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Role of Intraoperative Real-Time Three-Dimensional Transesophageal Echocardiography During Cone Procedure for Ebstein's Anomaly.

BSTEIN’S ANOMALY is a complex congenital cardiac condition associated with structural changes in the tricuspid valve (TV) and functional derangement of the right ventricle (RV). Cone procedure is a new modality of surgical correction of abnormal TV and is performed aiming for a biventricular repair. 1 Utility of intraoperative two-dimensional echocardiography (2DE) has been reported during the surgical repair of Ebstein’s anomaly. 2 Real-time three-dimensional transesophageal echocardiography (RT-3D-TEE) has been found to provide better information than 2DE during cardiac surgery in adult patients, especially for evaluation of left ventricular function 3 and mitral valve repair. 4 However, its role in the intraoperative monitoring of congenital heart lesions is not widely popular, mainly because of unavailability of appropriate probes as well as difficult image acquisition in children who have inherently high heart rates. This case report describes the utility of RT-3D-TEE in an adult patient who had surgery for Ebstein's anomaly of the TV. CASE REPORT A 21-year-old female patient, with a diagnosed case of Ebstein’s anomaly with severe tricuspid regurgitation (TR), had surgery for cone repair of the TV. She presented with history of 3 episodes of syncope and was evaluated in the electrophysiology laboratory for the presence of any aberrant cardiac conduction pathways, which turned out to be negative. Preoperative transthoracic echocardiogram found Ebstein’s anomaly of the TV with moderate-to-severe TR and an 18-mm 23-mm atrial septal defect (ASD), predominantly shunting left-to-right. Examination of the TV leaflets showed a 5-cm long and freely mobile anterior tricuspid leaflet (ATL) and a restricted septal tricuspid leaflet (STL). The morphology of the posterior tricuspid leaflet (PTL) was unclear. On the morning of surgery, routine monitoring was established, including invasive cannulation of the left radial artery. Anesthesia was induced with 300 μg of fentanyl, 5 mg of midazolam, and 8 mg of etomidate, and muscle relaxation was achieved with 9 mg of vecuronium. After tracheal intubation, a 3D-TEE probe was inserted in the esophagus, and the heart was inspected using an ultrasound system (IE 33; Philips Medical Systems, Bothell, WA). Initial 2DE examination confirmed the findings of the transthoracic echocardiogram (Fig 1, Video 1). The 2DE features were a 10-mm/m 2 apical displacement of the STL, moderate-to-severe TR with an eccentric and wallhugging jet, a second TR jet arising through the ATL near the lateral portion of the annulus, and a crescent-shaped left ventricle, which had adequate systolic function. The TV was inspected through virtual right atriotomy and right ventriculotomy using RT-3D-TEE after acquiring full-volume data and zoomed images from midesophageal windows. The PTL was rudimentary and stuck to the right ventricular wall when seen through the RV (Fig 2, Video 2). A fenestration was noticed in the ATL near the lateral aspect of the TV annulus on enfacedzoomed view after cropping through the right atrium (Fig 3). After systemic heparinization, cardiopulmonary bypass was established and electromechanical quiescence of the heart was achieved with antegrade administration of cold blood cardioplegia. The rudimentary nature of the PTL and the presence of fenestration in the ATL were confirmed by the surgeon after right atriotomy. During surgery, the STL and ATL were detached from the RV wall and the annulus, respectively. Cone repair was performed by rotating the ATL toward the STL and suturing their free margins together. Suture annuloplasty of the TV was performed along with closure of the fenestration and plication of the atrialized segment of the RV. The ASD was made restrictive. The patient was weaned from cardiopulmonary bypass with inotropic infusions of milrinone 0.5 μg/kg/min, and norepinephrine, 0.05 μg/kg/min. The TEE examination found a coneshaped TV that had its base at the atrioventricular junction and apex in the RV cavity, with a mean inflow gradient of 2 mmHg (Fig 4, Video 3). The apical area of the cone, which was about 2.4 cm 2 , could be measured in the enface view via virtual right ventriculotomy. The mitral valve and tricuspid valve were inspected side-by-side using RT-3D-TEE (Fig 5, Video 4), which showed the difference in the shape of the apparatus of both valves. Both the valves had simultaneous closure at the end of diastole. The patient was transferred to the intensive care unit and artificially ventilated for 10 hours. She had an uneventful recovery in the postoperative period and was discharged from the intensive care unit on the second postoperative day.