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Objectives: The National Blood Service issues 2.2 million units of blood per year, 10% of these (220000) are utilized in cardiac procedures. Transfusion reactions, infection risk and cost should stimulate us to decrease this transfusion rate. We test the efficacy of autotransfusion following surgery in a prospective randomized trial., Methods: One hundred and twelve patients undergoing CABG, valve or CABG + valve procedures were randomized into two groups. Group A received washed postoperative drainage fluid and group C were controls. The indication for transfusion was a postoperative haemoglobin (Hb) < 10 g/l or a PCV < 30. There was no significant difference in preoperative and operative variables between the groups., Results: Twenty-eight patients in group A and 46 in group C required homologous transfusion (P = 0.0008). Group A patients required 298+/-49 ml of banked blood per patient, group C 508+/-49 ml (P = 0.003). There was no difference in total blood required (volume autotransfused + volume banked blood transfused) between the groups (group A 404+/-50 ml, group C 508+/-50 ml) or in mean total mediastinal fluid drainage (group A 652+/-51 ml, group C 686+/-50ml). The mean Hb concentration was significantly higher in group A on day 1 (11.2 g/dl+/-51 vs. 10.6 g/dl+/-13 (P = 0.002)). No morbidity was associated with autotransfusion., Conclusion: Autotransfusion can decrease the amount of homologous blood transfused following cardiac surgery. This represents a benefit to the patient and a decrease in cost to the health service.

Objective: Following corrective cardiac surgery in infants and children for congenital heart disease, a persistent low cardiac output refractory to conventional modes of treatment is associated with a mortality approaching 100%. We advocate the use of whole body hypothermia to reduce tissue oxygen demand and provide a degree of cellular protection against ischaemia allowing time for recovery. We describe our experience., Methods: Between July 1986 and December 1995, 1885 infants and children underwent surgery (operative mortality, 6%), 1302 requiring cardiopulmonary bypass. Fifty-seven patients had a persistent low cardiac output, impaired respiratory function, decreased urine output and acidosis despite maximal intensive care treatment. Cooling to 32-33 degrees C was therefore started using a thermostatically controlled water filled cooling blanket., Results: Following cooling, there was a fall in heart rate (P<0.001), a rise in mean arterial pressure (P<0.001) and a fall in mean atrial pressure (P<0.001). Significant (P<0.001) increases in pH and urine output were also recorded. Thirty-one (54%) of the 57 patients treated with cooling survived to leave hospital. No long-term sequelae have been noted in these patients., Conclusion: Induced hypothermia is a useful salvage treatment, in children following corrective cardiac surgery when all conventional treatment has been tried and failed.

Background: Left untreated, severe mitral regurgitation in asymptomatic patients can lead to irreversible cardiac damage, which can develop with little warning. Over the period of this study, we have tended to operate earlier in the disease process and on less symptomatic patients. We report here our experience., Methods: Between January 1985 and June 1996, 710 patients with mitral regurgitation underwent operations. Three hundred twenty-nine (213 male and 116 female with a mean age of 65.5 years) had degenerative mitral valve disease and of this group, 169 patients underwent repair and 160, replacement., Results: The overall operative mortality was 4 patients (1.2%). There were no operative deaths among patients having isolated mitral valve repair. Survival at 1 year, 5 years, and 10 years was 94%+/-1.4% (+/- the standard error of the mean), 77%+/-2.9%, and 41%+/-5.8%, respectively. Survival was significantly better in the group having repair (p < 0.05). Ten patients (6%) in the repair group and 13 (8%) in the replacement group required reoperation. Increased age, worse left ventricular function, type of operation (replacement worse than repair), and increased left ventricular size were significantly associated with poorer survival., Conclusions: These data confirm the superior results achieved with mitral valve repair and support early mitral valve repair before functional deterioration.

Postoperative low cardiac output states are a major cause of postoperative mortality in infants and children following corrective cardiac surgery for congenital heart defects. In this unit, whole body hypothermia has been used since 1979 in the management of these low output states when they are refractory to conventional modes of therapy. Twenty cases treated in this way between July 1986 and June 1990 were reviewed in 1992. The current report reviews the 50 further cases treated with moderate hypothermia between July 1990 and December 1995. The median (range) age of patients was 8 months (0 days-16 years) with a median weight of 4.1 kg (2.5-33 kg). Following cooling, there was a decrease in heart rate (p < 0.001), an increase in mean arterial pressure (p < 0.001) and a decrease in mean atrial pressure (p < 0.001). Significant increases in pH and urine output were also noticed, the increase in urine output being greater in the surviving group (p = 0.02). A decrease in platelet count occurred (p < 0.001) but white blood cell count remained unchanged (p = 0.18). Twenty-five of the 50 patients survived to leave hospital. Induced hypothermia does not appear to be associated with any complications and after the failure of all conventional treatment, it seems likely that the technique may have been beneficial to outcome in some patients.

To evaluate the late effects of chronic pulmonary regurgitation against the putative benefits from the current surgical trend of primary repair of tetralogy of Fallot with a transannular patch in infancy, 10 patients > 10 years after early primary repair and 7 matched normal controls underwent exercise stress test and cine magnetic resonance imaging assessment of ventricular functions. Right ventricular impaired diastolic function and decreased exercise capacity, both significantly associated with pulmonary regurgitation in patients, indicated that early primary repair of tetralogy may not prevent late ventricular dysfunction and diminished exercise performance if chronic regurgitation results from right ventricular outflow tract reconstruction.

Background: Although the use of extracorporeal life support (ECLS) following repair of congenital heart defects in children is increasing, the criteria for ECLS usage in these patients is not well defined. The overall survival of such patients is disappointingly low and may depend on both the indication for support and the time at which ECLS is commenced., Methods: Between January 1993 and December 1996, 727 children underwent surgery for congenital heart defects at our institution with an overall hospital mortality of 5.8% (42 children). Nine of these children were treated with ECLS postoperatively. There were seven males and two females with a mean age of 7.2 months (range 2 weeks-3 years). Seven children could not be weaned from cardiopulmonary bypass (CPB) in the operating theatre. A further two were treated with ECLS later on during the postoperative period (commenced at 14 and 48 h). Full veno-arterial extra corporeal membrane oxygenation (ECMO) support was used in all children except one in whom a left ventricular assist device (LVAD) was used., Results: The median duration of support was 121 h (range 15-648 h). Four children (44%) were weaned from support and two of these are long-term survivors. Of the seven children in whom ECLS was instituted because of failure to wean from CPB, there was one long term survivor (LVAD support). Of the two patients in whom ECLS was instituted during the post-operative period there is one long-term survivor., Conclusions: Weaning form ECLS and decannulation in 44% of our patients is comparable to other series of post-cardiotomy patients requiring ECLS. However, full veno-arterial ECMO instituted because of a failure to wean from CPB during corrective surgery is associated with an extremely poor outcome (zero long-term survivors in six patients).

