Search

Leiomyoma of the mediastinum is rare and only 10 cases have been reported in Japan. A 57-year-old woman was admitted to our hospital complaining of dyspnea. Roentgenogram, CT scan and MRI of the chest showed a large mass with well defined margins occupying the posterior mediastinum. With the biopsy neurinoma was suspected and thoracotomy was performed which revealed the mass was not encapsulated and not related to the vagus, esophagus, bronchus, aorta, pulmonary veins. Only the mass was dissected carefully and removed. The operative findings and histology diagnosed it leiomyoma from unknown origin. But we suspected the tumor of originating from the small blood vessels, because they were surrounded with the spindle tumor cells.

In the last 14 years, 7 consecutive patients with supravalvular aortic stenosis (SVAS) underwent surgical treatment for SVAS and/or for associated lesions. There were 5 male and 2 female patients ranging in age from 3 months to 12 years. Six of them had associated other cardiac anomalies; two had severe multiple peripheral pulmonary stenoses (PPS) and one each had ventricular septal defect (VSD), valvular pulmonary stenosis, coarctation of aorta with patent ductus arteriosus (PDA), total anomalous pulmonary venous return (TAPVR) with pulmonary branch stenosis, PPS and left lower pulmonary venous obstruction. The type of SVAS were localized in all and 5 of them underwent successful surgical repair of SVAS (3 with extended aortoplasty and 2 with patch aortoplasty). In 2 patients with VSD or valvular pulmonary stenosis, associated cardiac anomalies and SVAS were repaired simultaneously. Four patients had undergone previous operations, which included repair of severe multiple PPS by extended peripheral pulmonary arterioplasty (case 4, 6), repair of coarctation of aorta and division of PDA (case 5), repair of TAPVR (Ia + IIa) and pulmonary branch stenosis (case 7). There was no operative death and one patient died late postoperatively (case 7) due to right heart failure in a follow up period of 3 to 14 years. In conclusion, it is important to select the appropriate surgical treatment according to the location and the severity of associated other cardiac anomalies as well as the severity of SVAS, and extended peripheral pulmonary arterioplasty is considered to be a recommended method for the relief of severe multiple PPS associated with SVAS.

The purpose of the present study is to evaluate the effects of addition of a bridge external traction to our conventional sternocostal elevation technique in the surgical management of funnel chest in children. During the period from 1970 to 1979, a total of 29 children with funnel chest were treated surgically using sternocostal elevation technique (Group I). Operative technique consisted of total subperichondral resection of deformed costal cartilages (usually 3rd to 8th, bilaterally), transection of deformed portion of the sternum in 2-3 points and fixation of the sternum in elevated position using 2 Kirschner wires, and shortening resuturing of the opened perichondrium. Since 1980, an addition of the bridge external traction to our conventional sternocostal elevation technique was applied for about 10 days after operation in 82 consecutive children (Group II). The vertebral index (B/A = sagittal diameter of vertebral body/minimum sternovertebral distance), D/C ratio (maximum sagittal depth of depression of sternum/distance from anterior surface of the vertebral body to the sternum at Louis's angle) and E/C ratio (E = minimal sagittal distance from anterior surface of the vertebral body to the sternum) were measured in all cases and compared before and after operation and also between the two Groups. Preoperatively, there was no significant difference in all 3 indices between the two Groups. Postoperative B/A, D/C and E/C ratio in Group I were 0.28 +/- 0.06, 0.14 +/- 0.19, 1.09 +/- 0.32 (mean +/- SD), respectively. There were a significant drop in D/C ratio and a significant elevation in E/C ratio after operation.(ABSTRACT TRUNCATED AT 250 WORDS)

One-hundred and thirty-six patients received the classical Blalock-Taussig shunts between 1980 and 1990. Their age range at operation was 4 days to 12.8 years and their median age was 13 months. The operative mortality rate was 0.7% (1/136). The survivors were followed up from 1 month to 11.5 years, 5.4 years in average. Twenty-five patients required another shunt and the mean interval to that procedure was 2.4 years (modified Blalock-Taussig 23, classical Blalock-Taussig 2, Glenn 1, internal mammary artery-pulmonary artery shunt 1). Forty-five patients received corrective operations, there were four operative deaths (Fontan 2, Rastelli 2). There were 15 late deaths of which three deaths were not cardiogenic. One year after operation, 91.0% of patients remained in well-palliated condition. At three years after operation, 76.4% of patients continued to be in well-palliated condition. There were twelve neonates in this series. Their age range was 4 to 26 days and their median age was 13 days. There was no operative death. Five patients required second shunt. There were two late deaths. At present, six patients continue to be in well-palliated condition 8 months to 9 years after first operation. Angiographic findings showed the stenotic change of the vascular anastomoses in 49.3% (35/71) of patients. This study suggests that polydioxanone suture (PDS) will be useful for the growth of the anastomoses in Blalock-Taussig operation.

