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We analyzed cases with brain damage after surgery for thoracic aortic aneurysm in our institution and investigated the causes, risk-factors and preventive measures for this disastrous postoperative complication. Irreversible brain damage was a complication in 25 out of 184 operative cases (13.6%) over a 21-year period. The cause of brain damage was determined to be embolism by manipulation of the aorta in six cases, clamping of the left subclavian artery in four cases, technical problems of separate cerebral perfusion (SCP) in four cases, severe shock in three cases, embolism unrelated to operative maneuver in three cases, stenosis of a branch of the arch with aortic dissection in two cases, and air embolism, circulatory arrest with insufficient hypothermia and hypoperfusion of a temporary bypass to the left carotid artery in one case each. The neurological symptom improved in eight cases and was unchanged in 17 cases. Eighteen cases died in the hospital. In the univariate analysis, age (p = 0.048), a portion of the aneurysm (p = 0.035), preoperative brain complication (p = 0.003), emergency operation (p = 0.033) and clamping of the arch (p = 0.001) were found to be prominent risk factors for brain damage. In the multivariate analysis, clamping of the arch (p = 0.0310), SCP (p = 0.0327) and emergency operation (p = 0.0223) were prominent. To prevent postoperative brain damage, the arch should not be clamped, appropriate operative techniques to avoid bleeding and to shorten SCP time should be employed, and proper and prompt management of the emergency operation and caution in clamping the left subclavian artery are considered to be necessary.

We reported a successful case of the modified Norwood operation for a 21-day-old neonate with hypoplastic left heart syndrome (MS and AS) associated with an aberrant right subclavian artery and a persistent left superior vena cava. The modified Norwood operation was performed without total circulatory arrest and Cardiac arrest. A 4 mm Gore-Tex graft, which was anastomosed between the right carotid artery and the right pulmonary artery for systemic-pulmonary shunt, was used for cerebral perfusion during aortic arch reconstruction. Coronary perfusion was performed with a small cannula placed on the relatively large ascending aorta during anastomosis between the main pulmonary artery and the ascending aorta. Equine pericardial patch was used for aortic arch reconstruction and the ascending aorta was directly anastomosed to a part of the main PA. Postoperative course was uneventful and postoperative MRI revealed no stenosis of the aortic arch and the pulmonary artery.

Konno's operation was successfully performed on a small infant, weighing 3.8 kg. She underwent pulmonary artery banding on the 29th day of life under the diagnosis of partial atrioventricular septal defect with a small left ventricle, associated with polysplenia. Intracardiac repair was subsequently performed on the 45th day of life, which included atrial partition and mitral valvuloplasty. Eleven months after the second operation, wedge resection on the narrow subaortic fibrous lesion was done concomitant with mitral revalvuloplasty. However, repeated echocardiac study disclosed progression of subaortic stenosis. Cardiac catheterization performed at 2 years of age showed the pressure gradinet of 90 mmHg between the aorta and the LV. The aortic annulus and the subaortic lesion measured 10 mm and 4 mm in diameter respectively. Severe subaortic stenosis was created by protrusion of abnormal mitral tissue to the LV outflow, in addition by secondary circumferential fibrous tissue growth and hypertrophy of the ventricular septum. Konno's operation using on 18A Carbomedics valve was chosen for the certain relief of the subaortic stenosis. Although the operation required extensive ventriculotomy and septostomy because of the small aortic annulus, the postoperative course was uneventful. Two months after the operation echocardiography showed good LV function and low RV pressure of about 40 mmHg.

We report a case of D-transposition of the great arteries associated with double aortic arch. An aortic root angiogram revealed that the left-sided aortic arch was anterior and smaller than the right one and the descending thoracic aorta located on the left side of the spine. Balloon atrial septostomy was performed at one day of age. The patient underwent a simultaneous arterial switch procedure and division of the vascular ring at the isthmus of the left aortic arch through a median sternotomy incision at the age of 16 days. There was persistent postoperative difficulty in weaning the patient from the ventilator. Magnetic resonance images showed re-formation of pseudovascular ring by the connective tissue grown around the divided arch. At the age of 23 days, resection of the remnant of left aortic arch including the left subclavian artery and the diverticulum concomitant with vascular suspension procedure was performed through a left lateral thoracotomy. The patient was subsequently extubated without difficulty and was discharged from the hospital. It is though that a vascular suspension procedure and resection of the subclavian artery are necessary to avoid respiratory obstruction when the great arteries are in an anteroposterior position and Lecompte procedure is performed.

