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Background: The transition from student to medical professional represents a major challenge for all medical students. Every intake of newly qualified doctors increases potential risks to patients. In the UK, this is often colloquially referred to as the ‘August Effect’. At one District General Hospital, a ‘Ward-Craft and Bleep Simulation Programme’ was implemented for final year medical students. This aimed to equip students with the procedural knowledge and soft skills to support the transition from undergraduate training to professional working life as a junior doctor. Methods: A series of six, weekly, two-hour workshops which focussed on common clinical problems encountered by newly qualified doctors was delivered to final year medical students (‘Ward-Craft Programme’). Students then participated in a two-hour high fidelity, in-situ, ‘Bleep Simulation Programme. The programme was facilitated by a Clinical Teaching Fellow and evaluated through an anonymous feedback survey Results: 98.9% of students (n=259) agreed, or strongly agreed, that the programme helped them feel more prepared and confident for life as a junior doctor. Participants provided overwhelmingly positive feedback, specifically noting that the programme addressed feelings of confusion, anxiety, and the feeling of being overwhelmed by the work of junior doctor. Conclusions: The Ward-Craft and Bleep Simulation Programme has been positively received by medical students from universities with very different types of learning styles and courses. The replication of similar programmes for final year medical students is a valuable training opportunity for final year medical students and may help mitigate the challenges posed by the ‘August effect’.

Global health education (GHE) continues to be a growing initiative in many medical schools across the world. This focus is no longer limited to participants from high-income countries and has expanded to institutions and students from low- and middle-income settings. With this shift has come a need to develop meaningful curricula through engagement between educators and learners who represent the sending institutions and the diverse settings in which GHE takes place. The Bellagio Global Health Education Initiative (BGHEI) was founded to create a space for such debate and discussion and to generate guidelines towards a universal curriculum for global health. In this article, we describe the development and process of our work and outline six overarching principles that ought to be considered when adopting an inclusive approach to GHE curriculum development.

Objective Advances in early management of congenital heart disease (CHD) have led to an exponential growth in adults with CHD (ACHD). Many of these patients require cardiac surgery. This study sought to examine outcome and its predictors for ACHD cardiac surgery. Methods This is an observational cohort study of prospectively collected data on 1090 consecutive adult patients with CHD, undergoing 1130 cardiac operations for CHD at the Royal Brompton Hospital between 2002 and 2011. Early mortality was the primary outcome measure. Midterm to longer-term survival, cumulative incidence of reoperation, other interventions and/or new-onset arrhythmia were secondary outcome measures. Predictors of early/total mortality were identified. Results Age at surgery was 35±15 years, 53% male, 52.3% were in New York Heart Association (NYHA) class I, 37.2% in class II and 10.4% in class III/IV. Early mortality was 1.77% with independent predictors NYHA class ≥ III, tricuspid annular plane systolic excursion (TAPSE)

A wealth of experience has been gained in the management of atrioventricular septal defect (AVSD) since the first complete correction of this malformation in 1955. The success of surgical therapy followed an enhanced understanding of morphology and physiology as well as major improvements in imaging of this congenital heart defect. Therapeutic success in the management of patients with AVSD has been extended to include those with associated lesions such as tetralogy of Fallot, double outlet right ventricle and relative degrees of ventricular hypoplasia. Although operative mortality is low and long-term survival is relatively good, important detrimental residual or AVSD-related complications such as left atrioventricular valve regurgitation, left ventricular outflow tract obstruction still carry significant late morbidity in a proportion of patients. This article reviews our current understanding of the morphology of this defect, aspects of diagnosis and surgical treatment options.

