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 Spontaneous Aortic Root Rupture / 日本心臓血管外科学会雑誌

The patient was a 74-year-old man who was brought to the emergency room with severe chest pain and shock. Transthoracic echocardiography showed moderate pericardial effusion, and contrast-enhanced computed tomography (CT) showed a dilated ascending aorta with hematoma. However, no evidence of an intimal flap in the aorta was found. Bloody pericardial effusion was suggested by the CT attenuation value ; therefore, type A aortic dissection was highly suspected. At surgery, an extramural hematoma was observed on the ascending aorta. An 8-mm dehiscence that had penetrated the adventitia was identified just above the commissure between the right and left coronary cusps of the aortic valve, without dissection in the ascending aorta, and thus spontaneous aortic root rupture was diagnosed. The dehiscence was closed directly with a mattress suture from outside of the sinus, and the dilated ascending aorta was replaced. The patient's postoperative course was unremarkable, and he was discharged 14 days after surgery.