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An unusual presentation in a patient with Shone's anomaly.

A 23-yr-old woman with Shone’s anomaly presented to her cardiologist because of worsening exercise tolerance and a history of mitral regurgitation (MR). Her preoperative transthoracic echocardiogram demonstrated severe left ventricle (LV) dilation, global LV hypokinesis, and an ejection fraction of 44%. The left atrial size measured 4 cm. There was a parachute mitral valve (MV) with a large lateral papillary muscle and marked chordal redundancy with systolic anterior motion of the MV without prolapse or LV outflow tract obstruction. There was moderate (3 ) MR with an anteriorly directed jet, and blunted systolic flow in the pulmonary veins. The vena contracta measured 5 mm, and a dense continuous wave mitral regurgitant Doppler signal was seen. There was moderate tricuspid regurgitation. The estimated systolic pulmonary artery pressure was 50 mm Hg. The aortic valve was bicuspid with minimal aortic insufficiency and no aortic stenosis. A cardiac catheterization confirmed the echocardiogram findings and she was sent to the operating room for MV repair or replacement. Intraoperative transesophageal echocardiography (TEE) demonstrated moderate to severe MR with a central jet (Video 1; please see video clip available at www.anesthesia-analgesia.org). Her MV and subvalvulvar apparatus were extremely redundant. (Video 2; please see video clip available at www.anesthesiaanalgesia.org). The mitral annulus measured 3.8 cm. Doppler interrogation of mitral inflow velocities using pulse wave Doppler demonstrated an E wave A wave velocity, E wave velocity of 1.3 m/s, and a deceleration time (DT) of 32 ms (Fig. 1). Continuous wave Doppler demonstrated a mean transmitral pressure gradient of 1.6 m/s and a dense jet of MR (Fig. 2). TEE clearly demonstrated redundant MV tissue in the LV outflow tract without LV outflow tract obstruction. On surgical inspection, her chordae were noted to be matted together in a tangled web (Fig. 3). She had one papillary muscle connected to the anterolateral LV wall. She underwent a MV replacement with a #31 bileaflet valve prosthesis with preservation of the subvalvular apparatus. Shone’s anomaly is a syndrome of left heart obstructive lesions and MV disease. The components of the complex as originally described consist of coarctation of the aorta, subaortic stenosis, a parachute MV, and a supra annular mitral ring. In addition, these patients may have a component of endocardial fibroelastosis which manifests as restrictive ventricular function, and some have a degree of LV hypoplasia which can contribute to morbidity and mortality. In Shone’s anomaly, the MV may be a parachute valve and may also have features of congenital mitral stenosis. As detailed in a previous echocardiogram report, a parachute MV consists of a single papillary muscle that creates an abnormally sized orifice and interferes with normal MV function. In congenital mitral stenosis, the valve can be thick and dysplastic. Chordae are short, thick and fused and form an extensive web. This results in obliteration of the interchordal spaces causing mitral stenosis. MR may exist with these malformations but is not the major problem. Her transmitral Doppler flows were consistent with severe restrictive LV physiology. The E wave velocity was much larger than the A wave velocity and there was a rapid and steep decline in the velocity of flow with a DT of 32 ms. The short DT is consistent with restrictive LV physiology without This article has supplementary material on the Web site: www.anesthesia-analgesia.org.