An 85 year-old woman underwent mitral valve replacement with Carpentier-Edwards PERIMOUNT (CEP) at the age of 72 because of rheumatic mitral stenosis. Thirteen years after its implantation, prosthetic valve dysfunction developed increasingly severe aortic valve stenosis and she underwent double valve replacement. Prolapse was found in one leaflet of the explanted CEP valve, while neither visible calcification nor tear was detected.

Extracardiac Conduit Repair (ECCR) is conventionally selected for the Tetralogy of Fallot (TOF) with unusual coronary artery distribution. However, in recent years conduit obstruction has been an important factor. Recently, we select the Right Ventricle Outflow Tract Reconstruction (RVOTR) using direct anastomosis between PA and RV as much as possible. In this paper, RVTOR was compared with ECCR. RVOT stenosis was relieved sufficiently and the ventricular function was well maintained after both surgical methods. We concluded that RVOTR should be selected instead of the ECCR because of the conduit obstruction.

From October, 1960, to December, 1976, a total of 167 patients with the tetralogy of Fallot (TOF) underwent corrective repair under simple deep hypothermia at Iwate Medical University. In 59 out of 167 patients the address or telephone number were identified. Fifty-four patients, consisting of 25 males and 29 females, were investigated by written questionnaire or telephone interview. They were followed for 20-35 years. The mean (±SD) age at operation was 5.3± 4.2 years old (range 6 months to 19 years). Reoperations were successfully performed on two patients with residual shunts. Among these, 43 patients (80%) were in NYHA class I, and 11 patients (20%) were class II. None of the patients were in class III or IV. Medication was not prescribed except in one patient. Twenty-eight patients (52%) married and gave birth to 34 children, none of whom had congenital heart disease. A total of 51 (94%) patients were employed, or were housewives. In conclusion, most patients were considered to have a good quality of life long after repair of TOF under simple deep hypothermia.

We report a successful open heart reoperation of a 14-year-old girl with Alagille syndrome. The patient underwent a living related donor liver transplantation at the age of 9 years in another hospital because of liver failure due to a paucity of interlobular bile ducts. Two years later, because of progression of her aortic valve stenosis, Ross operation and CABG were performed in the same hospital. Afterwards, her neoaortic valve regurgitation developed due to aortic root dilatation and myocardial ischemia developed by anastomosis site stenosis. She started to experience frequent angina attacks. She underwent AVR and redo CABG in our institution in April 2002. Her pre- and postoperative liver function was normal and no special procedure for the liver was needed, and she was discharged on the 18th postoperative day with no complications. In this country, few open heart surgeries for liver transplant recipient have been performed, and no case of reoperation has yet been reported. If pre- and postoperative liver function are normal, pre- and postoperative management of open heart surgery for a transplant may be perfomed conventionally.

The patient was a 15-month-old girl with Down's syndrome. She had a heart murmur on the first day after birth. The echocardiogram revealed that she had the tetralogy of Fallot (TOF) and mitral insufficiency (MI). She was observed because she had no heart failure or cyanosis. However, she developed heart failure with progressive MI. Then, she was admitted to our medical center for surgical treatment. During the operation, it was confirmed that the primary septum was intact and a large ventricular septal defect was located at the inlet to outlet portion with anterior malalignment. Each leaflet of the atrioventricular valve were attached to the same level and the ventricular septum was scooped out. TOF with endocardial cushion defect (ECD) without primary septal defect was diagnosed based on the operative findings. Surgical repair was performed through the right atrium and pulmonary artery. She was discharged 17 days after operation without any complications. This was a very rare combination of TOF with ECD without a primary septal defect. We discussed this rare condition with a review of the literature.

This study was designed to clarify the usefulness and pitfalls of hypothermic management after Fontan's operation. Twenty-five patients who underwent Fontan's operation and received hypothermic management in an acute postoperative phase from 1974 to 1991 were divided into two groups; the alive (S) group and the dead (D) group. The lowest rectal temperature during the procedure was 32°C on average. There were no significant differences in preoperative indices of pulmonary circulation and renal function. After rewarming, PaO₂ and daily urinary output were increased and central venous pressure decreased significantly in the S group. In all S group patients, urinary output was increased during hypothermia irrespective of peritoneal dialysis. Anuria occurred 2 days on average after induction of hypothermia in D group. Urinary output in D group decreased significantly for 4 days compared to S group. On the other hand, it was possible to save two patients who underwent take-down of Fontan's operation within 6 hours after the onset of anuria. We conclude that hypothermic management is useful in serious cases after Fontan's operation and that daily urinary output in relation to body weight during hypothermia is most important as an index of post operative circulation.