Blood cysts of the heart valves are commonly reported at postmortem examination of infants but are rare seen in older children and adults. A 42-year-old woman was referred to our hospital for cardiac evaluation because of dyspnea on exertion and heart murmur. There was no history of cyanosis or cardiac failure. Cardiac catheterization revealed valvular pulmonary stenosis and atrial septal defect. Right ventricular angiography showed the circular filling defect 0.7 x 0.7 cm in size at the suprapulmonary valve. However, this preoperative abnormal finding was diagnosed as a blood cyst after surgery. At operation a blood cyst originating from the right cusp of the pulmonary valve was found and resected, and was followed by commissurotomy of the pulmonary valve and direct closure of the atrial septal defect. Her postoperative course was uneventful.

Ruptured fusiform descending thoracic aortic aneurysms due to aortitis syndrome are rare. A 29-year-old woman was readmitted to our hospital with a chief complaint of back pain and shock, and diagnosed as having rupture of a descending thoracic aortic aneurysm. She had a past history of aortitis syndrome beginning eight years ago. On chest CT examination earlier a year, the descending thoracic aortic aneurysm was 4.5 cm in diameter, and calcification we observed in the aortic wall. An inflammatory reaction had been controlled by steroid therapy and her blood pressure had been controlled by vasodilator drugs. Emergent graft replacement of a descending aortic aneurysm was successfully performed under a femoro-femoral bypass using a centrifugal pump and membrane oxygenerator, and Cell Sver. Although in the literature surgical treatment is indicated only if there is a symptomatic or enlarging aneurysm especially of saccular type and no operation is justified for an aortic aneurysm showing marked calcification, we concluded that aggressive surgical treatment was necessary for this fusiform aneurysm due to aortitis syndrome.

We report an alternative method of repairing partial anomalous pulmonary venous return (PAPVR) wherein anomalous veins drain into the high portion of superior vena cava (SVC). A six year old girl, weighing 19 kg underwent cardiac catheterization for cardiomegaly. The study showed sinus venous defect and pulmonary veins anomalously drained from the right upper and middle lobe into the high SVC. Under cardiopulmonary bypass with aortic and bicaval direct cannulation, a 2-cm-wide pedicled flap made from the middle part of the right atrium (RA), was pulled into the SVC. The atrial flap divided the SVC from the ostium of the uppermost pulmonary vein (PV) to the ASD. This posterior tunnel drained anomalous PV blood into the left atrium. Postoperative echocardiogram and magnetic resonance imaging showed adequate space in the SVC and the PV channel. The patient maintained normal sinus rhythm after surgery. The advantage of this method is to minimize the risk of injuring the sinus node or sinus node artery, and to easily reconstruct both the SVC and PV channel without using prosthetic materials, even in patients with small RA.

We described a very rare case of malignant schwannoma of the chest wall, which was surgically resected. The patient, a 40-year-old woman, came to our hospital because of an abnormal shadow in the right chest wall of an X-ray film without any symptoms. Computed tomography revealed a solid tumor attached to the posteroinferior aspect of the intrathoracic chest wall. The tumor was a 2.5 x 2.1 cm, mass originating from the seventh intercostal nerve without pulmonary adhesion, and the patient underwent en bloc resection of the tumor. The pathological diagnosis was malignant schwannoma. The postoperative course was uneventful, and the patient, eight years after the operation is now doing well without local recurrence or distant metastasis. We reviewed seven cases of this type of tumor reported in the Japanese literature.

Five cases of traumatic aneurysm of the thoracic aorta associated with blunt chest trauma are described. Although we lost one case, four cases were successfully treated. Case 1, This was a 22-year-old female who underwent surgery for graft replacement of the descending thoracic aorta under extracorporeal circulation two days after injury. Case 2, This was a 17-year-old male who underwent surgery for graft replacement of the descending thoracic aorta under extracorporeal circulation 24 hours after injury. Case 3, This was a 56-year-old female who underwent surgery for graft replacement of the descending thoracic aorta under extracorporeal circulation 24 hours after injury. Case 4, This was a 69-year-old male who underwent surgery for graft replacement with three branches for an aortic arch aneurysm under extracorporeal circulation one month after injury. Despite an emergency operation, we lost case 5 (a 21-year-old male) because of a complete discontinuity of the aortic isthmus. The postoperative course of the other four cases was uneventful. Vessel injury may be more widespread than preoperative radiographic findings demonstrates. Blunt chest trauma is usually accompanied by multisystem injury. Therefore, it is imperative to determine the first priority of treatment based on further preoperative examination in cases having associated injuries.

