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.

Aortic Valve Replacement with Annular Enlargement for Congenital Aortic Valve Stenosis / 日本心臓血管外科学会雑誌

<b>Objective</b> : To investigate the efficacy of aortic valve replacement with annular enlargement for congenital aortic valve stenosis. <b>Methods</b> : Eleven patients underwent aortic valve replacement with annular enlargement for congenital aortic valve stenosis in our institute between January 2002 and July 2012. The clinical status of these patients, including preoperative and postoperative echocardiography, was evaluated in this study. <b>Results</b> : The median age of the patients was 15.5 years (range : 9-38 years). The patients had a mean body surface area of 1.48±0.3 m² (range : 1.00-1.92 m²). Mechanical prostheses were used in all patients and the techniques of aortic annular enlargement were the Nick procedure in 4 patients, Manouguian procedure in 3 (modified Manouguian in 2), Yamaguchi procedure in 2, and Konno procedure in 2. The average follow-up period was 32.1 months (range : 1-117 months). There was neither operative death nor late death. The peak/mean pressure gradient of aortic valve improved from 77.9±31.7/46.6±18.0 mmHg preoperatively to 27.9±7.7/14.8±4.7 mmHg postoperatively and to 28.3±11.1/14.1±7.0 mmHg at intermediate-term follow-up. The estimated left ventricular mass also improved from 206.8±93.4 g preoperatively to 179.7±61.1 g postoperatively and to 100.4±76.3 g at intermediate-term follow-up, respectively. <b>Conclusions</b> : Our series shows the efficacy and safety of aortic valve replacement with annular enlargement for congenital aortic valve stenosis.

A Case of Aortic Root Replacement after Arterial Switch Operation for Transposition of the Great Arteries / 日本心臓血管外科学会雑誌

We report a rare case of aortic root replacement after arterial switch operation (ASO). Ten years after undergoing ASO, a 10-year-old boy underwent a Bentall operation because of progressive aortic valve regurgitation and aortic root dilation. The operation was performed under the division of the right pulmonary artery. This view made it easy and safe to dissect the coronary arteries and to perform aortic root surgery.

A Case of Bentall's Operation at Ten Years after a Ross Operation / 日本心臓血管外科学会雑誌

A 22-year-old woman had been treated with a Ross operation for aortic root aneurysm and aortic regurgitation 10 years previously. In the initial Ross operation, a handmade tri-leaflet conduit was used for the right ventricle outflow tract (RVOT) reconstruction. The conduit was prepared preoperatively, by sewing a folded 0.1 mm expanded polytetrafluoroethylene (ePTFE) membrane onto the luminal cavity of the 24 mm woven double velour vascular graft, thereby creating a tri-leaflet valve. During ambulatory follow up after discharge, dilation of the pulmonary autograft had been observed, and its maximal diameter reached 60 mm. Furthermore, preoperative a pressure study revealed a 25 mmHg pressure gradient between the right ventricle and the pulmonary artery. At the time of reoperation, we performed an aortic root replacement combined with RVOT conduit replacement. A 24-mm woven double velour vascular graft integrating a 21-mm On-X mechanical prosthesis was used for aortic root replacement. A handmade ePTFE tri-leaflet conduit, 26 mm in size, was used to replace the previous RVOT conduit. The operation was successful, and the postoperative course was uneventful. The explanted conduit was sent for microscopic examination, which revealed that the graft was covered by a fibrocollagenous membrane. On the contrary, no surface membrane was found on the ePTFE valve. Moreover the microscopic examination showed cystic medionecrosis of the pulmonary autograft. Both dilatation of the pulmonary autograft and RVOT conduit failure were successfully treated at the second operation. However this young patient will require follow-up of the mechanical prosthesis and RVOT conduit for the rest of her life.