BACKGROUND: A fistulous tract in the mitro-aortic intervalvular fibrosa (MAIVF) is a rare entity, which presents as a complication of endocarditis or surgical trauma. Generally, it is associated to a pseudoaneurysm of the MAIVF (p-MAIVF) or aortic abscesses. MAIVF fistulas could potentially lead to devastating complications and a high mortality rate. This condition is managed surgically, either by a percutaneous closure or an open surgical approach. Herein we report the complex case of a patient with a MAIVF fistula secondary to bacterial endocarditis. Further clinical deterioration was caused by severe aortic valve insufficiency and hemodynamic compromise, requiring surgical intervention.
METHODS: A 74-year-old male patient was admitted to a primary care center with complaints of malaise, asthenia, adynamia, hyporexia, and lower limb edema over the past eight days. His past medical history is positive for arterial hypertension and being monorenal. A transesophageal echocardiogram (TEE) was performed, exhibiting a 56% left ventricle ejection fraction (LVEF) and complicated aortic valve endocarditis. Surgical management through an open approach included vegetation resection, valve replacement, and closure of the MAIVF fistula. After completing antibiotic therapy, the patient was discharged without complications. During postoperative follow-up, the patient remained asymptomatic, and the control echocardiogram showed no signs of MAIVF fistula.4.
CONCLUSIONS: The clinical case of a patient with a MAIVF fistula secondary to endocarditis by Streptococcus Anginous was presented. The fistulous tract was not associated to p-MAIVF or aortic abscess, findings which further deteriorate the patient\'s condition and increase the likelihood of fatality. This case reinforces the importance of a prompt diagnosis through cardiac imaging and timely surgical closure of the defect.

BACKGROUND: Mitral-aortic intervalvular fibrosa (MAIVF) is a fibrous region connecting the anterior mitral leaflet (AML) and aortic valve. Pseudoaneurysm of the MAIVF is a rare condition that has been reported as a sequela of infective endocarditis (IE) and surgical trauma. Here, we report a case of a ruptured pseudoaneurysm of the MAIVF, along with some literature reviews.
METHODS: A 65-year-old man diagnosed with moderate aortic regurgitation five years previously had a fever of unknown origin. He suddenly developed headache and apraxia and was transported to our hospital. He was diagnosed with intracranial hemorrhage and admitted. One week after admission, echocardiography revealed aorto-mitral discontinuity and protrusion with severe regurgitant flow from left ventricular outflow tract to the left atrium. The AML was suspected to have ruptured. However, intraoperatively, the AML structure was preserved. A ruptured pseudoaneurysm of the MAIVF was also observed. Therefore, we successfully performed pseudoaneurysm repair using a bovine pericardial patch, aortic valve replacement, and mitral annuloplasty.
CONCLUSIONS: P-MAIVF is a rare but potentially life-threatening complication of IE, for which timely diagnosis and prompt appropriate therapeutic intervention are required. In the present case, although neither obvious active IE nor history of previous IE could be identified, healed IE was considered based on the clinical course. The patient had intracranial hemorrhage (ICH) with well-controlled heart failure and underwent elective surgical repair more than one month after the onset of ICH, while the clinical course after the surgical procedure was uneventful.

Pseudoaneurysm of the mitral-aortic intervalvular fibrosa (P-MAIVF) can be a rare but life-threatening complication of infective endocarditis, cardiac surgery, or blunt chest trauma. Congenital heart diseases especially in young patients are reported as additionally predisposing factors. We present the case of an asymptomatic 52 years-old male with bicuspid aortic valve and gastrointestinal polyposis syndrome in whom a P-MAIVF was incidentally detected. The patient successfully underwent pseudoaneurysm surgical repair and aortic valve replacement and despite no evident causes were found we hypothesized addiotional underlying mechanism of P-MAIVF.

We report a neonate with severe Marfan syndrome (MS), prenatally identified to have persistent atrial tachycardia, biventricular dysfunction, and an unusual structure within the atria. Detailed postnatal echocardiographic evaluation and cross-sectional imaging confirmed congenital pseudoaneurysm of the mitral-aortic intervalvular fibrosa. Emergent testing by next-generation sequencing identified a FBN1 pathological variant, key to establishing goals of care. To our knowledge, this is the first reported case of a congenital pseudoaneurysm of the mitral-aortic intervalvular fibrosa in MS.