Objective: We proposed that angiotensin converting enzyme (ACE) inhibitor therapy would alter systemic vascular resistance (SVR) during rewarming and increase the requirement for vasoactive drugs in the immediate post-bypass period., Methods: Sixty-five sequential adult patients undergoing cardiac surgical procedures requiring hypothermic (28 degrees C) cardiopulmonary bypass (CPB) were recruited. Sixty-two fitted the inclusion criteria of which 21 were receiving ACE inhibitors prior to surgery. SVR was calculated at 1 min intervals during the rewarming phase of hypothermic CPB. The use of vasoactive drugs during and immediately after termination of CPB was recorded. The doctor administering these drugs was unaware of the nature of the study., Results: Mean SVR in the ACE group was 978 dyne/s per cm5 and in the control group was 1194 dyne/s per cm5 (P = 0.006). Mean arterial pressure was 48.8 mmHg in the ACE group and 56.3 mmHg in the control group (P = 0.004). There was a significant difference in vasoactive drug requirements between the groups (P < 0.01). There was no statistically significant difference in age, weight, body mass index, body surface area, theatre temperature, core temperature at which rewarming started, rate and time of rewarming, haematocrit on bypass or preoperative left ventricular function., Conclusion: Preoperative ACE inhibitor therapy decreases SVR during the rewarming phase of CPB and increases post-bypass vasoactive drug requirements.

Objective: An attempt was made to find out how many operations were performed in Europe in 1995 for congenital heart disease., Methods: Representatives from the 20 most active European countries which could supply reliable information were contacted. They were asked to contact all surgeons performing significant numbers of operations for congenital heart disease in their country. The numbers of operations on children under and over 1 year operated on with and without cardiopulmonary bypass were collected. No attempt was made to collect more detailed information such as operative mortality as it was felt that many surgeons would be reluctant to give their results and the data would be incomplete., Results: A total of 27,976 operations were recorded, of which 20,318 were performed with cardiopulmonary bypass and 7658 without. The total population of the 20 European countries was 448 million and the mean number of operations performed with cardiopulmonary bypass per million was 45.4. However, the rate varied from 9.1 to 70.1. The percentage of operations on cardiopulmonary bypass performed in the first year of life varied from 9.4% to 44.4%., Conclusions: The total of nearly 28,000 operations in 1995 is probably an underestimate, but it has been difficult to collect this data at all. Obviously many smaller countries have not been included, and some older patients undergoing operations such as atrial septal defect closure in adult units have been missed. The number of open heart operations per million vary in different countries and this presumably represents differing referral patterns. If their numbers are low in some countries, these results may be helpful in persuading their governments that more resources are needed for congenital heart surgery. It is also interesting to see the variation in the number of operations performed in the first year of life. Those countries with a lower rate may have a backlog of older patients who were previously palliated, or their surgeons may still prefer initial palliation and later correction in some conditions rather than early correction. It is hoped that with better prospective data collection, an assessment can be repeated in 2000.

Objective: Neurologic sequelae remain a well recognised complication of paediatric cardiac surgery. Monitoring of cerebral oxygenation may be a useful technique for identifying vulnerable periods for the development of neurologic injury. We sought to measure regional cerebral oxygenation in children undergoing cardiac surgery using near infrared spectroscopy to ascertain such vulnerable periods., Methods: Observational study of 18 children (median age 1.3 years) undergoing cardiac surgery (17 with cardiopulmonary bypass, 8 with circulatory arrest). Regional cerebral oxygenation was monitored using the INVOS 3100 cerebral oximeter and related to haemodynamic parameters at each stage of the procedure., Results: Prior to the onset of bypass, 10 patients had a decrease in regional cerebral oxygenation of > or = 15% points, reaching an absolute haemoglobin saturation less than 35% in 5 cases. The most common cause was handling and dissection around the heart prior to and during caval cannulation. With institution of bypass, regional cerebral oxygenation increased by a mean 18% points to a mean maximum of 75%. During circulatory arrest regional cerebral oxygenation decreased with rate of decay influenced by temperature at onset of arrest (0.25%/min at < 20 degrees C; 2%/min at > 20 degrees C). Reperfusion caused an immediate increase in regional cerebral oxygenation followed by a decrease during rewarming. Discontinuation of bypass caused a precipitous decrease in regional cerebral oxygenation in 5 patients, reaching less than 50% in 3 patients., Conclusions: These observations suggest that the pre- and early post-bypass periods are vulnerable times for provision of adequate cerebral oxygenation. Near infrared spectroscopy is a promising tool for monitoring O2 supply/demand relationships especially during circulatory arrest.

Objective: The value of coronary artery bypass grafting (CABG) at the time of repair of a post-infarct ventricular septal defect (VSD) remains controversial. The aim of this study was to analyse the effect of CABG on early mortality and survival following repair of an acquired VSD., Methods: Over 23 years, 179 patients, 118 male, 61 female, mean age 66 years (range 43-80), have undergone repair of a post-related VSD in our unit. A total of 29 patients, who predominantly form the earlier part of the series, were operated on greater than 1 month after the infarct and are, therefore, excluded. Coronary angiography was performed in 98 (65.3%) of the remaining 150 patients. Of these, 41 had coronary artery disease (CAD) limited to the infarct-related vessel and 57 had additional significant CAD. Those with CAD limited to the infarct-related vessel were not grafted (Group A). Of those, 40 with significant CAD underwent CABG at the time of VSD repair (Group B) and 17 did not (Group C). In 52 patients the coronary anatomy was not documented (Group D). Risk factors for early mortality were evaluated using logistic regression. Actuarial survival was compared using log rank and Wilcoxon tests. Cox's proportional hazards method was used to determine factors affecting survival., Results: Overall, 30 day mortality was 32%. CABG did not significantly decrease operative mortality (logistic regression). There was no statistically significant difference in early mortality or actuarial survival between the four groups. CABG was not associated with an increased survival (Cox's method)., Conclusions: Concomitant CABG at the time of VSD repair does not affect early mortality nor confer survival benefits. There seems to be no demonstrable benefit in revascularisation at the time of repair and, therefore, it may be unnecessary to perform CABG or coronary angiography in these patients.

