Yoshihiro Oshima

Surgery for mitral valve disease in the pediatric age group

OBJECTIVES We reviewed a 20-year experience with the surgical treatment of mitral valve disease in the pediatric age group at our institution with 2 objectives: to clarify the long-term results over the last 2 decades and to evaluate the recent advances in mitral valve operation in children. METHODS Since December 1978, 56 patients have undergone a total of 36 mitral valve repairs and 30 mitral valve replacements. Associated cardiac anomalies were present in 46 patients (82%), and concurrent repair of associated lesions was performed in 37 patients (66%). The age of the patients ranged from 3 months to 15 years (mean, 3.6 years) at mitral valve repair, and ranged from 2 months to 16 years (mean, 5.7 years) at mitral valve replacement. Mean follow-up period was 92.0 months (range, 1-235 months). RESULTS There were 2 hospital deaths and 2 late deaths in patients who underwent mitral valve repair. Reoperation was performed in 4 patients. Three of these patients underwent mitral valve replacement because of residual mitral incompetence. No hospital deaths occurred in patients who underwent mitral valve replacement. Two late deaths occurred after mitral valve replacement. Six patients had a total of 10 episodes of prosthetic valve thrombosis. Thrombolytic therapy with urokinase was successful in all episodes without serious complications. Five patients required reoperations 49 to 141 months (mean, 78.4 months) after the initial valve replacement for relative prosthetic valve obstruction as the result of somatic growth. A valve 2 or 3 sizes larger than the original prostheses was inserted without death. Actuarial survival and freedom from cardiac events at 10 years after the operation were 87.2% and 72.7% in children who underwent mitral valve repair, and 90.3% and 67.3% for those children who underwent mitral valve replacement. CONCLUSIONS The current risk of mitral valve operation in the pediatric age group is low, and the long-term results are satisfactory, irrespective of severe deformation of the mitral valve apparatus and associated complex cardiac anomalies.

Pulmonary atresia with intact ventricular septum: strategy based on right ventricular morphology.

OBJECTIVES Management strategy for pulmonary atresia with intact ventricular septum is controversial. We treated patients with this anomaly according to a management protocol based on our quantitative assessment of right ventricular morphology (index of right ventricular development and right ventricle-tricuspid valve index). In the present study, we reviewed a 20-year experience of the surgical treatment at our institution to define guidelines for selecting the appropriate type of surgical procedure. METHODS Between April 1981 and June 2002, 45 consecutive patients with pulmonary atresia with intact ventricular septum underwent surgical treatment. Open transpulmonary valvotomy was performed in 27 patients and Blalock-Taussig shunt in 18 patients as the initial palliative procedure. Three patients who underwent a successful transpulmonary valvotomy alone in the neonatal period required no further operation. Definitive repair was performed in 32 patients. Biventricular repair was performed on 19 patients, one and a half ventricular repair in 3, and Fontan-type operation in 10. RESULTS There were 1 early and 2 late deaths before the definitive operation in patients who underwent transpulmonary valvotomy. Two patients who had coronary artery interruption died 3 months and 13 years after the initial Blalock-Taussig shunt. There were 1 early and 2 late deaths after the definitive operation. Actuarial survival, including noncardiac death, was 91.1% at 5 years after the initial procedure and 81.5% at 10 years. CONCLUSIONS We treated patients with pulmonary atresia with intact ventricular septum according to a management protocol based on our quantitative assessment of right ventricular morphology with good results.

Risk factors influencing early and late mortality after total cavopulmonary connection

OBJECTIVE Among the later modifications of the Fontan type procedure, a significant alteration was introduced by de Leval and associates when they described the total cavopulmonary connection (TCPC). Although current results of TCPC have encouraged us to extend this procedure to high risk patients, risk factors influencing surgical outcome after TCPC have not been evaluated. We review our experiences with TCPC to identify which risk factors may have had a considerable impact on the outcome of patients undergoing TCPC and to clarify selection criteria of high-risk Fontan candidates for TCPC. PATIENTS AND METHODS We retrospectively reviewed the medical and surgical records of all 76 patients who underwent TCPC between July 1988 and August 2000. A cross-sectional review of these patients was undertaken. RESULTS There were eight early deaths and four late deaths. In a Fishers exact test, the following variables were associated with an increased early mortality after TCPC: systemic ventricular morphology (right ventricle), ejection fraction of the systemic ventricle less than 60%, and prolonged cardiopulmonary bypass time (240 min or longer). The log-rank test demonstrated that heterotaxy syndrome, moderate to severe atrioventricular valve regurgitation, prolonged cardiopulmonary bypass time (240 min or longer), and prolonged aortic cross clamp time (70 min or longer) were associated with late mortality after TCPC. Six deaths occurred in eight (75%) patients who had six or more risk factors, whereas six deaths (9%) occurred in those who had five or fewer. CONCLUSIONS Patients with complex cardiac anomalies who have six or more risk factors should be excluded from TCPC candidates.

