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Objective: To evaluate the extent of intrapulmonary right to left shunting in children after bidirectional cavopulmonary anastomosis (BCPA)., Design: Prospective study of patients who underwent BCPA in a single centre., Patients: 17 patients with complex cyanotic congenital cardiac malformations who underwent BCPA at 1-45 months of age (median 21 months) were evaluated 15-64 months postoperatively (median 32 months). Five children between 1 and 10 years (median 5 years) with normal or surgically corrected intracardiac anatomy and peripheral pulmonary circulation who required V/Q scanning for other reasons were used as controls., Interventions: All patients underwent cardiac catheterisation to exclude angiographically demonstrable venovenous collaterals followed by pulmonary perfusion scanning using (99m)technetium ((99m)Tc) labelled albumen microspheres to quantify the intrapulmonary right to left shunt., Main Outcome Measure: Percentage of intrapulmonary right to left shunt., Results: The mean (SD) level of physiological right to left shunting found in the control group was 5.4 (2.3)%. All patients with BCPA showed the presence of a significantly higher level of intrapulmonary shunting (26.8 (16.9)%, p < 0.001). The degree of shunting was significantly increased in the subgroup of 11 patients with BCPA as the only source of pulmonary blood flow (34.9 (15.8)%), when compared to the six remaining patients with an additional source of pulmonary blood supply (12.0 (2.6)%, p < 0.001). There was a negative correlation between age at BCPA and the shunt percentage found in the patients with a competitive source of pulmonary blood flow (r = -0.63, p < 0. 01)., Conclusions: Intrapulmonary right to left shunting develops in all patients following BCPA. This may be caused by a sustained and inappropriate vasodilatation resulting from absence or decreased levels of a substance that inhibits pulmonary vasodilatation. Augmenting BCPA with an additional source of blood flow containing hepatic factor limits the degree of intrapulmonary arteriovenous shunting and may help provide successful longer term palliation.

Background: The CarboMedics bileaflet prosthetic heart valve was introduced in 1986. We first implanted it in March 1991. The purpose of this study was to analyze our clinical experience with this valve., Methods: Between March 1991 and December 1997, 1,378 valves were implanted in 1,247 patients, 705 men (56.5%) and 542 (43.5%) women with a mean age of 62 +/- 11.9 years (+/- the standard deviation). Follow-up is 99% complete and totals 3,978 patient-years., Results: The early mortality rate was 4.4% (55/1,247). The survival rates at 1 year and 5 years were 91.8% +/- 0.8% (+/- the standard error of mean) (n = 1,062) and 79.2% +/- 1.4% (n = 281), respectively. Freedom from valve-related complications (linearized rate, 4.9% per patient-year) at 1 year and 5 years was 90.6% +/- 0.8% (+/- the standard error of the mean) (n = 996) and 80.6% +/- 1.4% (n = 243), respectively. Linearized rates for various complications were as follows: bleeding events, 1.73% per patient-year; embolic events, 1.76% per patient-year; operated valvular endocarditis, 0.18% per patient-year; valve thrombosis, 0.10% per patient year; and nonstructural dysfunction, 1.21% per patient-year. Freedom from reoperation at 1 year and 5 years was 98.6% +/- 0.3% (+/- the standard error of the mean) (n = 1,070) and 97.7% +/- 0.5% (n = 285), respectively., Conclusions: Midterm results demonstrate that the CarboMedics prosthetic heart valve exhibits a low incidence of valve-related complications.

Objective: We report the combined early results from two centers in the United Kingdom using a composite conduit consisting of a bileaflet mechanical valve incorporated into a gelatin-impregnated, ultra-low porosity, woven polyester graft (Carbo-Seal; Sulzer Carbomedics, Inc, Austin, Tex)., Methods: Between August 1992 and March 1997, 143 patients underwent aortic root replacement with the Carbo-Seal composite prosthesis. The indication for surgery was acute type A dissection in 31 (22%), chronic type A dissection in 9 (6%), ascending aortic aneurysm without dissection in 100 (70%), and false aneurysm of the ascending aorta in 3 (2%). Twenty-seven patients (19%) had undergone previous sternotomy, and 40 (28%) were seen as emergencies. Concomitant procedures were performed in 38 (27%), including 18 aortic arch or hemiarch replacements. Total follow-up is 270 patient-years. Follow-up is 100% complete., Results: The early (30-day) mortality was 7% (10 patients). Permanent neurologic events occurred in 2%. At a mean follow-up of 23 months, 94% of survivors were in New York Heart Association functional class I. Freedom from reoperation was 97.2% +/- 1.6% (1 standard error [1 SE]) at 12 months and 95.7% +/- 2.2% at 48 months. Including early mortality, survival was 90.1% +/- 2.6% at 12 months and 83.1% +/- 3. 5% at 48 months., Conclusions: Aortic root replacement with use of the Carbo-Seal prosthesis can be undertaken with a relatively low early mortality and morbidity. A low reoperation rate and high intermediate-term survival can be expected, but continued follow-up is needed to determine the long-term efficacy of this prosthesis.