We present the case of a 19-year-old student who underwent correction of a pectus excavatum deformity using a pectus bar. At least 6 months following surgery, one end of the bar had migrated into his right ventricle, across the interventricular septum, to lie with its free end in the left ventricular cavity. This acted as a source of thrombus formation and lead to several systemic embolic events. The patient made a full recovery after removal of the bar. A review of the literature demonstrates that this has not been reported before.

Objective: As the population continues to age, older patients are being referred for repair of acquired ventricular septal defect (VSD) following myocardial infarction (MI). The purpose of this study was to assess the effect of age (> or = 70 years) on operative risk and long term survival following repair of an acquired VSD., Methods: Between January 1972 and December 1995, 179 patients have undergone repair of acquired VSDs following MI in our unit. There were 118 males and 61 females (age range 43-80 years) of whom 60 were aged 70 years or above., Results: The overall early mortality was 27%. On univariate analysis risk factors for early death included shorter time from both MI and detection of murmur to operation (P < 0.01, P = 0.04), site of MI (P < 0.01), higher NYHA class (P < 0.01), lower preoperative blood pressure (P < 0.01) and longer cardiopulmonary bypass and cross clamp times (P < 0.01, P = 0.03). Non significant variables included age, sex, concomitant CABG and preoperative renal function. Early mortality was 28.6% (34/119) in patients under 70 and 25.0% (15/60) in those over 70. This difference was not significant. The only significant differences between the age groups were sex distribution (females > males, P < 0.01), in the older group, and shorter time from both MI and detection of murmur to operation (P = 0.04, P = 0.02). Cardiopulmonary bypass was the only statistically significant variable on multivariate analysis (P = 0.01)., Conclusions: There was no significant difference in early mortality between the two age groups. As shorter times from both MI and detection of murmur to operation adversely affect early mortality, age over 70 years should not be used to determine suitability for surgery.

Objective: To assess the value of necropsy examination in patients dying soon after cardiac surgery, particularly the proportion of clinical questions answered by the necropsy, the frequency of major unexpected findings, and the limitations of the procedure., Design: A three year prospective study of necropsy examinations in adult patients dying before discharge or within 30 days of cardiac surgery performed under cardiopulmonary bypass in one hospital., Setting: Tertiary referral centre., Results: 123 of 2781 patients (4.4%) died in the early postoperative period, and necropsy examination was performed in 108 of these (88%). The mortality after emergency procedures (18%) was much higher than after routine operations (2.6%). The main causes of death were cardiac failure (52%), haemorrhage (14%), cerebral disease (6%), and pulmonary emboli (5%). The necropsy changed the stated cause of death in 16 patients (15%), and answered clinical questions in 24 of 38 patients. In 15 patients necropsy examination did not provide a full explanation of death. Most of these patients died of cardiac failure soon after surgery or were sudden unexpected deaths., Conclusions: Necropsy examination in patients dying early after cardiac surgery is valuable as it answers the majority of clinical questions, and shows unexpected findings in a significant proportion of cases.

A persistent distal false lumen (PDFL) after surgical repair of type A aortic dissection is the most important factor in determining long-term survival. It has been suggested that changes in surgical technique reduce the incidence of distal false lumen. We report the findings of a 20-year follow-up (mean 5.2 years) on 87 patients who have undergone surgical repair of type A aortic dissection with all survivors undergoing magnetic resonance (MR) scanning of the entire aorta. Early mortality was 27.5%, and actuarial 5-, 10-, and 15-year survival was 65%, 28% and 20% respectively. Early mortality had decreased to 18% in the last 5 years. The most common cause of late death was related to distal aortic disease, accounting for 47% of all late deaths with a peak incidence at 7-10 years after surgery. The incidence of PDFL in survivors was 72%, despite the fact that 82% of all intimal tears were resected at time of operation. Incidence was not affected by extension of the repair into the aortic arch nor by the use of the open technique or Gelatin-Resorcine-Formal tissue glue. In patients with a distal false lumen 6% had reached a maximum aortic diameter of 6 cm in at least one plane on MR scanning and 25% had reached 5 cm. We conclude that if dissection has extended beyond the arch at time of presentation then the choice of surgical technique does not prevent the persistance of a distal false lumen. MR scanning gives ideal anatomical and functional assessment of distal aortic disease and provides the surgeon with all the necessary information to plan the timing and indications for further surgery.

Background: Conservative surgical options in the treatment of congenital aortic stenosis are limited. To relieve the obstruction necessitates full incision of the raphe of the larger valve leaflet, but this inevitably causes prolapse., Methods: We performed aortic valve repair in 6 children, aged 14 months to 17 years, with congenital aortic stenosis, 2 having had aortic valvotomy as infants. The repair consisted of suturing the base of a triangular piece of bovine pericardium, with a simple vertical fold, to the free edges of the incised raphe. The pericardial fold was then sutured vertically to the aortic wall., Results: At follow-up of 2 to 60 months, the mean peak systolic Doppler gradients had decreased from 80 +/- 15 mm Hg to 26 +/- 9 mm Hg. The effective valvular orifice area increased from 33% +/- 6% to 64% +/- 3%, allowing blood flow to increase by a factor of 3.76. Two patients have mild and 2 have mild-to-moderate aortic regurgitation., Conclusions: The described conservative repair renders the valve tricuspid and trisinusoidal, and the deficient interleaflet triangle is recreated, preventing cusp prolapse. Longer follow-up is required to assess the durability of unstented pericardium in the aortic position, but the early results are encouraging.

