mitral-aortic intervalvular fibrosa pseudoaneurysm with rupture into the left atrium: A three-dimensional trans-esophageal echocardiographic approach. Anatol J Cardiol 2015;15:1030-1.  Back to cited text no. 7
Velibey Y, Güngör B, Bolca O, Eren M. Pseudoaneurysm of the mitral- aortic intervalvular fibrosa and complementary role of 3D transesophageal echocardiographic imaging. Anadolu Kardiyol Derg 2013;13:E 44-5.  Back to cited text no. 8


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