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We evaluated postoperative right ventricular function in the sixty-four consecutive patients with tetralogy of Fallot underwent total correction. The patients were divided to three groups according to the method of right ventricular outflow tract reconstruction: transannular patching (TA group; n = 31); right ventricular outflow patching with preservation of pulmonary valve ring (RV group; n = 12) and transatrial-transpulmonary approach without right ventriculotomy (no-RV group; n = 21). The early results of postoperative cardiac catheterization and echocardiography were compared among the three groups. Degree of pulmonary regurgitation was significantly low in the RV group and no-RV group compared with TA group (p0.005). Right ventricular ejection fraction was the highest in the no-RV group (p0.002). The repair without right ventriculotomy for tetralogy of Fallot can provide the best results with respect to postoperative right ventricular function.

Open heart surgery without the use of homologous blood transfusion was attempted in 81 pediatric patients weighing 5.5-14.9 kg. Autologous blood was donated from arterial monitoring line after induction of anesthesia. This donated blood, actual volume of 9.2 +/- 1.7 ml/kg, was reinfused following the cessation of extracorporeal circulation. Hemodilution resulting from autologous blood donation was well tolerated by all patients. Sixty-eight patients (84%) were operated on successfully without the need for homologous blood. These data suggest that autologous blood donation from arterial monitoring line is a safe and effective method to avoid homologous blood transfusion especially in infants and young children undergoing open heart surgery.

We report a neonatal case of one-stage repair for transposition of the great arteries with intact ventricular septum (TGA with IVS) and coarctation of the aorta (CoA). The surgery was performed at 27 days of age when the patient, a male, weighed 3408 g. Preoperative cardiac catheterization and angiography confirmed the diagnosis of TGA with IVS, CoA, atrial septal defect, patent ductus arteriosus, persistent left superior vena cava, left sided juxta-positioning of appendages and dextrocardia. The procedure was simultaneous subclavian flap aortoplasty through a left thoracotomy and an arterial switch operation through a median sternotomy. The patient's postoperative course was uneventful and he has grown well. Postoperative cardiac catheterization revealed mild pulmonary stenosis (pressure gradient of 19 mmHg between the right ventricle and the pulmonary artery) and mild aortic arch kinking from the arterial switch maneuver. One-stage repair, the combination of subclavian flap aortoplasty and arterial switch operation, provides a good clinical result in this complex malformation.

From January 1981 to December 1990, intracardiac repair of tetralogy of Fallot in 148 pediatric patients, with one surgical death, was directed toward preservation of the native pulmonary valve. Using the accepted preoperative angiographic criterion for the pulmonary valve annulus area (PVA) of 1.8 cm2/m2, 85 patients were candidates for transannular right ventricular outflow patch (TAP). However, in 54 patients with a mean PVA of 1.5 cm2/m2 (range 1.06-1.76), the valve was preserved without using TAP because the morphological changes (cusp thickening and annular distensibility) seemed acceptable for preservation in view of its probable hemodynamic efficiency and growth potential. A morphological classification of pulmonary valve changes has evolved. Retrospectively, 24 (77%) of the 31 patients with TAP had moderate to severe cusp thickening and ring rigidity; this incidence was significantly higher (p0.001) than that in preserved patients (18 of 54 or 33%). The incidence of morphological changes increased with operative age; that is, 2 of 13 (15%) patients younger than 1 year versus 23 of 40 (58%) patients older than 4 years (p0.01). All 54 patients with preserved pulmonary valves were catheterized one month postoperatively. The intraoperative right to left ventricular systolic pressure ratio (RVP/LVP) decreased significantly (p0.005) in one month, from a mean of 0.79 (range 0.44-1.36) to 0.57 (range 0.36-0.97). The PVA increased from a mean of 1.5 to 1.9 cm2/m2 (range 1.20-2.65), and the rate of its increase was significantly larger (p0.005) as operative age decreased. Pulmonary valve regurgitation of greater than mild degree occurred in 8 of 54 (15%) patients with the valve preserved.(ABSTRACT TRUNCATED AT 250 WORDS)

