A Rare Case of Cornelia de Lange Syndrome Achieving Intracardiac Repair of Tetralogy of Fallot Following Ectopic Patent Ductus Arteriosus Banding / 日本心臓血管外科学会雑誌

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.

A Case of Resection of Fibroelastoma by Endoscopy / 日本心臓血管外科学会雑誌

We report a case of a 64-year-old woman who presented with symptoms for the common cold, for a medical examination. The chest X-ray showed enlargement of the heart, and echocardiography detected a mobile mass suggestive of a tumor 21×14 mm in the left ventricle. Because there was a risk of embolism, we decided to perform an operation. The tumor was elastic and soft and had a stalk arising from the wall of the left ventricle. We considered that it would be difficult to observe and expose the left ventricular tumor by direct vision. Therefore, we chose to perform a complete endoscopic resection. The tumor was determined histopathologically to be a papillary fibroelastoma. Here, we report the relatively rare benign tumor, a papillary fibroelastoma, and include a discussion of the literature.