Acquired Partially Flail Leaflet Causing Severe Mitral Regurgitation in a Congenital Double-orifice Mitral Valve Avinoam Shiran, MD, MSc, and Basil S. Lewis, MD, FRCP, Haifa, Israel Double orifice mitral valve is a rare congenital mal- formation. We report a 48-year-old woman with new onset congestive heart failure and mitral regurgita- tion. Transesophageal echocardiography showed a partially flail mitral valve and severe mitral regurgi- tation. A deep transgastric view showed a double orifice mitral valve with a smaller accessory antero- lateral orifice, and identified the origin of the regur- gitant jet from the larger posteromedial orifice. (J Am Soc Echocardiogr 2004;17:478-9.) Double-orifice mitral valve (DOMV) is a rare con- genital malformation reported in 1% of postmortem cases of congenital heart disease. 1 It is frequently associated with other cardiac malformations such as atrioventricular septal defects, Ebstein’s anomaly, and coarctation of the aorta. The valve may be regurgitant or stenotic, but can be functionally normal and detected accidentally on echocardiogra- phy. 2 We present an unusual case of a flail mitral valve (MV) leaflet leading to severe mitral regurgita- tion (MR) later in life, in a patient with congenital DOMV. CASE REPORT A 48-year-old woman was admitted for treatment of new onset congestive heart failure. She was morbidly obese and had a history of hypertension. In the last few months she had gained 20 kg and had worsening dyspnea on daily activity, orthopnea, and paroxysmal nocturnal dyspnea. On physical examination there was severe peripheral edema and a grade 2/6 holosystolic murmur was heard at the left sternal border. Transthoracic echocardiography showed normal left ventricular size and contraction, mild left atrial dilatation, and severe pulmonary hypertension (systolic pulmonary artery pressure 90 mm Hg). The MV anatomy was technically difficult to visualize, but there was an eccentric, posteriorly directed MR jet. Transesoph- ageal echocardiography (TEE) from the midesophageal view revealed a partially flail MV leaflet with a posteriorly directed wall-hugging jet and systolic flow reversal in both pulmonary veins (Figure 1,A and B). A transgastric short- axis view showed a DOMV with a large posteromedial orifice (3 cm 2 ) and a smaller (1 cm 2 ) anterolateral acces- sory orifice (Figure 1, C). Color flow Doppler identified the origin of the jet from the anterior portion of the larger posterior orifice (Figure 2,A and B). The chordae from each orifice were attached to a single papillary muscle (Figure 2, C). Midesophageal view at 90 degrees showed complete separation of the 2 orifices (Figure 2, D). The TEE findings were confirmed during operation (Figure 2, E and F). No torn chordae were seen, but the flail segment was devoid of chordae. Because the valve was deemed unsuitable for repair, it was replaced with a Carbomedics No. 27 bileaflet prosthesis (Carbomedics, Austin, Tex). DISCUSSION In this patient transthoracic echocardiography was technically difficult, and the correct diagnosis of severe MR as the cause of heart failure, and certainly the anatomic diagnosis of DOMV, was made only by TEE. Although the MR jet was unimpressive, the presence of an eccentric MR jet in a patient with unexplained heart failure, good left ventricular con- traction, and severe pulmonary hypertension was the indication for TEE. TEE demonstrated the flail segment, wall-hugging jet, and severe systolic flow reversal in both pulmonary veins. The key to the correct anatomic diagnosis of this rare malformation was the deep transgastric view (Figure 1, C). The superiority of TEE over transthoracic echocardiogra- phy in patients with DOMV has been previously reported. 3 In our case the DOMV was the classic type, similar to the first publication of DOMV by Greenfield et al in 1876. 1 In this variant, the small accessory orifice is anterolateral, the atrioventricular canal is usually From the Department of Cardiovascular Medicine, Lady Davis Carmel Medical Center, and the Bruce Rappaport Faculty of Medicine, Technion–Israel Institute of Technology. Reprint requests: Avinoam Shiran, MD, Department of Cardiovas- cular Medicine, Lady Davis Carmel Medical Center, 7 Michal St, Haifa 34362, Israel (E-mail: shiranad@netvision.net.il). 0894-7317/$30.00 Copyright 2004 by the American Society of Echocardiography. doi:10.1016/j.echo.2004.01.014 478