An extremely rare case of a primary hemangiopericytoma of the chest wall was reported. A 40-year-old female without any complaints was detected to have an abnormal shadow in the right chest wall on a chest X-ray film. The tumor was 2.6 x 2.2 x 2.0 cm in size and was resected together with the 8th rib which adhered to the tumor. Postoperative pathohistological examination led to the diagnosis of hemangiopericytoma. Postoperative course was uneventful, and the patient is now doing well and has had no recurrence for 11 months since the operation. A brief review was made on 9 cases collected from the literature.

To investigate factors affecting surgical outcome of open heart surgery in neonates, a retrospective review was carried out on 38 patients operated in the 9 years period from May 1981 to August 1990, in our institution (TAPVR19, TGA7, HLHS7, and others 5). In the former 3 years and 9 month period, 12 patients were operated with the use of profound hypothermia and circulatory arrest (Group I) and in the latest 5 year and 5 month period, continuous deep hypothermic bypass was used in all but for 7 patients with HLHS (Group II). In the group of neonates with TAPVR, operative mortality improved to 7% (1 of 14) in Group II compared to that of 60% (3 of 5) in Group I. CVP and LAP measured immediately after cessation of cardiopulmonary bypass (CPB) were lower in Group II and the value of CPK-MB was significantly lower in Group II. There was a significant negative correlation (r = -0.695, p less than 0.05) between CPB time and urine volume for 72 hours after operation. A significant negative correlation (r = -0.899, p less than 0.01) was also obtained between CPB time and urine volume in the group of 7 neonates with TGA. There was no significant difference in urine volume between patients with TAPVR and TGA despite of significantly longer CPB time in patients with TGA. It is concluded that, 1) improved protection of cardiac and renal function was obtained in neonates operated with the use of continuous deep hypothermic bypass, 2) surgical outcome was influenced by the method of CPB, CPB time and differences in cardiac function ascribed to primary lesions.

A 12-month-old infant, weighting 6.7 kg, underwent mitral valve replacement with a 17 mm Björk-Shiley prosthetic valve for parachute mitral valve, and patch closure of the associated ventricular and coronary sinus septal defects. Progressive pulmonary artery hypertension and heart failure required surgical intervention. The diagnosis of parachute mitral valve and associated lesions was established by echocardiogram and angiogram. Postoperative recovery was uneventful and he is doing well 3 years after the operation. To our knowledge this is the first successful case to be reported in the literature with such combination of the congenital anomalies.

This study was undertaken to assess the effect of a calcium antagonist, nicardipine (N), added in a cardioplegic solution on the ischemic myocardium. Isolated rat hearts were perfused with oxygenated Krebs Ringer Bicarbonate (KRB) solution by Langendorff's perfusion method and were subjected to 2 hours of ischemic arrest at 30 degrees C with multidose cardioplegia (every 30 min, for 5 min) and a subsequent 60 min of reperfusion. HR, LVP, coronary flow and oxygen tension of coronary effluent were monitored. Oxygen saturation of intracellular myoglobin and redox state of mitochondrial cytochrome aa3 in the myocardial cell were continuously measured throughout studies by a spectrophotometer. Oxygenated crystalloid cardioplegic solution (KRB) containing 25 mM of potassium was used. 40 rats were divided into 4 groups (10 rats each) according to the concentration of N (none, 0.5, 1 and 2 mg/L) in fully oxygenated potassium cardioplegic solution (PO2: 601 +/- 31 mmHg). The percent recovery of pressure-rate product after reperfusion was compared in each group and the optimal concentration of N was found to be 1 mg per liter of cardioplegic solution. No significant difference was found between Group Ia (N = 0 mg/L) and Group Ib (N = 1 mg/L) in metabolic or hemodynamic recovery after reperfusion. In other experiments, 40 rats in Group IIa (N = 0 mg/L, n = 20) and Group IIb (N = 1 mg/L, n = 20) received 10 ml of poorly oxygenated cardioplegic solution (PO2: 215 +/- 10 mmHg) on each reinfusion followed by a 25 min interval of ischemic arrest. The index of oxygen utilization, MVO2/pressure-rate product after reperfusion was significantly lower in Group IIb than in Group IIa (p less than 0.05). The results show that the addition of N (1 mg/L) to the cardioplegic solution preserved a more aerobic state (higher intracellular oxygen level) in the myocardium by further suppressing myocardial oxygen demand during the ischemic period which resulted in better myocardial protection. Therefore, it is concluded that the addition of N to the cardioplegic solution enhances myocardial preservation during myocardial ischemia.