Eight consecutive patients with tetralogy of Fallot associated with pulmonary atresia successfully underwent corrective surgery without using an extracardiac conduit at a mean age of 2 years 4 months. Each of these patients had undergone a classical or modified Blalock-Taussig shunt at a mean age of 55 days. In 6 cases, central PA was fully mobilized and pulled forward. An autologous flap was made from the rudimentary PA trunk or right PA, then it was anastomosed to the endocardium of RV infundibulum as a posterior wall of the outflow tract. In 7 cases, concomitant extensive pulmonary angioplasty was performed on stenotic and under-developed lesions which were caused by tissue contraction of the PDA. During follow up period from 11 to 25 months, all patients have remained in good condition without restenosis of the RVOT. The mean pressure ratio of RV over LV measured 0.42 in 5 patients who underwent catheterization after operation. We conclude that these procedures are useful for early corrective surgery of tetralogy of Fallot with pulmonary atresia.

We examined the surgical result for non-dissecting aneurysm of distal aortic arch and proximal descending aorta, for which aneurysm, proximal aortic clamp cannot be set at descending aorta because of the proximal progression of aneurysm. In 25 cases out of all 46 cases for 18 years, before 1990, aortic arch was clamped for proximal aortic control with the aid of temporary bypass, femoral artery-vein bypass or left heart bypass (clamp group). In 10 cases proximal aortic clamp was set between innominate and left common carotid artery and in 15 cases between left common carotid artery and left subclavian artery. In the former, temporary or permanent bypass was made to left common carotid artery. After 1990, in 21 cases, aortic arch was not clamped with the aid of separate cerebral perfusion (no clamp group). In the comparison of surgical result between two groups, no clamp group showed less postoperative complication rate in brain damage (28% vs 14%) and bleeding (16% vs 5%) and showed better operative mortality (20% vs 14%) and better hospital mortality (32% vs 19%). Now, in the surgery for non-dissecting aneurysm of diatal aortic arch and proximal descending aorta, it is preferable not to clamp aortic arc, employing separate cerebral perfusion.

A 56-year-old female who was diagnosed incomplete endocardial cushion defect, underwent closure of ostium primum defect and repair of mitral cleft 2 years ago. Mitral and tricuspid ring annuloplasty was done 9 months after the first operation because of an increment of mitral and tricuspid valve regurgitation. The Third operation, mitral valve replacement by mechanical valve and tricuspid valve replacement by bioprosthetic valve, was performed because of the gradually increased mitral stenosis and tricuspid regurgitation. Post-operative course was uneventful. Resected anterior cusp of mitral and tricuspid valve revealed hypertrophy and shortness macroscopically, and revealed fibrosis and calcification histopathologically. It was considered that remarkable regurgitation was due to secondary change of both valves. We concluded that valve replacement should be performed for case of secondary change of atrioventricular valve in adult patient.

A 54-year-old man with an acute retrograde type I dissecting aneurysm of the aorta underwent three operations for four years. The first operation was by the Collins method, the second was a graft replacement of the descending aorta, and the third involved a graft replacement of the ascending aorta and aortic arch, revascularization of the aortic branches and replacement of the aortic valve. In operations on acute retrograde type I dissecting aneurysms of the aorta, the methods of Koster or Collins are in appropriate in cases where sufficient entry treatment is impossible, and graft replacement of the ascending aorta at least is considered to be necessary.

Between January 1975 and July 1992, coronary artery reoperation was performed in 16 of 333 patients who had undergone primary bypass grafting. The interval between the operations ranged from 2 to 147 months. Reoperation was done within one year in two patients, within one to five years in five patients and after more than five years in nine patients. The mean number of bypass grafts was 2.31 per patient in the first operation and 1.75 in the reoperation. The main reasons for the reoperation were an occlusion of the graft in 12 cases and both graft occlusion and new coronary artery disease in 3 cases. In one other case, an emergency right coronary bypass graft done for an acute occlusion during PTCA to an area of right coronary artery stenosis 13 months after two bypass graftings to the LAD and circumflex coronary artery. Fourteen cases had vein graft disease bypassed to the LAD or new coronary artery disease in the LAD and required reoperations of the LAD. In the case with a recurrence of myocardial ischemia after coronary bypass surgery and with bypass grafts to the LAD that were patent, there were no cases requiring reoperation. These findings demonstrate that patency of the grafts to the LAD is an important factor for reoperation after myocardial revascularization surgery.