The mitral-aortic intervalvular fibrosa (MAIVF) is an avascular, fibrous structure that provides continuity between the anterior leaflet of the mitral valve and the aortic valve. Pseudoaneurysms of this area are rare and more commonly have been described in adult patients following trauma, cardiac surgery, or endocarditis.1,2 Limited case studies describe the occurrence in children following cardiac surgery3, 9 or endocarditis,4,5 with few case reports describing the congenital nature of these pseudoaneurysms.4,6-9 Here, we describe 5 cases of congenital pseudoaneurysms in the MAIVF area identified prenatally, with an additional 6 cases diagnosed postnatally. This is an unusual finding of varying clinical significance that can be isolated or associated with complex congenital heart disease, but importantly, can be identified and monitored in the fetus. This article is protected by copyright. All rights reserved.

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A pseudoaneurysm of the mitral-aortic intervalvular fibrosa (p-MAIVF) can be a catastrophic sequela of untreated active infective endocarditis. We describe a case of infective endocarditis complicated by p-MAIVF without valvular involvement. This case highlights the importance of this rare clinical entity and of transesophageal echocardiography in reaching a diagnosis. (Level of Difficulty: Beginner.).

The mitral-aortic intervalvular fibrosa (MAIVF) is the thin avascular fibrous structure located between the left side of the non-coronary cusp, the left coronary cusp and the anterior mitral leaflet. MAIVF pseudoaneurysm typically results as a complication of endocarditis, aortic valve surgery or chest trauma. We present a case of an incidental MAIF pseudoaneurysm in a 68-year female with a history of rheumatic fever without involvement of the mitral apparatus. We also discuss the presentation, evaluation and management of this rather rare valvular disorder.

BACKGROUND: The avascular region of the fibrous body between the mitral and aortic valves, named mitral-aortic intervalvular fibrosa (MAIVF), is often involved in the periaortic diffusion of infective endocarditis (IE), resulting in abscess or pseudoaneurysm formation. The early recognition of these life-threatening complications is of crucial importance, as urgent surgical correction is necessary. In the first stages of the abscess formation, the only sign is an increased thickness of the MAIVF. To the best of our knowledge, normal transesophageal echocardiography (TEE) examination reference values for MAIVF thickness has not yet been established. The aim of the study was to define the normal ranges of MAIVF thickness in a population of healthy adults who underwent a TEE examination.
METHODS: A population of consecutive adult patients who underwent a TEE examination was enrolled in the study. Measurement was performed in short-axis (SAX) and long-axis (LAX) views. Mean-2 standard deviations (mean-2SDs) and 5%, 10%, 90%, and 95% confidence intervals were evaluated. A comparison with MAIVF thickness in patients affected by aortic IE complicated by abscess formation was performed, and receiver operating characteristic (ROC) curves were constructed to achieve the optimal cutoff value of normality.
RESULTS: A total of 477 consecutive Caucasian adult patients were enrolled (mean age: 69 years, range: 27-93 years). Mean-2SD MAIVF measurement in SAX view was 0.325 cm (95% confidence interval [CI]: 0.319-0.330 cm) and in LAX view was 0.340 cm (95% CI: 0.334-0.346 cm). Computed tomography-MAIVF mean measurement (±2SD) was 0.237 cm (95% CI: 0.110-0.340 cm). ROC curves showed that a cutoff SAX value measurement of 0.552 (area under the curve [AUC]: 95.2%) had a sensibility of 88.2% and a specificity of 92.4%; a LAX measurement value of 0.623 (AUC: 93.3%) had a sensibility of 82.7% and a specificity of 85.7%. The multivariate analysis showed no significant correlation between MAIVF thickness, age, and sex.
CONCLUSIONS: In healthy patients, MAIVF thickness should not exceed 0.600 cm. Above these values, the suspicion of a periaortic abscess formation should be raised. MAIVF increased thickness may be an early sign of perivalvular diffusion requiring an urgent endocarditis team evaluation.

Infective endocarditis (IE) remains a serious and deadly disease despite recent advances in diagnosis and treatment. In all IE cases, the rate of healthcare-associated IE has been reported as 23%. Aortic valve endocarditis may extend to mitral-aortic intervalvular fibrosa (MAIVF), which may cause pseudoaneurysm formation and subsequent perforation. Direct perforation of the MAIVF is a rare clinical finding. In this report, we present a case of bicuspid aortic valve endocarditis which manifested as acute heart failure secondary to perforation of MAIVF and developed after diagnostic coronary angiography. .