Pseudoaneurysm in the region of the fibrous body between the mitral and aortic valve, the mitral-aortic intervalvular fibrosa (MAIVF), is a rare complication. The authors provide a comprehensive review of all relevant English-language articles published from 1966 to December 2009. Pseudoaneurysm of the MAIVF was identified in 88 patients in the medical literature and one from the authors’ institution (total reported cases, 89). Endocarditis and aortic valve surgery were the most frequently associated causative factors. Symptoms and signs of infection, chest pain, heart failure or shortness of breath, and cerebrovascular accidents accounted for 77% of clinical presentations. The formation of a fistulous tract, coronary artery compression, and death were important described complications. Patients with ring abscesses in the MAIVF region and those with prosthetic aortic valves and histories of endocarditis are at higher risk for developing pseudoaneurysm of the MAIVF. Transesophageal echocardiography was able to identify all cases in which it was used. Surgical correction is the treatment of choice.
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Target Audience:
This activity is designed for all cardiovascular physicians and cardiac sonographers with a primary interest and knowledge base in the field of echocardiography; in addition, residents, researchers, clinicians, intensivists, and other medical professionals with a specific interest in cardiac ultrasound will find this activity beneficial.
Target Audience:
This activity is designed for all cardiovascular physicians and cardiac sonographers with a primary interest and knowledge base in the field of echocardiography; in addition, residents, researchers, clinicians, intensivists, and other medical professionals with a specific interest in cardiac ultrasound will find this activity beneficial.
Objectives:
Upon completing the reading of this article, the participants will better be able to:
- 1.
Describe the location and echocardiographic appearance of a pseudoaneurysm of the mitral aortic intervalvular fibrosa (P-MAIVF).
- 2.
List the common and uncommon causative factors for P-MAIVF.
- 3.
Recognize the common clinical presentations of patients with P-MAIVF.
- 4.
Identify the complications and high risk features of P-MAIVF.
- 5.
Recognize the possible treatment options of P-MAIVF.
Disclosures:
The authors of this article reported no actual or potential conflicts of interest in relation to this activity.
The ASE staff, ASE ACCME/CME Committee members and article reviewers who were involved in the planning and development of this activity reported no actual or potential conflicts of interest: Imran Afridi, MD; Chelsea Flowers; Rebecca T. Hahn, MD, FASE; Dean Karalis, MD; Cathy Kerr; Ramdas G. Pai, MD; Priscilla P. Peters, BA, RDCS, FASE; and Cheryl Williams.
The following members of the JASE Editorial Staff reported no actual or potential conflicts of interest in relation to this activity: Julius M. Gardin, MD, FASE; Jonathan R. Lindner, MD, FASE; Victor Mor-Avi, PhD, FASE; Sherif Nagueh, MD, FASE; Alan S. Pearlman, MD, FASE; J. Geoffrey Stevenson, MD, FASE; and Alan D. Waggoner, MHS, RDCS.
Estimated Time to Complete This Activity: 1 hour
Methods
We identified relevant English-language articles published from 1966 to December 2009, searching by the keywords “pseudoaneurysm of the mitral-aortic intervalvular fibrosa,” “left ventricular pseudoaneurysm,” “subvalvular aneurysm,” and “left ventricular outflow tract aneurysm” using the PubMed database. A total of 901 abstracts and full-length articles were screened. A manual search was performed from the citations in primary articles to identify any other reports not identified by PubMed. Any cases of fistula between the left ventricle and the left atrium due to rupture of the MAIVF but without aneurysm formation were excluded. Autopsy series were not included. We identified 67 articles reporting 88 such patients. We also present a new case from our institution, making the total number of cases for analysis 89.