Background: Complete repair of infants with interrupted arch and ventricular septal defect through a midline incision has been the preferred method for more than 20 years. End-to-end anastomosis can result in restenosis if there is excess tension. Two methods of reducing this tension have been described, and the subsequent growth of the new aortic arch is demonstrated., Methods: In 2 infants (5 and 9 months old) the duct was used to create a new aortic arch. In 3 other younger infants the left carotid artery was divided, turned down, and anastomosed to the descending aorta to form the new arch. These operations were performed through the midline at the same time as the ventricular septal defect was closed., Results: All 5 patients are well now 8 to 19 years postoperatively. One patient required reoperation for stenosis at the anastomotic site, but all have subsequently shown good growth on follow-up angiographic and magnetic resonance imaging studies., Conclusions: Although end-to-end repair is best, these alternative methods have shown very satisfactory aortic growth into adult life.

A variety of approaches and surgical techniques have been described for the management of recurrent coarctation. When there is an additional intracardiac defect that requires surgical correction it is preferable to correct both lesions simultaneously and through the same incision. This article reports two new techniques of connecting ascending to descending aorta using an intrathoracic conduit and performed through a median sternotomy.

Objective: To define the lowest age at which the bidirectional superior cavopulmonary anastomosis can safely be used in infants with complex congenital heart defects., Design: A retrospective analysis of clinical, echocardiographic, haemodynamic, and angiographic data in four consecutive patients undergoing bidirectional superior cavopulmonary anastomosis below the age of 2 months., Patients: Between November 1990 and September 1993, four infants less than 8 weeks of age (3, 4, 6, and 7 weeks) underwent bidirectional superior cavopulmonary anastomosis as a primary palliation for complex heart disease. The indication for early surgical intervention was progression of cyanosis (n = 3) and high pulmonary blood flow causing heart failure (n = 1). In two infants with tricuspid atresia, surgery was performed through a right thoracotomy without the use of cardiopulmonary bypass. In one infant with double inlet left ventricle and discordant ventriculoarterial connection, cavopulmonary anastomosis was combined with an arterial switch procedure. The final infant had double inlet left ventricle with pulmonary atresia; the central pulmonary arteries were virtually discontinuous and each branch was supplied by a separate arterial duct. The central pulmonary arteries were reconstructed using the subaortic innominate vein. Temporary prostacyclin infusion was used in three patients in the immediate postoperative period., Results: Early postoperative extubation (5, 7, and 48 h) was successful in three patients. The youngest child required ligation of the ductus arteriosus four days later because of severe upper body oedema. The postoperative course was complicated by prolonged effusions in two patients. All were alive and well 14-48 months postoperatively, with satisfactory systemic saturations (80-87%) and haemodynamic indices., Conclusions: This limited experience challenges the widely held belief that the bidirectional superior cavopulmonary anastomosis cannot be used as a primary palliation for complex heart disease in early infancy. A wider experience is required to determine the safety and indications for this approach.

Objective: Antibiotic-sterilised homograft valves stored at 4 degrees C have been implanted in the subcoronary position in this unit since 1973. This study was undertaken in order to assess the long-term function of these valves., Methods: All 249 patients undergoing homograft aortic valve replacement (AVR) at the Wessex Cardiothoracic Centre between April 1973 and December 1994 were studied. Homograft valve sizes ranged from 15 mm to 28 mm internal diameter, 202 (81.1%) varying between 18 mm and 22 mm. The mean patient follow-up was 12.4 years with a total follow-up of 3096 patient-years. There were six early deaths (2.4%)., Results: On actuarial analysis, survival was 78.5+/-2.7% (1SE) at 10 years, 65.7+/-3.3% at 15 years and 55.0+/-3.9% at 20 years. The freedom from redo AVR was 87.9+/-2.4% at 10 years, 71.7 +/-3.8% at 15 years and 49.7+/-5.6% at 20 years. The freedom from structural degeneration was 85.6+/-2.5% at 10 years, 63.6+/-4.0% at 15 years and 41.9+/-6.4% at 20 years. On multivariate analysis the risk of valve failure was significantly higher in younger patients (P<0.0001) and in those who underwent aortic root tailoring (P = 0.024). The freedom from endocarditis was 98.4+/-0.9% at 10 years, 96.2+/-1.6% at 15 years and 95.1+/-1.9% at 20 years. Of the 249 patients, 218 had an isolated homograft AVR and were not anticoagulated. In this group there were two possible thromboembolic events., Conclusion: As well as the established haemodynamic benefits, this study has shown that homograft AVR with antibiotic-sterilised 4 degrees C stored homograft valves implanted in the subcoronary position, offers good long-term results.

Right-sided BSCA provides for satisfactory pulmonary arterial growth in infants and children with complex congenital heart defects, and it could enhance the growth of a small right pulmonary artery. The growth of the left pulmonary artery, particularly in younger patients, needs close attention to confirm the safe role of BSCA in long-term palliation.

Background: It is common practice to interrupt all alternative sources of pulmonary blood flow ("competitive flow") at the time of a bidirectional superior cavopulmonary anastomosis (BCPA), although the merits of this have not been systematically studied., Methods and Results: We reviewed the early and medium-term clinical and hemodynamic findings in 108 consecutive patients 3 weeks to 25 years old (median, 1.9 years) undergoing BCPA at one of three institutions. Preoperatively, pulmonary blood flow was dependent on antegrade ventricular flow (n = 50), systemic-to-pulmonary shunts (n = 33), or mixed sources (n = 25). Postoperatively, competitive sources of pulmonary blood flow were left patent in 43 of 108 patients (40%). There were four early (3.7%) and four late deaths, none related to persistence of competitive flow. After BCPA, patients with competitive flow had significantly higher systemic oxygen saturations at 1 hour (85% versus 79%), 24 hours (84% versus 78%), and at hospital discharge (84% versus 78%) and required a shorter period of artificial ventilation (median, 9 versus 24 hours) and intensive care (median, 2 versus 4 days). Oxygen saturations at late follow-up (median, 2.8 years; range, 1 to 7) did not differ (83% versus 82%). No patient developed pulmonary arteriovenous malformations., Conclusions: Competitive flow is well tolerated in the short and medium term after BCPA, and early postoperative systemic oxygen saturations are improved. The long-term influence of competitive flow on pulmonary arterial growth, arteriovenous malformation development, and ventricular function warrants investigation.

