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Deep hypothermia with total circulatory arrest is an important technique in the surgical treatment of complex congenital heart defects. However, the clinical use of this technique has been accompanied by occasional postoperative neurological abnormalities. Twenty new born lambs (four groups of five) were subjected to deep hypothermia (15 degrees C) and circulatory arrest for 30 min, 45 min, 60 min and 90 min respectively. Their brain electrical response was monitored by means of the Cerebral Function Analysing Monitor (CFAM), which produces a detailed analysis of the electroencephalogram amplitude and analyses the frequency of the waveforms into standard, beta, alpha, theta, and delta bands. After induction of circulatory arrest both the voltage and frequency recordings became isoelectric within 30-180 seconds and remained flat for the duration of the arrest. During the rewarming phase of the latency of time to initial appearance of electrical activity and continuous CFAM recording showed a good correlation with the duration of arrest and also a significant increase (p less than or equal to 0.001) when the circulatory arrest was maintained beyond 45 min. These findings suggest an increasing risk of neurologic damage when the arrest period is longer than 45 min.
Overall survival after Mustard's operation was assessed in 130 patients with complete transposition of the great arteries and an intact ventricular septum who were operated on at the Brompton Hospital in the 12 year period from January 1974 to December 1985. Actuarial analysis showed a survival at five years of 79.1% (25 deaths in 130 patients). Half the deaths occurred within a month of operation and half up to five years later. Operation under the age of three months carried a greater mortality (six deaths in 11 patients). Statistical analysis showed that these survival figures were consistent throughout the series and did not alter with the year of operation. In the light of recent trends in treatment of this lesion, it is concluded that for neonates an arterial switch procedure is now likely to carry the best chance of long term success. For the patient who presents after the age of three months, however, there may still be a place for the Mustard procedure.
Between January 1973 and February 1986, operations were performed on 120 consecutive patients having usual atrial arrangement (atrial situs solitus), concordant atrioventricular connection, and double-outlet right ventricle. The ages at operation ranged from 1 day to 44 years and the weights from 2.6 to 84 kg. Sixty-three patients had one or more palliative procedures. For those, the hospital mortality rate was 9.5%. Palliation was considered a definitive procedure in 13 patients. Ninety-three patients had a reparative operation, with a 26.9% early mortality rate. In the group who had complete correction, taken as a whole, the surgical outcome was significantly affected by the position of the ventricular septal defect and by the year of operation. The year of operation was the main factor that, by multivariate analysis, correlated significantly with the hospital mortality in those patients having a subaortic defect and spiraling great arteries (p less than 0.05). No difference was found among this group for those patients having the morphologic characteristics of tetralogy of Fallot. The change-over point from the Mustard to the arterial switch procedures was the event with the greatest effect on hospital mortality in patients with a subpulmonary ventricular septal defect (p less than 0.025). Two late deaths have occurred among the 21 patients who had palliative intervention only. Sixty of the 68 survivors with intracardiac repair have been followed up for a period of 2 to 184 months (median 44 months). There were five late deaths (8.3%). Eight patients underwent successful reoperation. All except three of the long-term survivors were in functional class I. Good early and long-term results can be anticipated for the intracardiac repair of double-outlet right ventricle when the ventricular septal defect is subaortic or doubly committed. The arterial switch operation has been demonstrated to be the optimal approach for double-outlet right ventricle with subpulmonary ventricular septal defect. Results in patients with noncommitted ventricular septal defect have remained poor.
In December 1985 a neonate was transferred to a Yorkshire Regional Neonatology Unit (RNU) from a neighbouring hospital and was subsequently found to be colonized with a 'methicillin-resistant' strain of Staphylococcus aureus (MRSA). Spread of the MRSA was controlled by routine cross-infection methods. The RNU was not closed to new admissions, the economic cost of control measures was small and no neonatal death was attributable to MRSA infection. Eradication of the MRSA from the RNU was associated with a decline in frequency of coagulase-negative staphylococci (CNS) from blood cultures, but the overall frequency of positive blood cultures did not decline and there was a rise in frequency of isolation of Gram-negative bacterial species. The decline in frequency of CNS isolates from blood cultures may have resulted from a reduction in the level of cross-colonization of neonates with antibiotic-resistant CNS strains and also increased usage of erythromycin and vancomycin.