Impaired neuroanatomic development in infants with congenital heart disease

OBJECTIVES We performed a regional volumetric study of the brain using 3-dimensional magnetic resonance imaging in infants with congenital heart disease to search for variables in anatomic development of the brain that may be associated with functional impairment. METHODS Forty infants with congenital heart disease-17 infants with single ventricle physiology, 5 with transposition of great arteries, and 18 with ventricular septal defect-were studied prospectively by 3-dimensional magnetic resonance imaging of the brain several months after heart surgery. RESULTS The global volume of gray matter was significantly reduced in the patients with congenital heart disease compared with normal controls (P < .001), whereas no significant difference in the volume of white matter was observed. Further, the decrease in gray matter volume was more apparent in the frontal lobe than in the temporal lobe, especially in infants with single ventricle physiology or transposition of the great arteries. Multivariate analysis revealed that preoperative hypoxia is strongly associated with decreased frontal gray matter volume (P < .01), as well as a diagnosis of hypoplastic left heart syndrome (P < .05). Of note, frontal gray matter volume, which includes the motor area, correlated weakly with psychomotor developmental index scores (P < .01). CONCLUSIONS Brain developmental impairment occurs in many infants with congenital heart disease, especially in those who have preoperative hypoxia and critical congenital heart disease. This quantitative volumetric study encourages larger scale and longitudinal follow-up to elucidate the significance of impaired neuroanatomic development on functional outcome.

Repair of atrial septal defect through a right posterolateral thoracotomy: A cosmetic approach for female patients

BACKGROUND Because the operation for atrial septal defect is considered a low-risk procedure, the cosmetic result has become an important issue. Principally for cosmetic reasons, anterolateral thoracotomy is frequently used for closure of atrial septal defect in young female patients. However, in anterolateral thoracotomy, the skin incision frequently crosses the future breast line, which may cause breast and pectoral muscle maldevelopment. METHODS We review the long-term results of a consecutive series of 126 patients in whom the atrial septal defect was closed through a right posterolateral thoracotomy. The mean age at operation was 7.1 years (range, 1 to 15 years), and the mean body weight was 23.9 kg (range, 6.9 to 56 kg). Defects repaired included 121 ostium secundum (central type), 3 sinus venosus, and 2 ostium secundum without inferior margin. RESULTS The average cardiopulmonary bypass time was 65 minutes (range, 37 to 130 minutes), with an average fibrillation time of 41 minutes (range, 23 to 70 minutes). There was no operative or late mortality. A majority of patients were pleased with their cosmetic results. There were no other late complications. CONCLUSIONS Atrial septal defect can be safely repaired through a right posterolateral thoracotomy approach. This approach offers the benefit of a total absence of scarring and cosmetic disfigurement of the anterior chest wall.

Management of Pulmonary Artery Sling Associated With Tracheal Stenosis

BACKGROUND Pulmonary artery sling is often associated with congenital tracheal stenosis and intracardiac anomalies. This study examines the influence of concomitant anomalies and individual surgical procedures. METHODS Between 1984 and 2006, 31 patients underwent surgical repair of pulmonary artery sling (median, 6 months; range, 29 days to 9 years). Twenty-eight of them underwent left pulmonary artery reimplantation and tracheoplasty, whereas the remaining 3 received only left pulmonary reimplantation. The first 4 patients with long segment tracheal stenosis were treated with a costal cartilage graft and the next 19 with slide tracheoplasty. Five patients underwent tracheal resection with end-to-end anastomosis. Associated cardiac anomalies in 10 patients were repaired concomitantly with left pulmonary artery reimplantation and tracheoplasty. RESULTS Two patients died early of low-output syndrome (n = 1) and ventricular arrhythmia (n = 1). Follow-up was complete in all patients (median, 4.6 years) with 3 late deaths arising from residual tracheal stenosis or pulmonary hypertension. Two survivors with a costal cartilage graft remain free of respiratory problems. Of 16 survivors who underwent slide tracheoplasty, 9 who did not have tracheostomy are fully active and 7 had tracheostomy to treat tracheomalacia and granulation. Among 5 survivors of tracheal resection, 4 are doing well without tracheostomy. Echocardiography showed that the left pulmonary artery was patent in all assessed patients, but 1 required balloon arterioplasty. CONCLUSIONS Left pulmonary reimplatation with simultaneous repair of tracheal stenosis and intracardiac anomalies results in low operative mortality and satisfactory left pulmonary artery patency. However, the management of younger infants with tracheoplasty for long segment stenosis involving carina or complex cardiac anomalies remains challenging.