Between 1975 and 1979, 443 biological valves (298 Carpentier-Edwards, 134 homograft, and 11 Hancock valves) were implanted in 415 patients (age, 16 to 77 years; mean, 59 years) with an operative mortality of 2.9%. Total follow-up was 4,248 patient-years. Overall event-free survival was 60% +/- 1.5% (standard deviation) at 10 years and 29% +/- 1.4% at 15 years. Ten-year and 15-year event-free survival were 72% +/- 3.4% and 41% +/- 3.3% for aortic homografts, 62% +/- 3% and 33% +/- 2.8% for isolated aortic xenografts, and 43% +/- 3.5% and 14% +/- 3.0% for isolated mitral xenografts. Freedom from structural valve degeneration was 87% +/- 1.3% and 63% +/- 2.5% for all patients at 10 and 15 years, respectively, 86% +/- 2.7% and 58% +/- 4.1% for aortic homografts, 93% +/- 1.8% and 76% +/- 5.1% for aortic xenografts, and 75% +/- 4.0% and 47% +/- 7.4% for mitral xenografts. Of the 110 remaining patients, echocardiography was performed in 61 patients (23 aortic xenograft, 24 aortic homograft, 9 mitral xenograft, and 5 tricuspid xenograft) between 14 and 17 years after implantation. An early diastolic murmur was heard in 57% of all aortic valve replacements (AVRs) 62.5% of homograft AVRs, and 52% of xenograft AVRs. Echocardiographically, aortic regurgitation was detected in 79%, 83%, and 74% of all AVRs, homografts, and xenografts, respectively. Aortic stenosis was present clinically in 11% of all AVRs, 4% of homograft AVRs, and 17% of xenograft AVRs.(ABSTRACT TRUNCATED AT 250 WORDS)

A 14-month-old child underwent routine transcatheter closure of her patent ductus arteriosus (PDA) using the Rashkind double-umbrella device. She developed severe persistent hemolysis in association with residual ductal flow. The problem was managed by the previously unreported approach of standard surgical ligation of the duct via a left thoracotomy, leaving the occlusion device in situ.

It has been widely stated that pulmonary artery banding (PAB) is contraindicated in the setting of double inlet left ventricle with transposition of the great vessels (DILV/TGA), especially if aortic arch obstruction is present. We postulated that the poor results for this condition reflect the tendency to leave the band in place long-term without early recognition and relief of subaortic stenosis (SAS). Short-term PAB with early relief of SAS remains an attractive option compared to a neonatal "Norwood" strategy. We reviewed our results applying this approach to 18 consecutive infants presenting since 1980 with DILV/TGA and an obstructive anomaly of the aortic arch (coarctation 16, interruption or atresia 2). Four of the infants (22%) were considered to have important SAS at presentation. One underwent neonatal aortopulmonary connection and died. The remaining 17 patients underwent arch repair with PAB (median age 1.4 weeks; range 2 days-22 weeks) with one early death. The 16 survivors have been followed for 5.6 +/- 3.7 years. All but one ultimately developed SAS. Relief of SAS was performed in 15 patients (median age 8 months) using a proximal aortopulmonary anastomosis. There were two early deaths, and one late death. Thirteen of the 18 patients (72%) are alive and well, and 12 have achieved Fontan repair or bidirectional superior cavopulmonary anastomosis (BCPA) with persistent relief of SAS. Most patients with DILV/TGA and aortic arch obstruction will tolerate temporary PAB with adequate protection of the pulmonary vascular bed. Our current approach (in the absence of severe SAS at presentation) is PAB at the time of arch repair, followed by careful surveillance for, and early relief of, SAS combined with BCPA in infancy.

We report on a young housewife who developed a spontaneous coronary artery dissection following unusually severe exercise. She survived an extensive anterior myocardial infarction with the help of an emergency coronary artery vein graft. This rare diagnosis must be considered when a young woman presents with an acute myocardial infarction.

Between 1978 and 1993 a total of 25 cases of ruptured aneurysm of the sinus of Valsalva underwent transaortic repair at the Railway Hospital and the Institute of Cardiovascular Diseases at Madras, India. The aneurysms ruptured into the right ventricle in 20 patients, into the right atrium in 2, into the left ventricle in 1, and into the interventricular septum in 2. They originated from the right coronary sinus in 22 patients and from the noncoronary sinus in 3. Associated congenital anomalies included ventricular septal defect in 21, tetralogy of Fallot in 1, and pulmonary stenosis in 1. All 25 patients underwent transaortic repair of the ruptured sinus of Valsalva, and in patients with a ventricular septal defect the defect also was closed via this route. The patient with associated pulmonary stenosis had a pulmonary valvotomy. The patient with tetralogy of Fallot had infundibular resection, ventricular septal defect closure via a transatrial route, and a right ventricular outflow patch. No intraoperative deaths occurred, but one early death resulted from septicemia and multiorgan failure after rupture of the aneurysm into the interventricular septum with consequent development of extensive intramyocardial and epicardial abscesses. One late death occurred as a result of recurrent subacute bacterial endocarditis and septicemia. Transaortic repair of ruptured aneurysm of the sinus of Valsalva can be accomplished with a low risk of mortality and has the advantage that an associated ventricular septal defect, which frequently accompanies this condition, can be conveniently closed via the same route.

Between July 1974 and October 1988 19 consecutive infants (mean age 2.7 months, range 5 days to 11.7 months) underwent surgical correction for persistent truncus arteriosus by one surgeon (J.L.M.). Continuity between the right ventricle and pulmonary arteries was achieved with an antibiotic-sterilized aortic homograft (diameter 13-18 mm) together with patch closure of the ventricular septal defect. There were 3 early postoperative deaths (16%): 1 patient had severe aortic regurgitation, the other 2 had preoperative cardiac arrests. Of the latter, 1 had suffered severe cerebral damage, and the other developed recurrent pulmonary hypertensive crises and low cardiac output. The 16 survivors have been followed for 3.1-17.3 years (mean 7.8 years). Four patients required subsequent replacement of the homograft for stenosis, aortic valve replacement for regurgitation, or both (3.0, 4.0, 8.5, and 12.0 years postoperatively). Of the 16 survivors, 15 are in NYHA class I. Of the 13 patients who have not had aortic valve surgery, 9 have no evidence of stenosis or regurgitation. In the 14 children with the original homograft the median of the residual peak gradient across the right ventricular outflow tract is 15 mmHg (range 10-40 mmHg), and no patient has severe homograft regurgitation at follow-up. Repair of persistent truncus arteriosus has been achieved with low early mortality and no late mortality, which reflects excellent long-term function of the homograft. Furthermore, if truncal valve function is good at presentation, patients are unlikely to require aortic valve surgery.