From June 1984 to November 1990, 109 patients with transposition of the great arteries underwent arterial switch operation. There were 5 deaths, yielding a mortality rate of 4.6%. During this period, modifications of the surgical technique were devised to minimize intra- and postoperative problems, such as bleeding, kinking of the coronary arteries, aortic regurgitation and pulmonary stenosis. The surgical refinements that evolved include (1) a more distal division of the ascending aorta, (2) a punch technique for reimplantation of the coronary arteries in a medially rotated position, approximating the commissure, and superior to the upper border of the sinus of Valsalva, and (3) removal of left coronary ostia by incision down from the transected site to include a button of aortic wall, avoiding the free margin of the aorta and patch enlargement of the neopulmonary artery. Since instituting these refinements: (1) the time consumed for hemostasis after termination of the bypass considerably decreased from 111 +/- 59 to 87 +/- 51 minutes (p less than 0.05), (2) the incidence of kinking of the coronary arteries decreased from 29% (4/14) to 7% (6/88) (p less than 0.05), and (3) the occurrence of aortic insufficiency 1 year after correction was reduced from 36% (5/14) to 8% (5/66) (p less than 0.02). However, the occurrence of pulmonary stenosis with a pressure gradient greater than 30 mmHg did not decrease significantly despite aggressive modifications of surgical techniques, and its incidence in the most recent series of 32 patients was still a high 19%.

Konno's aortoventriculoplasty was performed in two children aged 6 years and 10 years old with congenital aortic valvular stenosis. One had previous aortic valvotomy at 21 days of age. Preoperative peak systolic pressure gradients between the left ventricle and the aorta were 120 and 140 mmHg, respectively. The original diameter of the aortic valve ring were 12 mm and 16 mm and one had supra-annular aortic stenosis whose diameter was 7 mm. The 19 mm and 23 mm SJM prosthetic valves were inserted in the subcoronary position. The postoperative course was uneventful. Their ECG showed sinus rhythm with complete right bundle branch block. Both two patients made good recovery regarding clinical data and symptoms.

Three patients with anomalous origin of the right pulmonary artery from the ascending aorta were reported. Case 1 was a 16-day-old girl with the right pulmonary artery arising from the base of the ascending aorta. Division and direct anastomosis of the anomalous vessel to the pulmonary trunk were successfully performed. Case 2 was a 4-month-old girl with the kinked and stenotic right pulmonary artery arising from the distal ascending aorta. At the time of surgery, however, the right pulmonary artery had been completely occluded and the pulmonary hypertension of the main pulmonary trunk had been disappeared. The chest was closed without any corrections. Case 3 was a 17-month-old girl with the stenotic right pulmonary artery arising from the distal ascending aorta. The stenotic lesion of the right pulmonary artery was longitudinally incised and anastomosed with the PTFE graft in an end-to-end fashion, followed by the end-to-side anastomosis of the graft and the pulmonary trunk.

Forty patients with complete atrioventricular canal (CAVC) underwent primary repair at Fukuoka Children's Hospital in Fukuoka, Japan, between August 1, 1981 and July 31, 1989. The age at repair ranged from 2 months to 6 years (mean 19 months); weight ranged from 2.3 to 22 kg. The surgical mortality was 2.5%. Justification for early primary repair was examined. Eleven patients underwent repair before 6 months of age (Group 1), 12 patients, between 7 and 11 months of age (Group 2), and 17 patients, after 12 months of age (Group 3). Degenerative changes in the atrioventricular valve increased significantly as age at repair increased (p less than 0.05 Group 1 versus Group 3). The incidence of residual mitral regurgitation tended to increase in the order of Group 1, 2 and 3, though the degree ranged from trivial to mild. Study of the left atrium/aorta ratio by echocardiography revealed that stable values of around 1.1 in Groups 1 and 2 and around 1.3 in Group 3 continued during the follow-up period of 3 years. Assessment of the diameter of the repaired mitral valve in the mean interval of 26 months in groups 1 and 2 revealed normal growth of the mitral valve annulus. The angle between the repaired mitral valve and ventricular septum, which can be affected by the growth of the ventricular septum, converged to normal range in the mean interval of 26 months. Postoperative pulmonary vascular resistance in Groups 2 and 3 was higher at 4.4 +/- 2.3 and 6.3 +/- 2.2, respectively, than in Group 1 at 3.3 +/- 2.2 (p less than 0.01 versus Group 3).(ABSTRACT TRUNCATED AT 250 WORDS)

