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Hemi-Nikaidoh Operation With A Posterior Left Ventricular Outflow Tract Plasty Fortransposition Of The Great Arteries, Ventricular Septal Defect, And Pulmonary Stenosis
Stephanie N. Nguyen, MD, Maroun Yammine, MD, Emile A. Bacha, MD.
New York Presbyterian - Morgan Stanley Children's Hospital, New York, NY, USA.

A 3-year-old girl (17 kg) with d-transposition of the great arteries, ventricular septal defect, and pulmonary stenosis (d-TGA/VSD/PS) underwent a Blalock-Taussig-Thomas shunt as a neonate, followed by bidirectional Glenn at 6 months. She presented for stage III palliation versus biventricular repair. Preoperative echocardiography demonstrated normal biventricular function, moderately hypoplastic right ventricle, large perimembranous VSD with outlet extension, and functional pulmonary atresia. Given these findings and low filling pressures, she was an excellent candidate for biventricular conversion. Intraoperative assessment revealed a severely hypoplastic pulmonary valve and underdeveloped conus, such that the distance to be gained with conal septum division and posterior root translocation was <5 mm. We therefore opted to perform a hemi-Nikaidoh procedure. The geometric shift was achieved by mobilization of the anterior two-thirds of the aortic root; posterior translocation was augmented by plication of the left ventricular outflow tract (LVOT) and conus, and repositioning of the root deep within the neo-LVOT. The right ventricular outflow tract was reconstructed using a homograft and bovine pericardial patch hood. The Glenn shunt was maintained due to marginal size of right-sided structures. Postoperative echocardiography showed normal biventricular function, mild LVOT gradient (17 mmHg), laminar flow across the conduit and central pulmonary arteries, and no residual VSD. The patient is doing well at 3-month follow-up. Overall, the hemi-Nikaidoh procedure is an effective anatomic repair of d-TGA/VSD/PS in select patients with a diminutive pulmonary root and underdeveloped conus. Leaving one-third of the root in place may avoid bleeding complications of a posterior suture line.


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