To assess the longer term outlook for patients who have undergone surgery for acquired (postinfarction) ventricular septal defect, we interviewed and studied 60 survivors from a single regional cardiac center between 3 and 144 months after the operation. Including the patients who died within 1 month of the operation, the 5-, 10-, and 14-year survivals (with standard errors) were 69% (65% to 74%), 50% (44% to 57%), and 37% (27% to 46%). Eighty-two percent of patients were in New York Heart Association class I or II. Ten patients (17%) had a persisting but not hemodynamically significant ventricular septal defect. Mean left ventricular ejection fraction was reduced at 0.39 (standard deviation 0.15), but this did not correlate with either New York Heart Association class or exercise tolerance. Twenty-eight patients (47%) had asymptomatic arrhythmias (17 with ventricular premature beats). Angina and other medical problems were not prevalent.

Between 1977 and 1988, 27 patients between the ages of 4 and 22 years (mean 8.9) underwent a Fontan procedure with the use of an antibiotic sterilised aortic homograft. There were 15 patients with tricuspid atresia, 9 with univentricular heart and 3 others. The homograft was anastomosed to a right-sided pulmonary artery in ten, to a left-sided main pulmonary artery in eight and in nine patients the homograft connected the right atrium to the right ventricle. There were five early deaths (18.5%) unrelated to the homograft and two late deaths at 7 and 10 years postoperatively. Five patients have required removal of the calcified obstructed homograft with no death. In three patients patches were inserted, but in two patients with good right ventricles a second homograft was inserted. In three of the re-operated patients the homograft lay directly behind the sternum and the femoral artery was exposed and in two of them the femoral artery was cannulated before the chest was opened to control haemorrhage. Of the patients 74% are alive up to 15 years later, 15 with their original homograft. Eight (57%) of those still have their original homograft more than 10 years post-operatively.

We report the unusual occurrence of severe intra-vascular haemolysis following mitral valve repair. Mild to moderate mitral regurgitation was detected after repair, but severe haemolysis was the only indication for re-operation. Following prosthetic valve replacement there was an immediate cessation of haemolysis. We postulate that a small regurgitant jet directed against the teflon pledgets used in the repair was the reason for the haemolysis.

Between 1978 and 1990, 53 consecutive modified Blalock-Taussig (MBT) shunts were performed on 51 neonates with cyanotic congenital heart disease using 3 mm-5 mm Gore-Tex grafts. Only 4 of these children had uncomplicated tetralogy of Fallot. The remainder had more complicated pathology requiring urgent intervention. Retrospective analysis of the acute and long term results was performed with 100% follow-up, ranging from 1 month to 12 years (mean 3 years). There were 3 (6%) early deaths (within 30 days of operation) and 17 (33%) late deaths. Of the late deaths, 2 died after further palliation, 2 died after total correction and 13 died suddenly at home. Post mortem examination of the 13 sudden deaths revealed blocked shunts in only 3. Actuarial survival at 2 years was 58%. Shunt patency at 12 months was 87% and at 2 years, 62%. No patient used their initial MBT shunt for more than 40 months. Although this shunt provides good initial palliation, there is a high incidence of late sudden death. We are also concerned about the limited life span of the shunt which partly (3/13) explains the sudden deaths. Therefore we have adopted an aggressive approach to re-study by angiography within 3 months of surgery.

Post-operative low cardiac output states remain a major cause of mortality following cardiac surgery in infants and children. Since 1979 we have used moderate induced whole-body hypothermia in the management of low-output states refractory to conventional modes of therapy. This is based not only upon the relationship between body temperature and oxygen consumption, but also on experimental work showing a beneficial effect of cooling upon myocardial contractility, particularly when there is pre-existing impairment of ventricular function. Between July 1986 and June 1990, 20 children with refractory low-output states were cooled by means of a thermostatically controlled water blanket to a rectal temperature of 32-33 degrees C. The median age was 12 months (1 week-11 years) with a median weight of 6 kg (3.5-33 kg). Ten children survived to leave hospital while a further two made a haemodynamic recovery. There was a marked reduction in heart rate (P < 0.001). The mean arterial pressure rose (P = 0.037) while there was a fall in mean atrial pressure (P < 0.001). There was a significant improvement in the urine output (P = 0.002). A fall in the platelet count (P < 0.001) was not accompanied by any change in the white cell count (P = 0.15). Although it is impossible to say whether cooling influenced the outcome in any of these children, it was usually effective in stabilising their clinical condition. The technique is simple and has a sound theoretical basis.(ABSTRACT TRUNCATED AT 250 WORDS)

Sixteen patients (10 males and six females) aged 7 months to 20 years, who had undergone surgical repair in the first year of life for total anomalous pulmonary venous connection (TAPVC), underwent assessment of cardiac rhythm by 24-h electrocardiogram (ECG) monitoring, nine of them also had maximal exercise treadmill tests, 3 months to 19.5 years after surgical repair. No patient had symptoms of an arrhythmia and the resting ECG was normal in all except one who had occasional single supraventricular ectopic beats. But on 24-h ECG monitoring significant arrhythmias were recorded in six of the 16 patients, including supraventricular tachycardia (three patients), bradyarrhythmia (two patients), sick sinus syndrome (two patients), and multiform supraventricular and ventricular ectopic beats (two patients). Five of these six patients were assessed more than 6 years after surgery. An inappropriate chronotropic response was seen on maximal exercise treadmill testing in four patients; three of them had arrhythmias previously recorded by 24-h ECG monitoring. Our observations show that significant arrhythmias may occur in asymptomatic patients long after TAPVC correction, and we therefore recommend long-term follow-up of these patients, even if they are asymptomatic.

