ABSTRACT
We describe the case of a 1-year and 7-month-old girl who was born at 36 weeks and 6 days of pregnancy weighing 1,351 g. In addition to the diagnosis of Cornelia de Lange syndrome and Tetralogy of Fallot, we confirmed shunt blood flow from the lesser curvature of the aortic arch to the main pulmonary artery. Thus, we additionally diagnosed ectopic patent ductus arteriosus (PDA). Ultrasonography showed interruption and retrograde flow of the diastolic blood flow in the anterior cerebral artery. Therefore, we made a diagnosis of blood stealing due to an ectopic PDA, and we performed surgical intervention via a median sternotomy at 25 days. When we ligated the shunt blood vessel, the percutaneous oxygen saturation decreased from the high 90% range to the low 70% range, thus we temporarily released the ligation. We narrowed the ectopic PDA so that the percutaneous oxygen saturation could be maintained the high 80% range. Postoperative ultrasonography showed improvement of the pressure waveform in the anterior cerebral artery. After discharge, oxygen demand increased gradually with weight gain, and we performed intracardiac repair using a monocusp valve patch at 1 year and 7 months. We report a rare case of Cornelia de Lange syndrome wherein we achieved intracardiac repair of Tetralogy of Fallot after ectopic PDA banding in the neonatal period.
ABSTRACT
A male infant was diagnosed with aorto-left ventricular tunnel (ALVT) because of advanced left ventricular hypertrophy and massive reflux from the aorta to the left ventricle during the fetal period. Aorto-left ventricular tunnel closure was emergently performed 2 h after birth in cooperation with the hospital where he was born. The aortic end of the ALVT was located above the right-left coronary commissure, and the measured diameter was 8 mm. The aortic valve was tricuspid, but it was thickened and the opening was limited ; it was further complicated by a right coronary artery anomaly. Taking the valve distortion and surgical invasion into consideration, only the aortic end was closed using an autologous pericardial patch. Although mild aortic valve stenosis and regurgitation remained after surgery, the postoperative course was uneventful, and the left ventricular hypertrophy and cardiac function were improved.
ABSTRACT
A nine-month-old boy with a prenatal diagnosis of aorto-left ventricular tunnel (ALVT) and aortic valve disease underwent an urgent ALVT patch closure approximately 2 h after birth. The patient subsequently developed airway compression due to the progressive dilatation of the ascending aorta postoperatively associated with aortic regurgitation and stenosis. After 9 months of prolonged respiratory symptoms following the ALVT repair, he underwent a posterior plication of the ascending aorta. After plication, the tracheal compression and respiratory symptoms improved. A computed tomography (CT) scan 1 month after surgery revealed significant free space between the aorta and the trachea, which relieved the aortic compression. Posterior plication of the ascending aorta is achieved by resecting the posterior aortic wall adjacent to the airway. It is the procedure of choice for longer and more effective relief of airway compression compared to other procedures such as anterior aortopexy, the lateral sliding technique, and side clamp plication.