Mitral regurgitation caused by perforation of a pseudoaneurysm of the mitral-aortic intervalvular fibrosa.

A 68-year-old male patient with ex-alcoholic and liver transplantation was admitted with heart failure. Two months earlier, he had been admitted for cholangitis, with positive blood cultures for Escherichia coli, and the possibility of infectious endocarditis (IE) was not suspected. On admission, the patient was afebrile, with heart failure signs and a newly appearing apical pansystolic murmur. Blood cultures were negative. A transthoracic echocardiogram (TTE) showed a severe mitral regurgitation (MR) jet originating from the mitral-aortic intervalvular fibrosa (MAIVF) (Supplementary material online, and Video 1). A transoesophageal echocardiogram (TOE) confirmed the existence of a pseudoaneurysm of the MAIVF (P-MAIVF), posterior to the left coronary and non-coronary sinuses, with a fistula connecting with the left atrium (LA) causing severe MR ( and Videos 2 and 3). A three-dimensional (3D) TOE showed a perforation in the P-MAIVF followed by severe regurgitation from the left ventricular outflow tract (LVOT) to the LA ( and Supplementary material online, ). A cardiac computed tomography angiography (CCTA) confirmed the finding of a P-MAIVF with a connection neck with the LVOT (Supplementary material online, and Video S2). Transthoracic echocardiogram allowed the diagnosis of MR with a non-commissural regurgitant orifice origin. Transoesophageal echocardiogram two-dimensional determined the mechanism of this MR, a P-MAIVF with a fistula towards LA. The use of 3D TOE visualized the fistula entrance orifice in the LA and its anatomical position regarding to the aortic valve and the P-MAIVF. Finally, CCTA established the size and diameter of the neck of the P-MAIVF, as well as its position between the LVOT and the LA. The patient underwent cardiac surgery with mitral and aortic valve replacement (Supplementary material online, ) but he died due to low cardiac output within a few hours after surgery.

Upper transoesophageal echocardiograph showing a pseudoaneurysm of the mitral-aortic intervalvular fibrosa connecting through a fistula to the left atrium. LA, left atrium; P-MAIVF, pseudoaneurysm of the mitral-aortic intervalvular fibrosa.

Transoesophageal three-dimensional echocardiogram, superior view, showing the orifice of the perforation (arrow) and the pseudoaneurysm of the mitral-aortic intervalvular fibrosa. AoV, aortic valve; LA, left atrium; P-MAIVF, pseudoaneurysm of the mitral-aortic intervalvular fibrosa.

In the formation of a P-MAIVF, three possible causes have been reported, including chest trauma, cardiac surgery or transcatheter aortic valve implantation, and IE. The most frequent cause is IE of both native and prosthetic aortic valves, especially when the infection has not been adequately treated. It has been described that a subaortic abscess can organize chronically into a P-MAIVF and that it can rupture, forming a fistula connecting the LVOT and LA. The diagnostic sensitivity of TTE to detect P-MAIVF is 43%. This sensitivity to diagnosis P-MAIVF may increase up to 90% when TOE is used. The preoperative use 3D TOE is very useful to obtain exact information regarding the anatomical relationship of the P-MAIVF with the surrounding structures. Advances in cardiac imaging techniques have led to the recognition of P-MAIVF, almost doubling the number of cases described in the literature in the last decade. Some cases of patients with uncomplicated P-MAIVF without surgery and close long-term follow-up with the combined use of TTE and TOE have been published.,

Supplementary material

Supplementary material is available at European Heart Journal - Case Reports online.

Consent: The authors confirm that written consent for submission and publication of this case report including images and associated text has been obtained from the patient in line with COPE guidance.

Conflict of interest: None declared.

Funding: None declared.

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