The optimal management of pulmonary atresia with an intact ventricular septum in the neonate remains controversial. The introduction of balloon septostomy and prostaglandin has significantly reduced early mortality but early surgical intervention is necessary to obtain a more adequate pulmonary blood flow. Fourteen neonates with pulmonary atresia and an intact ventricular septum were admitted to the Wessex Cardiothoracic Unit, Southampton from 1979 to 1986. Thirteen patients underwent cardiac catheterization. Cardiac catheterization data and right ventricular angiograms were reviewed retrospectively. Four patients with tripartite ventricles underwent total repair. The others received various palliative operations (valvotomy + modified Blalock-Taussig shunt or modified Blalock-Taussig shunt alone). Retrospective analysis of the angiograms indicated that right ventricular morphology alone is not a satisfactory criterion for surgical management. We have been able to demonstrate that there is a good correlation between the diameter of the tricuspid valve and the diameter of the infundibulum and that successful neonatal repair is possible when the tricuspid valve diameter is above 80% of the normal value for weight and when the tricuspid valve diameter to infundibular diameter ratio (TV/Inf ratio) is 2.2 or less. In patients with a tripartite ventricle but inadequate TV diameter and TV/Inf ratio, a closed pulmonary valvotomy with a modified Blalock-Taussig shunt remains the treatment of choice.

A Gerbode defect is a ventricular septal defect communicating directly between the left ventricle and right atrium. There has been some debate about the existence of such an entity. We present the case of a previously healthy 15-year-old boy who developed bacterial endocarditis following ear piercing and was subsequently found to have a defect between the left ventricle and right atrium, which was successfully repaired surgically.

The cases of 110 infants less than 1 year of age, who had surgical repair for coarctation of the aorta between June 1974 and February 1988, were analysed. Three groups of patients were identified. In group 1 there were 39 patients with isolated coarctation. In group 2 there were 25 infants with additional ventricular septal defects (VSD), while in group 3 there were 46 infants with other associated congenital cardiac defects. Repair was performed using the subclavian flap aortoplasty (SFA) procedure in 83 patients, resection with end-to-end anastomosis (EEA) in 23, patch aortoplasty in 3 and Goretex tube bypass in 1. Twenty-eight patients had simultaneous pulmonary artery banding and one concomitant closure of the VSD. The overall early mortality rate was 8.2% (5.1% in group 1, 0% in group 2, and 15.2% in group 3). Age at operation (under 1 month, p = 0.04) and other associated cardiac anomalies (p = 0.03) increased early mortality significantly. There were 11 late deaths (10.8%) among 101 patients followed from 1 to 15 years (mean 5.3 years). Twelve patients underwent further surgery for recoarctation, eight of them within 11 months. A further 11 patients currently have a Doppler gradient across their coarctation site of more than 20 mmHg, but have not undergone further surgery to the coarctation repair site.

The total surgical experience of a supraregional paediatric cardiology unit over a nine year period (January 1980 to December 1988) was reviewed to assess the effect of the introduction of the full range of ultrasound techniques. A total of 1517 patients underwent cardiac surgery (955 cardiopulmonary bypass, 562 closed procedures). Of these, 485 patients (32%) did not undergo cardiac catheterisation before operation: 217 bypass (23% of all procedures under cardiopulmonary bypass) and 268 closed procedures (48%). The overall ratio of catheterisations to operations for patients undergoing palliative or corrective surgery fell from 0.97 (1980) to 0.38 (1988). The patients were classified as (a) neonates (0-28 days), (b) infants (one to 12 months), and (c) children (one to 14 years). The main impact of non-invasive surgical referral was in neonates (total catheter:operation ratio 0.38; neonates 0.2 for 1988). The surgical population was further divided according to the principal echocardiographic technique available: (a) 1980-4 cross sectional imaging; (b) 1985-6; imaging plus spectral Doppler ultrasound; (c) 1987-8; imaging plus spectral Doppler ultrasound and colour flow mapping. A fall in the catheter:operation ratio for all age groups was most pronounced in the last four years. This reflects increased familiarity and surgical confidence with non-invasive diagnostic assessment. The introduction of each new echocardiographic technique was associated with a significant fall in the total catheter:operation ratio compared with the preceding period. Six incorrect ultrasound diagnoses were made during the entire period; one of these patients died in the early postoperative period. The integration of Doppler ultrasound with cross sectional imaging has made non-invasive assessment an increasingly practical alternative to preoperative cardiac catheterization.

A total of 101 patients (mean age 64.9 years) underwent surgical correction of postinfarction ventricular septal defect at this institution over a 15-year period (1973 to 1988). The overall early mortality rate was 20.8%, although the most recent experience with 36 patients (January 1987 to October 1988) has seen this decline to 11.1%. Factors found to influence early death significantly, when analyzed univariately, were as follows: (1) site of infarction (anterior 12.1%, inferior 32.6%, p = 0.02); (2) time interval between infarction and operation (less than 1 week 34.1%, greater than 1 week 10.5%, p = 0.008); (3) cardiogenic shock (present 38.1%, absent 8.5%, p = 0.001). Nonsignificant variables included preoperative renal function, age, and concomitant coronary artery bypass, although older age (greater than 65 years) became significant when examined in a multivariate fashion. Of the 80 hospital survivors, eight were subsequently found to have a recurrent or residual defect necessitating reoperation, with survival in seven. Late follow-up is 99% complete and reveals an actuarial survival rate for 100 patients of 71.1% at 5 years (95% confidence interval 60.6 to 80.0), and 40.0% at 10 years (95% confidence interval 21.7 to 58.4). A significant recent change in policy of not using coronary angiography in patients with a ventricular septal defect caused by anterior wall infarction has not resulted in any increase in either the early mortality or in the late prevalence of angina. The functional status of 38 surviving patients has been analyzed by a graded treadmill exercise protocol, whereas left ventricular functional assessment was by nuclear scan with additional information on mitral valve function by echocardiogram. Color Doppler flow mapping has been used to determine the presence of a residual defect. Most late survivors have limited exercise tolerance related to both cardiac and noncardiac factors. Left ventricular function is moderately impaired (mean ejection fraction = 0.39). However, many patients are elderly and have adapted to their residual symptoms without significant changes in life-style.

Two neonates with truncus arteriosus (TA) and type B interrupted aortic arch are reported. In both, a homograft was used to connect the right ventricle to the pulmonary artery, while the arch continuity was established by direct anastomosis of the aortic arch to the descending aorta.