During an 8-year period, 53 infants less than six months of life underwent repair of coarctation of the aorta (CoA). Preoperatively 58 percent of patients received intensive circulatory and/or respiratory care. The diagnosis was established by echocardiography and counter current aortography via a radial artery in the majority of recent cases and the surgical relief of CoA was effected within 24 hours of the diagnosis. The last 51 patients were managed under the policy of simple repair of the CoA in patients with significant VSD (Group II), with the addition of pulmonary artery banding for patients with complex cardiac lesions with VSD (Group III) or for patients with incremental risk factors in Group II such as prematurity of body weight of less than 1500 gm, other organ anomalies requiring major operation in neonate, pulmonary complications or severe malnutrition. There was no intraoperative death, one (2%) postoperative and five (10%) late deaths among the lost 51 patients. Only one (2%) showed sign of recurrence among 46 patients followed for 16-112 (mean 47) months.(ABSTRACT TRUNCATED AT 250 WORDS)

A 12-year-old girl with tricuspid atresia who underwent a side-to-end anastomosis between the ascending aorta and the right pulmonary artery at the age of two months, developed a false aneurysm of the right interlobar trunk artery. The diagnosis was established by angiography, computed tomography and bronchoscopy. Emergency surgical intervention was required because of impending aneurysmal rupture into the right interlobar bronchus. Creation of a left Blalock-Taussig shunt to tide over the anticipated postoperative hypoxemia and right middle and lower lobectomy were carried out successfully. The false aneurysm in this case occurred following an episode of a streptococcal infection and was probably mycotic in origin. But it is possible that the presence of aorto-pulmonary shunt itself might have affected as a predisposing factor for aneurysm formation in this case.

A case of thoracoabdominal ectopia cordis with single ventricle and pulmonary stenosis is reported. A cyanotic boy with omphalocele was brought to our hospital soon after his birth and was diagnosed as thoracoabdominal ectopia cordis with single ventricle and pulmonary stenosis. A conservative treatment of omphalocele was performed successfully and a plastic chest shield had been made for him which protected the anterior surface of his chest. He continued to develop in a fairly satisfactory condition and operation was delayed until he was 8 years old when he had severe cyanosis and frequent episodes of headache. The operative procedures were; 1) ascending aorta to main pulmonary artery shunt with EPTFE tube; 2) to bring the heart into the thoracic and abdominal cavity by developing widely the pericardial and diaphragmatic defect behind the heart's preoperative position; 3) to reconstruct the new pericardial sac lying from thoracic to abdominal cavity by covering the heart using EPTFE sheet. This trial of making a new pericardial sac for thoracoabdominal ectopia cordis is not reported so far in the literature. It is expected that the heart has being raised to the thoracic cavity with patient's physical growth after this operation.

This paper presents a review of our experience of 3 cases of systemic to pulmonary artery shunt operation and 2 cases of subclavian flap aortoplasty using aberrant subclavian artery. The aberrant subclavian arteries were divided to release the vascular rings and were used as shunt grafts or reversed subclavian flaps. In case of shunt operation, this graft would be superior to the EPTFE graft of modified Blalock-Taussig shunt, from the point of view of the grafts' growth ability. In case of aortoplasty, if arch hypoplasia exists, this flap can repair it beyond the coarctation segment. When the aberrant subclavian artery is dissected, it should be avoided to use the contralateral subclavian artery, as it may cause cerebral damage because of the impairment of bilateral vertebral arterial circulation.

From March, 1982, through March, 1988, 8 infants less than 3 months of age underwent surgical treatment of interrupted aortic arch. Five patients had IAA type A and 3 patients had type B. Seven patients with associated VSD underwent staged operations and 1 infant with aortopulmonary window underwent primary repair off cardiopulmonary bypass. Aortic arch reconstruction was by subclavian aortic anastomosis (6) or large tube graft (8 mm) interposition (2) combined with pulmonary artery banding (5). All seven patients with associated VSD survived the first-stage operation. One patient subsequently underwent pulmonary artery banding, two underwent patch aortoplasty and four patients underwent intracardiac repair and removal of a pulmonary artery band, with six survivors (86%). The operative death occurred in an infant in whom modified Damus-Kaye-Stansel operation was carried out for severe subaortic stenosis, which rerouting all left ventricular blood through VSD and the main pulmonary artery into the ascending aorta and reconstructing right ventricular-distal pulmonary artery connection by a valved external conduit. All six surviving patients are doing well at present (mean follow up of 3 years) without any significant pressure gradient between the ascending and thoracic aorta. Based on these data we conclude: (1) Aortic arch reconstruction and pulmonary artery banding can be reliably performed even in critically ill infants with IAA and isolated VSD. (2) The primary repair will provide better chance of survival in infants with IAA associated with significant LVOTO, truncus arteriosus or aortopulmonary window that do not readily lend themselves to pulmonary artery banding.