Giant anterior circulation aneurysms and some basilar aneurysms can cause problems due to their size, the presence of clot in the aneurysm and the difficulty of applying a clip without kinking the perforating vessels. By utilising cardiopulmonary bypass via the femoro-femoral perforating vessels. By utilising cardiopulmonary bypass via the femoro-femoral route the patient can be cooled to below 20 degrees C allowing the circulation to be stopped for up to 3/4 hour. This will enable the neurosurgeon to unhurriedly dissect out the aneurysm without fear of rupture and where necessary open the aneurysm to remove clot and clip the aneurysm. By draining the circulating volume into the venous reservoir of the pump, a large aneurysm may collapse thus enabling it to be clipped more easily. It is, therefore, a useful technique for difficult aneurysms. We present here a series of 11 patients who underwent this procedure with excellent results in 7. All patients had aneurysms which would otherwise have been either inoperable or very risky to tackle.
New 2-site labeled monoclonal antibody techniques were used to measure serially plasma levels of brain-type creatine kinase (CK-BB), heart-type creatine kinase (CK-MB) and muscle-type creatine kinase (CK-MM) during a 20-hour postoperative period in 24 infants after deep hypothermia and total circulatory arrest used in pediatric cardiac surgery. A control group of 7 children undergoing cardiovascular procedures without extracorporeal circulation or circulatory arrest also were studied. There were marked increases in CK-MB and CK-BB levels in the circulatory arrest group but not in the closed group. CK-BB increased from 3.2 +/- 0.5 to 27 +/- 10 ng/ml and CK-MB from 5.9 +/- 2.1 to 137 +/- 12 ng/ml. The CK-MM concentrations increased from 299 +/- 91 and 194 +/- 49 ng/ml to 1,220 +/- 274 and 1,322 +/- 142 ng/ml in the closed and circulatory arrest groups, respectively. Peak levels of CK-MB and CK-BB occurred an average of 133 and 127 minutes, respectively, after reperfusion. The half-time of CK-BB differed significantly from that of CK-MB (149 +/- 15 vs 359 +/- 20 minutes). The arrest time had a more marked effect on CK-BB concentration than on CK-MB and CK-MM concentrations. Arteriointernal jugular venous concentration differences were consistently negative for CK-BB in the circulatory arrest group, but not for CK-MM and CK-MB.(ABSTRACT TRUNCATED AT 250 WORDS)
Hearts from 29 children with complete transposition were examined postmortem to analyze the pattern of the coronary arteries and the origin of the sinus node artery. Four patterns of origin and distribution of the coronary arteries were found. In all, the coronary arteries arose from one or both of the facing sinuses, which were termed sinus 1 and sinus 2. The patterns were left coronary artery from sinus 1 and right coronary artery from sinus 2; anterior descending artery from sinus 1 and right coronary artery and circumflex artery from sinus 2; left coronary and right coronary from sinus 2; and circumflex artery from sinus 2 and right coronary artery from sinus 1. This study highlights the patterns of the coronary arteries in complete transposition and categorizes the variability using a simple classification. It also emphasizes the surgical significance of variations in distribution of the sinus node artery.
The results of surgical repair of ostium primum atrioventricular septal defect show continued improvement. This improvement reflects the advances in open-heart surgery in general and, in particular, the better understanding of the anatomy of the conduction tissue and the morphology and function of the left atrioventricular valve. We have corrected this defect in 84 patients over a ten-year period. There were 2 early deaths (2.4%) and 2 late deaths (2.4%). Two patients had problems related to conduction. Our surgical approach has been to place the interatrial baffle in such a way as to avoid the displaced atrioventricular node and thereby leave the coronary sinus in the left atrium. Our approach to repair of the so-called cleft in the left atrioventricular valve (in reality the space between the ventricular components of the bridging leaflets), is based on the unequivocal triple-leaflet morphology of this valve.