One hundred reoperations were performed on 93 patients of a total of 2287 patients who underwent initial coronary artery bypass surgery (CABG) between September 1972 and August 1988. The mean age of the 84 males and 9 females was 55.5 years (range 31-75 years). All patients prior to reoperation had severe angina and were judged to be in NYHA class III or class IV. Late graft failure alone or in combination with progressive atherosclerosis accounted for more than 60% of the cases requiring reoperation. The early mortality for reoperation was 1% compared with 1.4% for initial CABG. Perioperative myocardial infarction was recorded as a complication in 3.2% of cases at initial operation compared with 1% at reoperation. Symptomatic improvement occurred in 89.1% of cases after reoperation and almost 60% became entirely asymptomatic (mean follow-up interval 17 months).

A prospective randomised trial was performed on 100 patients undergoing coronary artery bypass grafting without concomitant procedure. The study group commenced oral verapamil 40 mg three times daily on the first post-operative day while the control group received no antiarrhythmic agents. The pre-operative characteristics of both groups were similar with the exception of the incidence of hyperlipidemia which was greater in the verapamil group (P = 0.04). Myocardial protection was achieved with cold crystalloid cardioplegia. Cardiopulmonary bypass times, aortic cross clamp times and graft numbers were similar for both groups. Nine patients were excluded on the first post-operative day; the remainder were studied for 8 days. Supraventricular tachyarrhythmias (atrial fibrillation, atrial flutter or paroxysmal supraventricular tachycardia) were detected in 8 patients in the study group (n = 44) and in 5 patients in the control group (n = 47). The difference was not significant (P = 0.3). The ventricular rate in patients taking verapamil who developed supraventricular tachycardia was 138 +/- 14.9 compared with 156.8 +/- 17.9 in the control group, but the difference failed to reach significant levels (P = 0.065). In conclusion, prophylactic oral verapamil 40 mg given three times daily after coronary artery surgery failed to decrease the incidence of post-operative supraventricular tachycardia or to significantly influence the ventricular rate if tachycardia developed.

The management of three infants born with a combination of tetralogy of Fallot and a vascular ring causing tracheoesophageal compression is described. There was a double aortic arch in two patients and an aberrant left subclavian artery with left ligamentum arteriosum and right aortic arch in one. Single-stage corrective surgery of both lesions during infancy, performed under profound hypothermia and circulatory arrest, was successful. In our opinion, this is the treatment of choice, when the anatomy of the tetralogy is favorable for primary correction.

The past 9 years' experience with ventricular septal rupture complicating myocardial infarction has been reviewed. Thirty-six patients were treated surgically, with 10 early deaths (28%) and one late death, for an 8 year actuarial survival rate of 63%. The mortality was highest for those defects which followed inferior infarction, 38% compared with 13% following anterior infarction. The infarction-operation interval also greatly influenced mortality; under 2 weeks, 43%; over 2 weeks, 18%. Concomitant coronary artery bypass grafts (13 patients) or left ventricular aneurysmectomy (14 patients) did not carry an increased mortality. Of 17 patients who presented with cardiogenic shock, eight died (47%). The intra-aortic balloon pump (IABP) was used in 16 patients (44%) and helped greatly in the management of the critically ill. With an estimated 17 acquired septal defects occurring each year in persons under 65 years of age in Wessex, awareness of this complication and of the favorable outcome of operation is essential among those who treat the aftereffects of myocardial infarction.

A transpulmonary arterial approach to the closure of a high ventricular septal defect (VSD) has been used, between 1978 and 1982, in eight patients. The reasons were ease of access and the wish to overcome the problems associated with right ventriculotomy. The patients' ages ranged from three weeks to 15 months, their weight from 2.9 kg to 9 kg. The approach was used both when the VSD was an isolated anomaly and when there were major associated defects. It is in this latter group, four with aortic arch anomalies, two with additional double outlet right ventricle (DORV), that avoidance of ventriculotomy was most helpful. It was especially important in the two patients with DORV and a perimembranous, outlet subpulmonary VSD, where it was possible to close off the left ventricular outflow tract and pulmonary valve using a patch, without opening the right ventricle, which was subsequently to become the systemic ventricle. This technique obviates the need for ventriculotomy in the closure of some perimembranous outlet and doubly committed subarterial VSDs, and is the approach of choice for the closure of a perimembranous, outlet, subpulmonary VSD in DORV.

The results of 3000 consecutive operations using cardio-pulmonary bypass show that the overall early mortality was 6.1%, dropping from 8.9% in the first 1000 to 4.4% in the third 1000. Operations for valve disease have been the most common, the early mortality for aortic valve replacement being 3.1% and for mitral valve replacement 2.9%. Combined aortic and mitral valve replacement had an early mortality of 4.4%. The number of patients undergoing isolated coronary artery bypass grafting has increased from 59 in the first 1000 to 292 in the third 1000 operations, with an overall early mortality of 1.3%. Six hundred and ninety seven patients underwent surgery for congenital heart disease with an overall early mortality of 10.9% (7.5% in the last 2000 cases). The patients have been followed up from one to 8.5 years. A high proportion have returned to work and enjoy a normal life. At the time of review, 87% of the 3000 patients were alive. Long waiting times for outpatient and inpatient care indicate underprovision of facilities relative to regional demand.

Dura mater obtained from human cadavers and preserved in glycerol was used as patch grafts in various positions in the repair of acquired and congenital cardiac defects in 107 patients over a four-year period. The ages of the recipients ranged from 2 days to 75 years. The mean duration of preservation of the dura mater before use was 25.2 +/- 8.1 weeks. The dural grafts were used as an aortic root gusset in 38 patients (35.5%) undergoing aortic valve replacement, for enlargement of the pulmonary artery or right ventricular outflow tract or both in 38 patients (35.5%), and for repair of coarctation of the aorta in 10 patients (9.4%). The grafts also were used for closure of atrial septal defects, for a variety of other congenital cardiac anomalies, and for replacement of segments of the aorta (2 patients). There were no complications attributable to the use of dura mater. Dura mater preserved in glycerol would seem to be a suitable material for repair of various cardiac defects because of its strength, low antigenicity, athrombogenicity, ease of handling, availability in large sizes, and rapid bonding to host tissue. However, long-term follow-up is important to assess the lasting properties of dura mater.