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.

A Case of Aortic Valve Blood Cyst with CoA Complex / 日本心臓血管外科学会雑誌

A 2-month-old girl had been urgently seen on postnatal day 10 due to poor weight gain and tachypnea. Echocardiography showed congenital valvular aortic stenosis (AS), ventricular septal defect (VSD), atrial septal defect (ASD), and aortic valve dysplasia, but no cyst image was seen at the aortic valve level. Aortography revealed a dysplastic aortic valve along with coarctation of aorta (CoA) and patent ductus arterious (PDA). Balloon aortic valvotomy (BAV) was performed on day 53. Ballooning was satisfactory, but there was no change in gradient. Operation was performed on day 70 under a diagnosis of congenital AS and CoA complex. After cardiopulmonary bypass was established, the ascending aorta was transected. The blood cyst originated from the center of the anterior leaflet and was resected. The pressure gradient at the aortic valve decreased to 22.5mmHg. The patient was discharged 25 days after surgery.

Smooth Muscle Cells Transplantation is better than Heart Cells Transplantation for Improvement of Heart Function in Dilated Cardiomyopathy

Muscle cell transplantation may delay or prevent cardiac dilation in dilated cardiomyopathy. The present study was designed to compare the effects of the heart function of smooth muscle cell (SMCs) auto-transplantation and heart cell (CMs) allo-transplantation in dilated cardiomyopathic hamsters, and to determine which cells are better for cell transplantation. CMs and SMCs were isolated from BIO 53.58 hamsters, and cultured for transplantation. CMs, SMCs (4 X 10(6) cells each) or culture medium were transplanted into 17 weeks old BIO 53.58 hamsters to achieve CM transplantation (CMTx), SMC transplantation (SMCTx), and controls (Con) (N=10 each). Cyclosporine (5 mg/Kg) was administered subcutaneously to CMTx. Healthy hamsters (sham, N=6) were used to compare heart functions. Four weeks after transplantation, heart function was evaluated in all groups using a Langendorff perfusion apparatus. Histology demonstrated severe focal myocardial necrosis in the dilated cardiomyopathic hearts. CMTx and SMCTx formed huge muscle tissue in the dilated myocardium. Sham, SMCTx, and CMTx had a better heart function than Con (p < 0.01), and SMCTx had a better peak systolic pressure (p < 0.05) and developed pressure (p < 0.05) than CMTx at any balloon volume. However, sham and SMCTx were not statistically different. SMCTx and CMTx formed muscle tissue and produced better heart function in the cardiomyopathic hearts, and SMCTx showed better systolic and developed pressures than CMTx, even though they were similar in other functions. Significantly, SMCTx had heart functions, which were similar to those of healthy hamster's hearts.