Seven patients with double-outlet right ventricle and subpulmonary ventricular septal defect (the Taussig-Bing anomaly) underwent anatomical repair at the arterial level with transfer of the coronary arteries. At the time of operation, patient ages ranged from 6 weeks to 33 months (mean 14.1 months) and weight ranged from 3.7 to 11.5 kg (mean 7.0 kg). Four patients had prior pulmonary artery banding: Two of these four also had coarctation repairs, and one had a Blalock-Hanlon septectomy. Three different patterns of coronary artery distribution were encountered. Five patients had side-to-side great arteries, and two had more or less anteroposterior great arterial relationships. There was one operative death (14.3%: 70% confidence limits 1.9 - 40.7%) resulting from muscular subvalvular right ventricular outflow tract obstruction (RVOTO). There have been no late deaths in the six survivors followed 6 to 31 months postoperatively (mean 14.8 months). One patient required closure of a residual ventricular septal defect (VSD) and infundibular resection for RVOTO 4 months postoperatively. All other survivors are functionally NYHA Class I. Five of the six survivors have undergone postoperative catheterization (mean interval 5.8 months). There was no aortic insufficiency and good ventricular function in all patients. In addition to the patient with the residual VSD, two other asymptomatic patients had mild or moderate RVOTO. Compared with alternative surgical procedures for this anomaly, anatomic correction has the advantages of acceptable operative mortality, use of the left ventricle as the systemic ventricle, no need for extracardiac conduits, and applicability to patients with all variations of coronary artery and great artery anatomy.
Profound hypothermia and circulatory arrest in infants and children undergoing cardiac surgery were followed by abnormally high plasma levels of creatine kinase isoenzyme BB (CK-BB). Differences in the levels of enzymes in the femoral arterial and jugular venous blood indicated that the origin of the additional enzyme was the brain. The extent of the rise in enzyme levels was related to the duration of circulatory arrest. These data suggest that measurements of the CK-BB enzyme in plasma provide quantitative information about cerebral damage during cardiac surgery.
Thirty three patients were followed up after anatomical correction of transposition of the great arteries or double outlet right ventricle and subpulmonary ventricular septal defect (Taussig-Bing anomaly). There were no late deaths and clinical progress was excellent. Cardiac catheterisation was performed in 17 patients two weeks to 44 months after operation. There were six patients with simple transposition, six with complete transposition and large ventricular septal defect, and five with the Taussig-Bing anomaly. Pressure gradients across the right ventricular outflow tract ranged from 5 to 72 mm Hg, being greater than 40 mm Hg in five patients. No patient was shown to have important valvar regurgitation and in 15 patients the coronary anastomoses were widely patent. Left ventricular function was assessed from digitised ventriculograms. Ventricular volume, shape, and ejection fraction were all normal for the group although patients with complex transposition showed a significantly lower mean (SD) ejection fraction than those with simple transposition (62(9) vs 77(9]. Analysis of regional wall motion showed a totally normal pattern in four patients; however, in seven patients a characteristic abnormality of anterior hypokinesis with delayed onset of inward wall motion was seen. Anatomical correction of transposition of the great arteries and the Taussig-Bing anomaly can be performed with satisfactory anatomical and functional results. The implications of the left ventricular wall motion abnormalities is unknown.
Following open heart surgery using cardiopulmonary bypass, 18 (4%) of 441 operated children required treatment with peritoneal dialysis for acute renal failure or refractory oliguria. Nine recovered renal function (50%) and 5 (28%) survived. Only 2 died from renal causes. Despite both adequate symptomatic treatment of renal failure and few complications of dialysis itself, the prognosis remains poor, even with early treatment, but an aggressive approach is justified because some survivors can be expected.
Seven postmortem hearts from children who underwent anatomical correction for complete transposition (6 cases) and double outlet right ventricle (1 case) were examined in order to gain a better understanding of the pathological morphology in relation to the surgical procedure. Associated malformations were ventricular septal defect in all, patient arterial duct in 4 and aortic coarctation in 3. In all hearts the coronary arteries arose from the facing aortic sinuses and were relocated into the facing sinuses of the pulmonary trunk with minimal displacement. The orifices of the coronary arteries were reduced in caliber in one case. The sinus node artery arising from a lateral atrial artery was transected in one case. The Lecompte maneuver was performed in 4 cases and tension on the pulmonary trunk was observed in 2 cases. Potential right ventricular outflow tract obstruction was evident in all (including one in which it had been resected during surgery). The substrates of obstruction were anomalous parietal insertion of the outlet septum (2 cases), anomalous muscular trabeculation (1 case), rightward displacement of the outlet septum (1 case), parietal insertion of the outlet septum (1 case) and restrictive muscular infundibulum (1 case--DORV). In addition, all the complete transposition cases had hypertrophy of the parietal right ventricular wall. This study highlighted the anatomical features which could lead to poor results in the anatomical correction procedure. Some of the potential problems could be avoided by minor modifications to the technique.