Improved patient survival and increasingly complex surgery have expanded the requirement for specialist care for patients with adult congenital heart disease (ACHD). Despite the recent publications of management guidelines for ACHD, data concerning optimal patterns of care in the peri-operative/critical care period of this challenging population are sparse. The aims of the current study were to therefore to determine the pattern of intensive care unit (ICU) management, resource utilisation and predictors of mortality in critically ill ACHD patients. Data were collected prospectively for patients with ACHD stratified for complexity of disease admitted to the ICU of a tertiary cardiothoracic centre (1997–2002). Multivariate analysis of pre-operative indices as predictors of mortality was performed. Of 342 ACHD admissions (total mortality 4.4%, simple 0%, moderate/complex 10.6%), the requirement for specialist investigations and interventions was high, reflected in ICU admission costs per patient (simple $5391 ± 130, moderate $13218 ± 261, complex $30074 ± 689). Standard severity of illness scoring systems did not accurately predict mortality; however, abnormal pre-operative thyroid function (p = 0.0048), creatinine (p = 0.0032) and bilirubin (p = 0.0021) were highly predictive of mortality. Peri-operative mortality in patients with ACHD is low overall but varies with disease complexity. Such patients have a high requirement for specialist ICU investigation/intervention. Although standard severity of illness scoring is unhelpful, simple pre-operative parameters may predict peri-operative mortality. These findings reflect the requirement for specialist care, and have implications for planning service provision, training and operative consent in ACHD patients.

We reviewed the surgical outcomes in adults and children with subaortic stenosis and intact ventricular septum in the current era. The case notes of 50 patients were reviewed for retrospective evaluation of preoperative, intraoperative and postoperative data. Data of primary operations during the period 2000-2005 were compared with data from patients who had re-do surgery during the same period. Thirty-five patients had primary operation and 15 patients had re-do surgery. The median age at primary operation was eight years (range 3 to 44), at second operation was 14 years (range 9 to 26) and at third operation was 15 (range 9 to 47). The entire group had been followed up postoperatively for a median of 2.5 years (range 0 to 5). Pre-operatively, aortic regurgitation was moderate in 13 and severe in three patients. Moderate to severe aortic regurgitation was present in 7 (20%) patients with primary operations and 9 (60%) patients with re-do surgery (P=0.01). Reviewing the first operations of all the re-dos (15 patients) in our series, one patient had myectomy and the rest (14 patients) had isolated resection. Aortic valve regurgitation is more prevalent in patients with recurrent subaortic stenosis. Addition of myectomy is better than shelf resection only.

Congenital anomalies of the tricuspid valve, and/or its supporting apparatus, leading to severe tricuspid regurgitation are rare. Although well tolerated in early childhood, long-standing and progressive volume loading of the right heart leads to symptoms of decreased exercise tolerance, and may predispose to arrhythmias in the long term. We report three cases of severe tricuspid regurgitation related to anomalies of the cords supporting the antero-superior leaflet of the tricuspid valve. Shortened cords leading to tethering of the leaflet were seen in two cases, and hypoplasia of the leaflet in the other. In all cases, the regurgitant jet was directed posteriorly towards the coronary sinus and atrial septum. Surgical repair was possible in one case, while it proved necessary to replace the valve in a second. The third child is asymptomatic and under regular review.

Background In hearts with congenitally corrected transposition of the great arteries, the atrial and ventricular septums are usually malaligned. This is associated with an abnormal location of the atrioventricular conduction system. However, well-aligned septums and normally positioned atrioventricular conduction tissues have been found in a few cases, some of which also happened to have a small or atretic pulmonary trunk. Our aim was to determine whether septal alignment could be predicted on the basis of the size of the pulmonary trunk. Potentially this could provide a guide to the disposition of the atrioventricular conduction tissues. Methods We examined 14 hearts. In all cases there was usual atrial arrangement. We carried out histologic examination of the sites of the atrioventricular conduction tissues in 1 heart. Results We found a statistically significant correlation between the size of the pulmonary trunk and the degree of septal malalignment. In the presence of a small or atretic pulmonary trunk, the septums were well aligned. Our histologic study of one such specimen revealed dual atrioventricular nodes connecting to a sling of conduction tissue. This suggests that presence of a postero-inferiorly situated atrioventricular conduction bundle in addition to an anteriorly located bundle may be anticipated when the septums are well aligned. Conclusions The presence of a small or atretic pulmonary trunk in congenitally corrected transposition of the great arteries is associated with good septal alignment. This should alert the surgeon to the possibility of a slinglike arrangement of the atrioventricular conduction system.

