3D Imaging of Mitral Valve Perforation as a Complication of Austrian Syndrome

Abstract

The following are echocardiographic findings in a 68-year-old male with Austrian syndrome.

The patient presented with sinus drainage, headache, fever, mild productive cough, and conjunctivitis, all of which worsened over 2 weeks. Physical exam revealed a holosystolic murmur in the apex radiating to the axilla, and bilateral rales on auscultation. The work-up revealed multidrug-resistant Streptococcus pneumoniae bacteremia, meningitis, and right eye endophthalmitis.

Initially, a transthoracic echocardiogram (TTE) was ordered to determine if there was any valvular involvement. TTE findings confirmed mitral valve vegetation and quantified the mitral regurgitation (MR) as severe (Figure 1). Measurements included a peak MR gradient of 71.4 mmHg, an MR volume of 83.9 mL, and an MR effective regurgitant orifice area (EROA) of 0.8 cm². The MR volume and EROA were measured using the proximal isovelocity surface area (PISA) method [1].

Transesophageal echocardiography (TEE) was performed to obtain a detailed view of the vegetation and to detect any other potential structural abnormalities. A large vegetation was seen on the mitral valve with associated anterior leaflet perforation (Figures 2, 3), resulting in severe regurgitation (Figure 4). These findings represented advanced valvular destruction, a hallmark of aggressive pneumococcal endocarditis.

The constellation of pneumococcal endocarditis, meningitis, and pneumonia established the diagnosis of Austrian syndrome, a rare but classical triad associated with invasive pneumococcal disease [2].

The confirmation of a massive mitral valve vegetation with anterior leaflet perforation established the anatomical basis for severe regurgitation, thus requiring surgical intervention. The patient responded favorably to antibiotic therapy with eventual clearance of bacteremia. Following clinical stabilization, cardiothoracic surgery was consulted, and the patient subsequently underwent successful mitral valve replacement.

This case is notable for its devastating echocardiographic findings related to Austrian syndrome, which revealed a massive vegetation on the mitral valve. The vegetation caused a perforation extending through both the vegetation and the anterior leaflet. This case illustrates the highly destructive nature of pneumococcal endocarditis, which can rapidly erode cardiac tissue and necessitate emergency surgical intervention.