Results
Case Report
A 29-year-old woman with type 1 diabetes underwent transthoracic echocardiography (TTE) after emergency room presentation with atypical chest pain. She had had bacterial endocarditis with Staphylococcus aureus 2 years before presentation. TTE revealed an echolucent area adjacent to the aortic valve, raising suspicion of a P-MAIVF. TEE showed an echolucent area measuring 27 mm in its widest dimension, which was freely communicating with the LVOT. This cavity expanded during systole, bulging into the left atrium and collapsing during diastole. Doppler examination confirmed blood flow into the cavity during systole and flow into the LVOT during diastole ( Videos 1 and 2 ). There was no fistulous communication to the left atrium or aorta. The three echocardiographic studies performed at weekly intervals during her prior admission were reviewed. The third echocardiogram was notable for a small, weakly pulsatile echolucent area in the MAIVF area, which was a new development in comparison with the previous study done a week earlier ( Figure 1 ). This finding had not been identified earlier and suggests that the possible P-MAIVF had developed at that time. However, no evidence of a flow communication by Doppler was observed.
The patient was offered at the time of the current hospitalization and initially refused surgical correction of the P-MAIVF. Seven weeks after diagnosis, she underwent surgical repair with closure of the communication with the LVOT using a Dacron patch (DuPont, Wilmington, DE).
Review of the Medical Literature
Five case series (two or more patients) and 62 case reports of P-MAIVF were identified from reports published between 1966 and August 2009. The largest series included 16 patients. Overall, 90 patients with P-MAIVF were identified. We excluded two patients from one case series report because patient-specific information was not presented, leaving 88 patients from the medical literature. With our patient included, the total number of patients was 89.
Patient Demographics
There were 65 male and 24 female patients (male/female ratio, 2.7:1; Figure 2 ). P-MAIVF developed in all age groups, with the youngest patient being 3 months old.
Etiology
Endocarditis and aortic valve surgery were the two most frequently associated causative factors. Forty-six patients (52%) had prosthetic aortic valves, either bioprosthetic ( n = 6) or mechanical. Of the 43 patients (48%) with native valves, 14 (33%) had bicuspid valves, one had a unicuspid valve, and 27 had normal tricuspid aortic valves. In one patient, there was no information regarding aortic valve structure.
Active or prior endocarditis was present in 64 patients. There was no endocarditis in 19 patients, and in six patients, the reports did not have adequate information regarding the presence or absence of endocarditis. All but two patients with endocarditis had aortic valve involvement; in the other two, there was mitral valve endocarditis with a normal aortic valve. Active endocarditis was present in 28 patients at the time of diagnosis, 22 patients had histories of endocarditis from 2 months to 6 years earlier, and in 14 patients, temporal information was not presented. Of the 32 patients who had the causative organism described, 17 had S aureus , 13 had various subtypes of Streptococci , one had Brucella suis , and one had fungal infection with Paecilomyces lilacinus . Among the 46 patients with prosthetic valves, 35 (76%) had histories of endocarditis at some point.
Aortic regurgitation has been suggested as a contributing factor for the development of P-MAIVF. Of the 87 patients, 42 were noted to have some degree of aortic regurgitation at the time of diagnosis. Possible contributing factors in the 13 patients without endocarditis or aortic valve surgery are listed in Table 1 . There were two patients described who had no histories of endocarditis, valve surgery, trauma, or significant aortic regurgitation.
| Study | Age (years) | Sex | Aortic valve | AR | Surgery | Possible contributing factors |
|---|---|---|---|---|---|---|
| Waldhausen et al. | 5 | Male | Tricuspid | None | None | Blunt injury to chest 1 year PTA |
| Taliercio et al. | 22 | Male | Tricuspid | None | Aortic repair 2 years PTA | Blunt injury to chest 2 years PTA |
| Normann | 36 | Female | Tricuspid | None | None | None |
| Popovic et al. | 49 | Male | Bicuspid | Moderate | None | None |
| Yokoyama et al. | 17 | Male | Bicuspid | Moderate | None | Infective pericarditis ( Corynebacterium ) |
| Park et al. | 56 | Female | Tricuspid | Severe | None | Meningitis |
| Yamamato et al. | 35 | Female | Tricuspid | Moderate | VSD repair 28 years PTA | None |
| Bolcal et al. | 22 | Male | Tricuspid | Mild | None | None |
| Linhartová et al. | 59 | Male | Tricuspid | No data | MV surgery 1 year PTA | MV surgery with maze procedure |
The shortest time reported after valve surgery to the development of a P-MAIVF was 9 days, but that patient had had an MAIVF abscess preoperatively. Pseudoaneurysms had developed within 1 month of valve surgery in two patients.