Background Surgical valvotomy for critical aortic stenosis in children enables relatively accurate commissurotomies to be fashioned, resulting in the formation of two or three leaflets. We hypothesized that outcomes after surgery may be best in patients in whom three leaflets are produced. Methods A retrospective review of infants undergoing primary surgical valvotomy at our institution during a 12-year period was carried out. Patients who had additional intracardiac defects were excluded. Clinical and echocardiographic follow-up data were analyzed. Results Fifty-four patients fulfilled the study criteria. Median age at surgery was 3 weeks (range, 0 to 51 weeks). Commissurotomy resulted in bileaflet anatomy in 41 patients (group A) and trileaflet anatomy in 13 patients (group B). Operative mortality was 5% in group A and 0% in group B ( p = 1.0). In group A, 18 patients required one or more aortic valve reinterventions, including valve replacement in 8 patients. In group B, there was only one reintervention (repeat valvotomy). Kaplan-Meier analysis showed that at 10 years, comparisons of group A versus group B were as follows: actuarial survival, 85% versus 100% ( p = 0.15); freedom from reintervention, 33% versus 92% ( p = 0.01); freedom from aortic reoperation, 45% versus 92% ( p = 0.04); and freedom from aortic valve replacement, 57% versus 100% ( p = 0.07). Conclusions Long-term outcomes after aortic valvotomy are significantly better in infants in whom surgery results in trileaflet rather than bileaflet anatomy. Preoperative evaluation of valve morphology may enable selection of a group of patients in whom results of surgery are excellent.

Background . Less than perfect coronary artery translocation accounts for the majority of perioperative deaths after the arterial switch procedure for transposition of the great arteries. Some types of coronary arterial anatomy are associated with a higher risk of death. Methods . Prospective epicardial ultrasound examination of all neonates with failing left ventricle or difficulty in weaning off cardiopulmonary bypass was performed after completion of the arterial switch operation during a 2-year period from March 1994 to February 1996. The aim was to identify any mechanical, and potentially remediable, factors accounting for ventricular failure. Results . Four neonates fulfilling the above criteria were identified during a 2-year period when epicardial echocardiography was routinely applied. In 2 patients coronary arterial problems in the form of kinking of the proximal left coronary artery (1 patient) and extrinsic compression of the artery by the neo-pulmonary trunk (1 patient) were identified and successfully corrected. In 2 other patients, supravalvar aortic stenosis was recognized, leading to prompt revision. Conclusions . Epicardial echocardiography has an important "troubleshooting" role in the subgroup of patients with a suboptimal hemodynamic result after the arterial switch operation. Patients with unusual coronary anatomy should routinely be candidates for such studies. (Ann Thorac Surg 1997;63:445–8)

We describe an individualised approach in the rare situation of a tunnel between the left aortic sinus of Valsalva and the junction of the superior caval vein with the right atrium. It is necessary, in this rare anomaly, to identify the origin of the left coronary artery prior to intervention.

To evaluate the impact of Down syndrome on the early postoperative outcomes of children undergoing complete atrioventricular septal defect repair.Retrospective cohort study.Single tertiary pediatric cardiac center.All children admitted to PICU following biventricular surgical repair of complete atrioventricular septal defect from January 2004 to December 2009.None.A total of 107 children, 67 with Down syndrome, were included. Children with Down syndrome were operated earlier: 4 months (interquartile range, 3.5-6.6) versus 5.7 months (3-8.4) for Down syndrome and non-Down syndrome groups, respectively (p0.01). There was no early postoperative mortality. There was no significant difference in the prevalence of dysplastic atrioventricular valve between the two groups. Two children (2.9%) from Down syndrome and three children (7.5%) from non-Down syndrome group required early reoperation (p = 0.3). Junctional ectopic tachycardia was the most common arrhythmia, and the prevalence of junctional ectopic tachycardia was similar between the two groups (9% and 10% in Down syndrome and non-Down syndrome, respectively, p = 1). One patient from each group required insertion of permanent pacemaker for complete heart block. Children with Down syndrome had significantly higher prevalence of noncardiac complications, that is, pneumothorax, pleural effusions, and infections (p0.01), than children without Down syndrome. There was a trend for longer duration of mechanical ventilation in children with Down syndrome (41 hr [20-61 hr] vs 27.5 hr [15-62 hr], p = 0.2). However, there was no difference in duration of PICU stay between the two groups (2 d [1.3-3 d] vs 2 d [1-3 d], p = 0.9, respectively).In our study, we found no difference in the prevalence of atrioventricular valve dysplasia between children with and without Down syndrome undergoing complete atrioventricular septal defect repair. This finding contrasts with previously published data, and further confirmatory studies are required. Although clinical outcomes were similar, children with Down syndrome had a significantly higher prevalence of noncardiac complications in the early postoperative period than children without Down syndrome.