Effects of Modified Ultrafiltration on Coagulation Factors in Pediatric Cardiac Surgery

AbstractPurpose. Modified ultrafiltration (MUF) after cardiopulmonary bypass (CPB) in children has been reported to reduce the need for postoperative blood transfusion and minimize postoperative blood loss. This study was undertaken to quantify the effects of MUF on coagulation factors in pediatric patients. Methods. Seven children scheduled to undergo open-heart surgery for congenital heart defects were studied. CPB cir-cuits were primed with crystalloid solutions and no blood transfusions were performed. Hematocrit, platelet count, total plasma proteins, albumin, fibrinogen, prothrombin, factor VII, factor IX, and factor X were measured preoperatively, at the termination of cardiopulmonary bypass, and at the end of modified ultrafiltration. Results. MUF was associated with significant (P < 0.05) increases in hematocrit (17.6% ± 1.6% to 21.6% ± 2.4%), platelet count 11.1 ± 2.5 to 12.8 ± 2.4 × 104/mm3), total plasma proteins (2.7 ± 0.3 to 3.4 ± 0.4 g/dl), and albumin (1.6 ± 0.2 to 2.1 ± 0.2 g/dl). Fibrinogen, prothrombin, and factor VII also increased significantly (P < 0.05) during MUF, but factor IX and factor X did not change. Conclusion. We conclude that MUF attenuates the dilutional coagulopathy that occurs during CPB in children. There were slight improvements in the coagulation factors when MUF was employed.

Clinical and pathological features of accessory valve tissue

BACKGROUND Accessory valve tissue is a rare congenital cardiac malformation causing a variety of clinical features. We describe our experience with the surgical treatment of accessory valve tissue to clarify clinical and pathological features of this anomaly. METHODS Between December 1990 and February 1999, 11 children underwent resection of accessory valve tissue together with repair of coexisting intracardiac malformations. Eight patients had accessory tricuspid valve, while 3 had accessory mitral valve tissue. Associated intracardiac malformations included tetralogy of Fallot in 6 patients, transposition of the great arteries in 3, coarctation complex in 1, and ventricular septal defect in 1. RESULTS Accessory valve tissue was completely resected in all but 1 patient with accessory mitral valve and transposition of the great arteries. As total resection of the accessory valve tissue through the pulmonary artery was unsuccessful, partial resection and bidirectional Glenn shunt operation was performed instead of arterial switch operation in this patient. CONCLUSIONS The clinical signs and symptoms of accessory valve tissue vary according to its location and coexisting cardiac malformations. Congenital heart surgeons should be familiar with the clinical and pathological features of accessory valve tissue, so as not to overlook it during operation.

Intrahepatic venovenous shunting to an accessory hepatic vein after Fontan type operation

Because there are various types of systemic venous connections in patients with visceral heterotaxy, unanticipated abnormal systemic venous channels may pose additional problems after a Fontan type operation. We report a case of severe cyanosis caused by anomalous intrahepatic venovenous fistula to an accessory hepatic vein early after total cavopulmonary anastomosis.

Congenital tracheal stenosis: the prognostic significance of associated cardiovascular anomalies and the optimal timing of surgical treatment

BACKGROUND/PURPOSE Cardiovascular anomalies (CA) are frequently associated with congenital tracheal stenosis (CTS), but their prognostic impact on CTS and the optimal timing of surgical treatment remain uncertain. The aim of this study was to explore the prognostic factors and the optimal timing of surgical treatment in CTS patients with CA. METHODS After obtaining institutional review board approval, a retrospective review of 42 patients who underwent surgical repair of CTS between 1996 and 2006 was conducted. The patients were divided into 3 groups: CTS without CA (n = 10, group A), CTS with CA repaired simultaneously (n = 27, group B), and CTS with CA repaired in stages (n = 5, group C). Seven clinical characteristics, including gestational week and weight at birth, the age and body weight at operation, the length of tracheal stenosis (%), the duration of cardiopulmonary bypass (CPB) during surgery, and operation time were compared among the groups using analysis of variance, Fishers Exact test, and Students t test. RESULTS Although no operative mortalities occurred in groups A and C, there were 3 early deaths and 1 late death in group B. The deaths occurred in cases with associated complex CA (critical pulmonary stenosis, tetralogy of Fallot with an absent pulmonary valve, right ventricular outflow block, and cor triatrium). The duration of CPB was significantly different between groups A and B (P = .017), and furthermore, CPB time was significantly longer in early death cases than in surviving cases in group B (318.3 +/- .71.1 vs 204.0 +/- 67.8 minutes; P = .012). CONCLUSIONS Complex CA and long CPB duration would be prognostic factors for the outcome of surgical management for CTS and CA. Simultaneous reconstruction of CTS and simple CA appears to be a reasonable method of surgical intervention, but patients with long segment CTS with complex CA may still be difficult to cure using this strategy, and staged correction may be considered.