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.

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: 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.

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.

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 1973 and 1993 sixty aortic homograft valved conduits in fifty-six patients were used to establish continuity between the right ventricle and the pulmonary artery in congenital heart disease. Age range was one day to 23.5 years (median 3.6 years) which included twenty-six patients less than one-year-old. Conduit size ranged from 11 to 23 mm (median 17.6 mm). there were nine hospital deaths and eight late deaths. The 45 survivors have been followed for a median of 8.6 years (range 6 months to 20 years). All patients have had serial echocardiographic assessments and 35 have had post repair cardiac catheterization. Almost all patients had mild-to-moderate degrees of homograft regurgitation. There were eleven with severe homograft regurgitation and two are being considered for reoperation. The follow up homograft gradient ranged from 0 to 64 mmHg (mean 24 mmHg). Freedom from reoperation for conduit obstruction was 98.2% at five years falling to 91% (C.L. 82%-100%) at 10 years. Of the 23 homografts inserted more than 10 years ago, only one (4.3%) has been replaced because it was causing important obstruction. None have been replaced for regurgitation. Our results indicate that larger sized aortic homografts used in reconstruction of the right ventricular outflow tract give satisfactory results and there is a low incidence of reoperation for replacement at medium term follow up.

The expected level of systemic arterial saturation may not be present after bidirectional superior cavopulmonary anastomosis in children with complex congenital cardiac anomalies. We present a case of persistent severe cyanosis in a patient with azygos continuation of the inferior vena cava after bidirectional superior cavopulmonary anastomosis (Kawashima procedure) due to an intrahepatic venovenous malformation. Subsequent transcatheter deployment of two Rashkind double umbrella devices into the malformation reduced the shunt and markedly improved systemic arterial saturation.

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.

An unexpected left atrial mass was found during routine postoperative transthoracic echocardiogram in an infant following surgical closure of ventricular septal defect. Thrombus could not be excluded. At reoperation it was found to be an inverted left atrial appendage. This case demonstrates the echocardiographic appearance of an inverted left atrial appendage.

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.

Translocation of the coronary arteries remains a technical problem in anatomic correction of transposition of the great arteries. Myocardial ischemia related to difficulties with coronary relocation is a significant factor in perioperative morbidity and mortality, particularly in those patients with complex coronary anatomy. Two neonates with transposition of the great arteries and intact ventricular septum are described in whom the coronary arteries arose from multiple ostia, all lying within sinus 1, with one of the ostia in each instance being severely eccentric. An anatomic switch of the great vessels was done without coronary relocation. This was achieved by means of an aortopulmonary fenestration with a bovine pericardial tunnel to allow coronary artery perfusion with blood from the neoaorta. An additional pericardial patch was placed to the contralateral wall of the proximal neopulmonary artery. Both infants had an uneventful postoperative recovery with no evidence of myocardial ischemia, although both have a mild gradient across the proximal pulmonary artery. This operative technique may be appropriate in those patients in whom there is concern over the feasibility of translocating the coronary arteries without producing myocardial ischemia.

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.

Having had surgical repair of aortic coarctation at the age of 12 years, and re-operation at the age of 19 years for stenosis at the site of the previous repair, a 29 years old man presented as an emergency with a 24 hour history of interscapular pain, haemoptysis and collapse. At thoracotomy he was found to have a ruptured superior intercostal artery which was ligated. Spontaneous rupture of an intercostal artery has not been previously recorded.

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.

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).