Background. Accurate perioperative assessment of the adequacy of pulmonary artery banding has hitherto relied on oximetry and direct pressure measurements. We report the use of epicardial Doppler echocardiography for assessing banding procedures. Methods. Six consecutive infants (3 male, 3 female; median age, 17 days) who underwent pulmonary artery banding for complex defects were studied by intraoperative epicardial Doppler echocardiography. Results. Excellent visualization of the relation of the band to the pulmonary valve and branch arteries was obtained, enabling or confirming optimal positioning. Doppler echocardiographic estimation of the pressure drop across the band was possible in each patient and resulted in further tightening of the band in 2 patients (33%) with satisfactory increases in transband pressure gradients. Intraoperative echocardiographic estimates of the transband gradients (median, 57 mm Hg; range, 52 to 71 mm Hg; mean ± standard deviation, 59 ± 7 mm Hg) correlated well with those obtained at subsequent transthoracic echocardiography (median gradient, 66 mm Hg; range, 52 to 67 mm Hg; mean ± standard deviation, 63 ± 6 mm Hg). Conclusions. Epicardial echocardiography is a valuable adjunct to direct pressure and saturation measurements for adjustment of final band size and aids in confirming accurate band placement.

OBJECTIVES--A combined atrial and arterial switch procedure was performed in selected patients with congenitally corrected transposition to establish the morphological left ventricle as the systemic ventricle. Immediate and early follow up results are presented. BACKGROUND--Progressive right ventricular dysfunction and tricuspid regurgitation are common in patients with congenitally corrected transposition who undergo repair of associated lesions. A surgical procedure which re-establishes the left ventricle as the systemic ventricle should improve functional results. METHODS--Four symptomatic children aged from 9 months to 3 years 1 month (mean 2 years 3 months) with congenitally corrected transposition and ventricular septal defect underwent both an atrial and arterial switch procedure and were followed up for a mean of 12 months (range 6-21 months). RESULTS--There were no early or late deaths. Conduction abnormalities worsened in two patients. Hospital stay ranged from 8 to 17 days (mean 13 days). The cardiothoracic ratio decreased from a mean (range) of 0.65 (0.6 to 0.71) to 0.58 (0.52 to 0.6). Currently, three patients are in functional class I and one child is in functional class II. CONCLUSIONS--The combination of an atrial and an arterial switch procedure in symptomatic children with congenitally corrected transposition establishes the left ventricle as the systemic ventricle. The initial experience is encouraging with excellent immediate and early follow up results.