From October, 1980, to June, 1987, thirty-eight infants less than one year old underwent correction for total anomalous pulmonary venous connection (TAPVC). Overall operative mortality and late mortality were 13% and 6%, respectively. Residual pulmonary hypertension was noted in 4 patients: three had pulmonary venous obstruction at the site of atrial anastomosis (PVOA) and one had supracardiac (Ia) lesion left after repair of mixed type (IV: Ia + III) of TAPVC. Two late deaths occurred in these with PVOA. Twenty-two patients with supracardiac (I) or infracardiac (III) TAPVC were divided into three groups according to the technical development in atrial anastomosis: the large anastomosis in which venous incision reached into at least one pulmonary vein beyond common pulmonary vein and the continuous running suture were used in 10 patients (group 1), the appropriate size of anastomosis in which venous incision limited within the common pulmonary vein and the continuous running suture used in 4 patients (group 2), and the appropriate size of anastomosis and the interrupted suture in 8 patients (group 3). PVOA were 3 (33%) in group 1, but 0 (0%) in group 2 and 3. Two late death occurred all in group 1 with PVOA. Cardiopulmonary bypass time and aortic clamp time in group 3 were 91 min and 74 min respectively, which did not become longer than those in both group 1 and group 2. Interrupted suture technique does not make operating time longer than continuous running suture one. PVOA is one of the important factors predicting late operative result.(ABSTRACT TRUNCATED AT 250 WORDS)

The postoperative left ventricular function (LV) of Jatene's operation for transposition of the great arteries (TGA) was evaluated by angiocardiography and echocardiography in 39 patients. In 16 patients repaired at less than 3 months of age, left ventricular endodiastolic volume (LVEDV) was significantly decreased at the angiography 2 months after repair: form 215 +/- 685% normal of preoperative LVEDV to 120 +/- 14% normal postoperatively (p less than 0.05) in 7 patients with simple TGA (Group IA), and from 220 +/- 64% normal to 130 +/- 33% normal (p less than 0.05) in 8 patients associated with ventricular septal defect (Group IIA). On the other hand, among 24 patients repaired at 3 or more months of age, 19 patients with simple TGA (Group IB) showed the significant increase of LVEDV (from 159 +/- 40% normal to 183 +/- 23% normal, p less than 0.05), and 5 patients associated with ventricular septal defect (Group IIB) showed no remarkable change (from 208 +/- 96% normal to 193 +/- 23% normal). There was no significant difference of postoperative pulmonary artery (PA) wedge pressure among the four groups. Postoperative left ventricular diastolic dimension (LVDd) revealed the tendency of normalization in all groups during the first postoperative year. Group IA and IIA completed the normalization of LVDd in 2 months and Group IIB in 6 months after the repair. However, LVDd in Group IB could not decrease to normal range even in 12 months after repair. There was a significant correlation between postoperative LVDd and the age at PA banding in 14 patients of Group IB, who had the preparatory PA banding for LV training.(ABSTRACT TRUNCATED AT 250 WORDS)

The current status of Jatene's operation for transposition of the great arteries was described by showing the analysis of our results of an arterial switch operation addition to the review of the literatures. The arterial switch operation recently has become the operation of first choice because of its low surgical mortality (4.5% in author's series) and low incidence of postoperative complication. Surgical techniques to avoid complications such as postoperative bleeding, kinking of the transplanted coronary arteries, aortic valve insufficiency and pulmonary arterial stenosis were presented. One-stage anatomic correction has been performed with low mortality less than 10% in centers dealing high volumes of neonatal surgery (7% in author's series). One-stage correction in the neonate is advocated in views of its low surgical mortality and excellent postoperative left ventricular function.

