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Article in Japanese | WPRIM | ID: wpr-924528


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.

Article in Japanese | WPRIM | ID: wpr-367301


We experienced the coronary artery bypass grafting (CABG) with essential thrombocythemia (ET). A case is a man of 73 years old. As for him, 3 vessel disease including left main trunk was recognized on coronary angiography, and it was planned CABG. However, we recognized blood cell aberration in blood examination, and it was diagnosed as ET. ET is classed as a chronic myeloproliferative disorder. It has two opposite tendencies, a bleeding tendency and thrombus tendency. Ischemic heart disease to merge ET is acute myocardial infarction by thrombus in case of most, and there are a few cases to need blood circulation reconstruction of coronary artery for angina pectoris. Perioperative hemorrhage and postoperative graft closure become a problem in CABG with ET. With the hydroxycarbamide which is DNA synthesis inhibitor of a platelet count, a function controlled it, and enforced CABG. He doesn't have any cardiac events and complications due to ET for 7 years post CABG. We report this case with a review of the literature.

Article in Japanese | WPRIM | ID: wpr-367212


A 67-year-old man presented complaining of unstable angina. Coronary angiography revealed 50% stenosis of the left main trunk of the left coronary artery and a coronary artery aneurysm in the left anterior descending artery (LAD) #6. Furthermore, significant stenosis was noted in the peripheral LAD #7 and #9. The patient had hepatitis C, probably due to an earlier transfusion, and was suffering from liver cirrhosis (Child-Pugh classification grade A) and advanced thrombocytopenia. We anastomosed the saphenous vein graft (SVG) to the LAD and diagonal branch by off-pump coronary artery bypass grafting; we did not treat the aneurysm. There were no postoperative complications and the patient's progress was good. On postoperative coronary angiography, the aneurysm was occluded and the patency of the SVG was satisfactory.

Article in Japanese | WPRIM | ID: wpr-367033


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.