Ventricular function and hemodynamic parameters before and after Fontan operation were studied in patients with univentricular heart (UVH) of left ventricular (LV) type (9 cases, ages: 6-66, mean 13 years) and right ventricular (RV) type (13 cases, ages: 6-17, mean 9 years) without atrioventricular valve regurgitation. The preoperative ejection fraction (EF) was poor and only 44% of LV type and 23% of RV type met the Choussat's criterion (EF greater than or equal to 60%). There were 2 hospital deaths each in LV type and in RV type, but their causes could not be attributed to preoperative parameters of ventricular function. In survivors, the EF reduced from 62.9 +/- 7.0% before Fontan procedure to 43.4 +/- 12.6% after the procedure in LV type (p less than .05) and from 54.5 +/- 7.0% to 47.0 +/- 9.0% in RV type (p less than .01), although the ventricular end-diastolic pressure (EDP) also reduced from 12.7 +/- 2.7 mmHg before to 6.6 +/- 1.9 mmHg after the operation in LV type (p less than .05) and 10.4 +/- 2.6 mmHg to 5.5 +/- 2.4 mmHg in RV type (p less than .05). the ventricular end-diastolic volume (EDV) also reduced from 185 +/- 35% to 126 +/- 58% of expected normal volume in LV type (p less than .05) and 173 +/- 28% to 99 +/- 18% of expected normal volume in RV type (p less than .01).(ABSTRACT TRUNCATED AT 250 WORDS)

Twelve (37.5%) out of 32 patients who underwent surgical repair of complete transposition of the great arteries (d-TGA) with ventricular septal defect (VSD) and pulmonary stenosis (PS) from 1978 to 1987 had insertion of tricuspid valve (TV) chordae to the infundibular septum, i.e. superior margin of the VSD, which precluded the ordinary Rastelli type operation. Of these 12 patients early 5 patients (ages: 2-11 mean 7 years) underwent atrial switch operations with VSD closure and pulmonary valvotomy with or without fibromyotomy, and recent 6 patients (age: 4-8, mean 6 years) underwent the Rastelli operation (3 cases) or REV (3) by translocating the infundibular septum with TV chordae to the right ventricular side of the internal conduit. None died in hospital in the former group and 1 in the latter. Pulmonary/systemic ventricular peak pressure ration (Ppv/sv) was 0.75 +/- 0.22 (mean +/- standard deviation) in the atrial switch group v. 0.49 +/- 0.08 in the Rastelli/REV group 1 month postoperatively. The higher Ppv/rv in the atrial switch group was attributed to the pressure gradient across the pulmonary outflow tract, which was 37.8 +/- 16.6 mmHg in the atrial switch v. 16.7 +/- 4.2 mmHg in the Rastelli/REV (p less than 0.05). No pressure gradient was demonstrated between left ventricle (LV) and aorta in the both groups. Pulmonary wedge pressure was higher in the atrial switch group (13.8 +/- 1.9 mmHg) than in the Rastelli/REV (10.0 +/- 2.7 mmHg) (p less than 0.05). Systemic atrioventricular (AV) valve regurgitation was noted in 2 of the atrial switch group but none in the Rastelli/REV.(ABSTRACT TRUNCATED AT 250 WORDS)

Two patients, 8-year-old and 7-year-old boys, underwent Konno's operation for left ventricular outflow tract obstruction, which had developed after the repair of interrupted aortic arch and ventricular septal defect. Both cases had bicuspid aortic valve and severe subaortic stenosis. The annular diameters were 15 mm and 16 mm, and pressure gradients between the left ventricle and the ascending aorta were 110 mmHg and 105 mmHg, respectively. A SJM prosthetic valve (23 mm, 25 mm) was used. Postoperative courses were good. Left ventricular outflow anomalies are frequently found in cases of interrupted aortic arch complicated with ventricular septal defect. The stenosis not infrequently develops and becomes apparent several years after primary or staged repair for interrupted arch with VSD as in the present cases. Therefore, careful observation should be carried out following the repair. Konno's operation can be safely performed for this lesion with low mortality and morbidity for valvular and subvalvular aortic stenosis even with small aortic ring.

Two cases of anatomically corrected malposition of the great arteries (SDL) with aorto-mitral fibrous continuity and pulmonary-tricuspid discontinuity are presented. Case 1 was a 24-year-old male with an associated ventricular septal defect (VSD) and pulmonary stenosis. Case 2 was a 1-year-old boy also with VSD. Both cases successfully underwent total correction. The aorto-mitral fibrous continuity was verified in both cases through right ventriculotomy during the operation. The postoperative course was uneventful in both cases and they have remained asymptomatic 5 years after operation. We believe this report to be the first one of anatomically corrected malposition of the great arteries with aorto-mitral fibrous continuity.