Clinical Presentation
Information on clinical presentation was available in 82 patients ( Figure 3 ). The most frequent presentation was symptoms and/or signs of infection from active endocarditis (39%), followed by dyspnea and heart failure (16%). Cerebrovascular accidents and embolic complications accounted for 12% and chest pain for 10%. Asymptomatic individuals accounted for 9%. One patient presented with a chest wall mass.
Diagnostic Investigations
Information regarding the use of TTE was available in 57 patients (65%). Diagnoses were made in 31 (54%), missed in 24 (42%), and suspected but not definitive in two (4%). Of the 56 patients who had information on TEE, diagnoses were made in all cases. Contrast left ventriculography was the diagnostic technique in eight patients. Three-dimensional echocardiography was performed in five patients, with excellent visualization of the pseudoaneurysm. Computed tomography was performed in four patients and magnetic resonance imaging in six patients. Both modalities provided very good three-dimensional information of the pseudoaneurysm size and its relationship to adjacent structures.
Complications
P-MAIVFs ruptured into the pericardium in three patients. Ongoing endocarditis was present in two patients; in the third, who had a sudden death, autopsy did not reveal active endocarditis. In these three patients, the diagnoses of the pseudoaneurysms were made only at autopsy. Pericardial-related complications were noted in five other patients: one had hemorrhagic pericarditis, one had purulent pericarditis, one had pericardial tamponade with nonhemorrhagic pericardial effusion, and two had uncomplicated pericardial effusions. The pseudoaneurysms had fistulous communications in 18 patients: nine to the left atrium and nine to the aorta. The pseudoaneurysms compressed one or more coronary arteries in 10 patients: circumflex in 100%, left main in 70%, and left anterior descending in 30%. One of these patients also developed a circumflex aneurysm, and another developed an intimal dissection. The pulmonary artery was compressed in three patients. P-MAIVFs were described as “large” in most cases of compressed coronary arteries; the smallest aneurysm described was 2.9 cm in its longest diameter. In five patients, intra-aneurysmal clots were described.
Management and Outcomes
Management and outcomes are depicted in Figure 4 . Surgery was performed in 68 patients: 56 patients had aortic valve replacement with P-MAIVF repair, 10 patients had P-MAIVF repair with no valve surgery, one patient had P-MAIVF and mitral valve repair, and one patient had orthotopic heart transplantation because of severe left ventricular dysfunction. There was no information on surgery in five patients.
Eight patients did not undergo surgery: seven refused, and one was sent to hospice for palliative care ( Table 2 ). Follow-up periods varied from 10 months to 4 years. In the patient with 4-year follow-up, the P-MAIVF diameter increased from 1 to 1.7 cm. In two other patients, there were no changes in the pseudoaneurysm sizes at 10-month and 3-year follow-up.
| Study | Age (years) | Sex | Valve type | Endocarditis | P-MAIVF diameter (cm) | Follow-up (years) |
|---|---|---|---|---|---|---|
| Meyerowitz et al. | 37 | Male | Mechanical | Prior | 1 | 4; ↑ diameter to 1.7 cm |
| Afridi et al. | 45 | Male | Mechanical | Active | 0.8 | 1 |
| Afridi et al. | 69 | Male | Mechanical | Active | 1.1 | 1 |
| Pai et al. | 34 | Male | Mechanical | Prior | No data | 2 |
| Koch et al. | 54 | Male | Mechanical | Prior | “Large” | 0.11 |
| Ghersin et al. | 61 | Female | Mechanical | Active | 1.6 | 0.83; no change in diameter |
| Kahveci et al. | 37 | Male | Mechanical | Prior | “Large” | 3; no change in diameter |
| Salerno et al. | 82 | Female | Bioprosthetic | Possible | 6.2 | 1 |
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