The present status of the surgical treatment of transposition of the great arteries was described by showing the analysis of our results of an arterial switch operation in addition to the review of the literatures. The arterial switch operation recently has become the operation of first choice because of its low surgical mortality (author 4.5%) and low incidence of postoperative complication. One-stage anatomic correction has been performed with low mortality less than 10% in centers dealing high volumes of neonatal surgery (author: 7%). One-stage correction is preferable and it can be accomplished with the cooperative treatment plan of surgeon and pediatrician, starting immediately after birth.

Twenty five consecutive patients with complete atrioventricular canal (CAVC) underwent one-stage operation from April 1981 to Aug. 1987. Average ages at operation was 18 months (2 to 72) and average weight was 7.0 kg (2.8 to 13.8). Fifteen patients were infants and fifteen had Down syndrome. Conventional cardiopulmonary bypass with pulsatile bypass pump (PBP) and moderate hypothermia at 28 degrees C was utilized in all patients. Single patch technique (SPT) was adopted for initial five patients and two patch technique for the latter twenties. Two patients died perioperatively (operative mortality 8.0%), one of whom from mitral stenosis after SPT and the other from misdiagnosis of large subpulmonary VSD. There was no hospital death. Mean pulmonary artery pressure (mPA), pulmonary systolic pressure to systemic systolic pressure ratio (Pp/Ps) and pulmonary vascular resistance index (PVRI) were decreased remarkably from preoperative values of 56 +/- 14 mmHg, 0.92 +/- 0.13 and 6.2 +/- 4.9 WU.m2 to postoperative of 31 +/- 16 mmHg (p less than 0.001), 0.54 +/- 0.20 (p less than 0.001) and 4.6 +/- 4.0 WU.m2 (NS), respectively. Six patients had residual pulmonary hypertension in which mPA was more than 40 mmHg. One patient who was complicated with severe mitral regurgitation due to dehiscence of suture line and torn chordae had mitral valve replacement. Mean follow-up period was 26 months (5 to 63). The mean weights of 67%N at operation catched up with 87%N 3 years after operation. There were two late deaths, 4 and 20 months after operation between age at operation, both of whom had residual pulmonary hypertension.(ABSTRACT TRUNCATED AT 250 WORDS)

Pulsatile high-flow cardiopulmonary bypass (2.5 l/m2/min with a rectal temperature of 28 degrees C) combined with the Pulsatile Bypass Pump (Kontron Instrument) has been used at Fukuoka Children's Hospital in 259 cases of open-heart surgery in patients less than 1 year of age for a period of 5 years beginning July 1982. The overall results were satisfactory with a surgical mortality of 6.2% (VSD: 96 cases/2 deaths, TGA: 48/1, TAPVD: 34/3, Complete ECD: 15/1, IAA: 10/1, DORV: 10/1, HLHS: 7/4, TOF: 6/0, Truncus Art: 5/0, Others 28/3). The mean duration of cardiopulmonary bypass was 123 +/- 50 minutes during which time the patients had a positive water balance of only 28 +/- 38 ml per kg of body weight. The lowest positive water balance was noted in the patient group with 60-75 mmHg in peak systolic pressure and 30-45 mmHg pulse pressure divided by the pulsatile wave form. This value was significantly lower than other groups of patients with lower or higher peak systolic and pulse pressures. Urinary output during the first 24 hours after operation was 4.1 +/- 1.3 ml/kg/hour. Weight gain on the first postoperative day was 10 +/- 43 g per kg of body weight, and the duration of postoperative respiratory support was 4 +/- 5 days. In conclusion, pulsatile high-flow cardiopulmonary bypass is useful in infant open-heart surgery in light of operative techniques, water balance and postoperative recovery.