We present details of 15 children, aged 3 months to 11 years, with discrete sub-aortic stenosis and ventricular septal defect. We emphasise a high index of clinical suspicion and echocardiography as the best means of diagnosing this dangerous combination. Physical signs were those of ventricular septal defect in all patients, with auscultatory evidence of additional sub-aortic stenosis in only one. Five patients had a short early diastolic murmur of mild aortic incompetence. The electrocardiograph showed isolated left ventricular hypertrophy in eight patients. Cardiac catheterisation and angiography identified the ventricular septal defect in all cases but detected the sub-aortic stenosis in only eight. Cross sectional echocardiography showed both lesions in all 11 patients to whom it was available.
Between January, 1976, and June, 1983, 16 patients with transposition of the great arteries and fixed subpulmonary obstruction underwent repair by means of a combined Mustard procedure and placement of a conduit between the left ventricle and main pulmonary artery. Their mean age and weight were 5.3 years and 19 kg. Ten patients had an intact ventricular septum and six had a ventricular septal defect, which because of its size or location precluded a Rastelli repair. A fibromuscular tunnel was the most common type of subpulmonary obstruction (10/16, 62%). There were three early deaths and one late death. Early cardiac catheterization (mean interval from operation, 45 days), performed in 10 of 13 survivors, showed a significant decrease in the mean left ventricular-pulmonary arterial gradient from 66 to 8.5 mm Hg. Late cardiac catheterization (mean interval from operation, 4 years) showed continuing relief of the subpulmonary obstruction with a mean increase in peak systolic transconduit pressure gradient of 6.4 mm Hg. This procedure is an option to direct relief in the management of severe fixed subpulmonary obstruction in patients with transposition and intact ventricular septum or in the presence of a ventricular septal defect when a Rastelli repair is precluded.
Between February, 1981, and December, 1984, 30 patients underwent anatomic correction of transposition of the great arteries with intact ventricular septum (n = 8), transposition with ventricular septal defect (n = 15), and double-outlet right ventricle with subpulmonary ventricular septal defect, the Taussig-Bing anomaly (n = 7). At operation, ages ranged from 18 hours to 6 years (mean 11.3 months) and weights ranged from 2.6 to 16.4 kg (mean 6.1 kg). The group with transposition and intact ventricular septum on average was younger (mean 1.2 months) and smaller (mean 3.5 kg) than the other two groups. Associated congenital heart defects were seen in 12 patients, including five with coarctation, three with multiple ventricular septal defects, two with right ventricular hypoplasia, two with juxtaposed atrial appendages, and one each with interrupted aortic arch, Wolff-Parkinson-White syndrome, and left ventricular outflow tract obstruction. All 10 patients who had undergone prior palliative operations had pulmonary artery banding. In addition, four of these patients had coarctation repairs, four had atrial septectomy, and one had systemic/pulmonary shunting. All recognized patterns of coronary anatomy were encountered. The aorta and pulmonary artery were side by side in 14 patients and anteroposterior in 16 patients. The Lecompte maneuver to establish right ventricular-pulmonary arterial continuity was successfully used in 12 of 13 patients with anteroposterior great vessels but in none of those with side-by-side arteries. Seven patients had subvalvular right ventricular outflow tract obstruction, recognized either at operation (five) or postoperatively (two). This was responsible for death in three patients. The 30 day hospital mortalities were as follows: one death (12.5%) in the group with transposition and intact ventricular septum, six deaths (40%) in the group with transposition plus ventricular septal defect, and one death (14.3%) among patients with double-outlet right ventricle and subpulmonary ventricular septal defect; the overall mortality was eight deaths (26.7%). There have been no late deaths (mean follow-up 17.2 months). Ninety-five percent of the survivors are in New York Heart Association Functional Class I. Postoperative catheterization in 13 patients has shown normal left ventricular function, no coronary stenosis, and no aortic incompetence. Sixty-nine percent of these patients had clinically unsuspected gradients across the right ventricular outflow tract, which may be prevented by avoiding the Lecompte maneuver or the use of conduits.
Malalignment of the interatrial and interventricular septal structures is rarely seen associated with atrioventricular septal defects. Such malalignment gives rise to an anomalous disposition of the conduction tissue specifically in regard to the siting of the atrioventricular node. Recognition of this abnormality prior to (or at) surgery is important if post-operative rhythm problems are to be avoided.