Extracorporeal membrane oxygenation has been advocated as the most appropriate mode of circulatory support in the paediatric age group for post-cardiopulmonary bypass ventricular dysfunction. The results in infants who have predominantly left ventricular failure, or who require such support in order to be weaned off bypass, have been disappointing. Three infants with severe left ventricular dysfunction following cardiopulmonary bypass for correction of congenital heart defects have been managed with a left ventricular assist device. Two required this form of circulatory support in order to be weaned from full bypass while in the third infant it was instituted for progressive left ventricular dysfunction postoperatively. All three were less than 10 kg in weight. Left atrial appendage to aortic bypass was effected using a closed loop circuit with a constrained vortex pump (Biomedicus). Duration of support ranged between 40 and 146 h. One infant made a complete recovery and was able to be discharged home 20 days postoperatively. Another made a circulatory recovery such that both mechanical and inotropic support could be discontinued but had sustained massive neurological damage. The third died of progressive left ventricular dysfunction. This experience with a left ventricular assist device demonstrates that it is technically feasible in small infants, and can be performed to good effect in infants with predominant left ventricular dysfunction following cardiac surgery. It may well be more appropriate than extracorporeal membrane oxygenation in this group of patients.

Between 1968 and 1985, 80 children underwent correction of total anomalous pulmonary venous drainage. There were 47 boys and 33 girls whose ages ranged from 3 days to 16 years (median 2 months, interquartile range 5 years). Seventy (87.5%) were less than 1 year of age at operation. Fifty-eight (72.5%) weighed less than 5 kg, the range being 1.6 to 42 kg (median 3.7 kg, interquartile range 2.4 kg). Forty-five (56%) patients had supracardiac, 14 (17.5%) cardiac, 15 (19%) infracardiac, and 6 (7.5%) had mixed total anomalous pulmonary venous drainage. Follow-up was complete in 78 (97.5%) and ranged from 6 to 189 months (median 58 months, interquartile range 59 months). There were 14 (17.5%) early and six (7.5%) late deaths. Analysis by various factors revealed year of operation as the only factor to affect survival at the 5% level of significance. Early mortality was 29% between 1968-1977 and 11% between 1978-1985 (p = 0.04). Postoperative pulmonary venous obstruction occurred in five (6%) patients between 6 weeks and 3 months after operation. All 5 died, three after reoperation. Five (6%) other children had reoperations, four for residual shunts and one for superior vena caval obstruction.

Fifteen infants with tetralogy of Fallot, who would otherwise have required a palliative operation, underwent balloon dilatation of the right ventricular outflow tract. The mean period of palliation was 8.5 months (range 0-26 months). The procedure was performed without serious complications on 88% of occasions. This preliminary study suggests that balloon dilatation may be useful in the management of tetralogy of Fallot.

Sixty patients undergoing coronary artery bypass surgery were studied prospectively in order to investigate the effect of a cardioselective beta-blocker on the incidence of postoperative supraventricular arrhythmias. Patients with good left ventricular function were randomly divided into two groups: 30 patients treated with atenolol and 30 patients acting as controls. Atrial fibrillation was seen in 11 patients and frequent premature atrial extrasystoles were noted in one. Eleven (37%) patients in the control group experienced arrhythmias whilst atenolol significantly reduced this incidence to 3% (one patient), P = 0.001. There was no significant relationship between the development of supraventricular arrhythmias and the following variables: age, sex, severity of preoperative symptoms, previous myocardial infarction, extent of coronary artery disease, technique of myocardial preservation used, ischaemic time, number and site of saphenous vein grafts, endarterectomies performed and perioperative serum potassium levels. It is concluded that the use of atenolol (started 72 h before operation) is effective in reducing the incidence of supraventricular arrhythmias following elective coronary artery bypass operations in patients with good left ventricular function.

A 3-year-old girl with Fallot's tetralogy underwent balloon pulmonary valvoplasty without any obvious complications. Elective total correction was performed 15 months later. At operation, a well-healed longitudinal tear measuring 2 cm was noted in the posterior wall of the main pulmonary artery 1 cm from the pulmonary valve extending into the right pulmonary artery.

An air gun pellet was found in the right superior pulmonary vein after penetrating the left ventricle of a 14 year old boy. This apparent retrograde movement in the left side of the heart has not been reported previously.