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Intraoperative echocardiography in a patient with a double-orifice mitral valve.

• Volume 117 • Number 4 www.anesthesia-analgesia.org 773 CASE PRESENTATION A 50-year-old man presented with unstable angina. Cardiac catheterization demonstrated 80% ostial stenosis of the left anterior descending coronary artery and 70% proximal stenosis of the second obtuse marginal branch. A preoperative transthoracic echocardiogram revealed normal left ventricular (LV) systolic function, no mitral regurgitation or stenosis but mentioned a mitral valve (MV) deformity comprised of an ectopic set of leaflets. No further workup was performed. He was scheduled for coronary artery bypass graft surgery. Written consent was obtained from the patient for this publication. After induction of anesthesia, a transesophageal echocardiography probe (IE33 X7-2t xMATRIX Array probe, Phillips Healthcare, Andover, MA) was inserted without complication. The midesophageal mitral commissural view (ME MC) in 2D echocardiography (2DE) demonstrated a pivot point on either the anterior or posterior leaflet (Fig. 1A). The transgastric basal short-axis view (TG basal SAX) demonstrated this pivot point on A2 and P2 and showed 2 separate, equal-sized orifices that were side-by-side inferomedial to anterolateral (Fig. 1B). Using color flow Doppler (CFD) in the TG basal SAX view demonstrated 2 streams of blood across the MV. In the midesophageal CFD examination, there was no turbulent anterograde diastolic flow into the LV through either orifice, which would have indicated stenosis. There was trace mitral regurgitation at the A3/P3 region of the inferomedial orifice. Pulsed-wave Doppler also demonstrated no mitral stenosis with a mean pressure gradient of 1 mm Hg through either orifice. Real-time 3D echocardiography (RT3DE) examination confirmed the findings of a doubleorifice MV (DOMV) (Fig. 2; Video 1, see Supplemental Digital Content 1, http://links.lww.com/AA/A536). Given the apparent normal function of the MV, it was elected to continue with the scheduled coronary artery bypass graft surgery which was uneventful with a normal postoperative course. DISCUSSION The embryological mechanism of DOMV is thought to occur due to an abnormal union into a single structure of the dorsal endocardial and left lateral endocardial cushions during the early developmental stage.1 DOMVs are always associated with an anomaly of the subvalvular apparatus, leading to a separate tensor apparatus for each orifice.2 This abnormality was found in 1% of autopsied cases of congenital heart disease in the cardiac registry of Children’s Hospital, Boston, MA.1 It is associated with other congenital anomalies such as atrioventricular canal defect, bicuspid aortic valve, Ebstein anomaly, dysplastic tricuspid valve, interrupted aortic arch, patent ductus arteriosus, and coarctation of the aorta.3,4 DOMV is usually discovered at autopsy or as an incidental finding on imaging studies. Trowitzsch et al.2 recognized 3 types of DOMV (Table 1). The fibrous bridge, seen in all 3 types, consists of mitral leaflet tissue and chordae tendinae.5 The classic lesion, termed the “hole” or “eccentric” type, occurs in approximately 85% of these patients, and is characterized by a smaller accessory orifice at either commissure (Table 1, Fig. 3).2 The second type, which we describe in this report, contains a complete bridge dividing the orifice into inferomedial and anterolateral parts (Table 1, Fig. 3). 2DE views, including ME MC and TG basal SAX, demonstrated the rare congenital anomaly of DOMV. The appearance of the pivot point on the MV leaflets (Fig. 1A) in the ME MC suggests that the tissue bridge is at the annular level. Unfortunately, we did not acquire a TG 2-chamber view or a TG long-axis view. These views could have been instrumental in demonstrating separate subvalvular apparatuses for each orifice as well as showing 2 separate jets on CFD coming into the LV. Even though this patient had no hemodynamically significant valvular abnormalities associated with his complete bridge type, approximately 50% do have significant regurgitation or stenosis that needs to be surgically repaired. It is much more common to have regurgitant lesions rather than stenotic ones with double-orifice MVs. When examining these patients for mitral stenosis, it is crucial that the pulsed-wave Doppler sampling gate be placed at the tips of the MV leaflets of both orifices. If there is only 1 orifice with stenosis, the echocardiographer should calculate the total MV area (MVA) by combining the area of both orifices. If this total MVA is <1.0 to 1.5 cm2, it necessitates surgical repair. The MVA of both orifices can be calculated by the pressure half-time method or deceleration time and added together. Likewise, it can be calculated by the continuity equation but the 2 combined velocity time integrals (VTIs) of the MV orifices should be in the denominator.