Background Whilst pulmonary valve replacement (PVR) in patients with repaired tetralogy of Fallot (rTOF) is shown to provide symptomatic benefit and reduction of right ventricular (RV) volumes, there are scarce data on the rate of ventricular mechanical and biological adaptation. We aimed to assess early and late post-PVR volumetric and functional changes. Methods and Results Patients with rTOF (≥16 years) for PVR were prospectively recruited for Cardiovascular Magnetic Resonance (CMR): pre-PVR (pPVR), early post-PVR at median 6 days (ePVR) and late post-PVR at median 3 years (mPVR). Fifty-seven patients with rTOF (mean age 35.8 ± 10.1 years, 38 male) were included. There was an acute reduction in indexed RV end-diastolic (EDVi), end-systolic (ESVi) volumes and mass early post-PVR, which was sustained at latest time point (RVEDVi pPVR 156.1 ± 41.9ml/m² vs ePVR 104.9 ± 28.4ml/m² vs mPVR 104.2 ± 34.4ml/m 2 and RVESVi pPVR 74.9 ± 26.2ml/m² vs ePVR 57.4 ± 22.7ml/m² vs mPVR 50.5 ± 21.7ml/m 2 ;p 2 and RVESVi≤82ml/m 2 . PVR produced a stepwise reduction in RVESVi (load independent variable) together with an improvement in corrected RVEF after restoring valve competency (which is load dependent). There was also a modest but significant improvement of LVEF, as well as reverse right atrial remodelling. Conclusions Cardiac remodelling is generally regarded as a gradual process post-PVR. We demonstrate for the first time that the major improvement in RV volumes seen at midterm follow-up have already taken place within days after surgery. This occurs with an apparent transient impairment of RVEF, although corrected RVEF more easily illustrates the immediate effect of PVR. However, RVESVi may be a more appropriate, load-independent marker that better reflects the early and sustained benefit of PVR on RV contractility.

Total anomalous pulmonary venous connection to the inferior vena cava is a rare form of total anomalous pulmonary venous connection infrequently described in the literature. We report two cases where the pulmonary venous connection was to the supradiaphragmatic portion of the inferior vena cava. In both patients, preoperative echocardiography findings were misleading, which suggested a cardiac type of total anomalous pulmonary venous connection.

To review 13 years' data from a unit for grown ups with congenital heart disease (GUCH) to understand the change in surgical practice.Records were reviewed of patients over 16 years of age undergoing surgery between 1 January 1990 and 31 December 2002 in a dedicated GUCH unit. Patients with atrial septal defects were included but not those with Marfan's syndrome or undergoing a first procedure for bicuspid aortic valves. Three equal time periods of 52 months were analysed.Of 474 operations performed, 162 (34.2%) were repeat operations. The percentage of repeat operations increased from 24.8% (41 of 165) in January 1990-April 1994 to 49.7% (74 of 149) in September 1998-December 2002. Mortality was 6.3% (n = 30). The median age decreased from 25.4 years (interquartile range 18.7) in January 1990-April 1994 to 23.9 (interquartile range 17.3) in September 1998-December 2002 (p = 0.04). The proportion of patients with a "simple" diagnosis decreased from 45.4% (74 or 165) in January 1990-April 1994 to 27.5% (41 of 149) in September 1998-December 2002 (p = 0.013). Pulmonary valve replacements in operated tetralogy of Fallot increased from one case in January 1990-April 1994 to 23 cases in September 1998-December 2002 and conduit replacement increased from five cases to 17. However, secundum atrial septal defect closures decreased from 35 cases to 14 (p0.0001). The estimated cost (not including salaries and prosthetics) incurred by an adult patient with congenital heart disease was pound2290 compared with pound2641 for a patient undergoing coronary artery bypass grafting.Despite the impact of interventional cardiology, the total number of surgical procedures remained unchanged. The complexity of the cases increased particularly with repeat surgery. Nevertheless, the patients do well with low mortality and the inpatient costs remain comparable with costs of surgery for acquired disease.

An 8-year-old boy who suffered from Hirschsprung's disease had development of tricuspid valve endocarditis that progressed to aortic root abscess formation, development of a fistulous communication between aorta and right atrium, atrial and ventricular septal defects, and a left ventricle to right atrium defect. Several surgical procedures were required. Operation consisted initially of closure of the septal defects and aortic valve repair. This was followed by homograft replacement of the aortic valve for persistent infection, and further closure of a left ventricle to right atrium fistula.

Introduction Until recently intra-operative transesophageal echocardiography (TOE) has not been feasible in neonates and small infants. TOE is routinely used intra-operatively at our institution in children >5.0 kg, below this weight epicardial echocardiography is used. Earlier surgery that includes repair of complex congenital heart disease is increasingly frequent. Aims To ascertain whether a newly available micro-TOE probe is beneficial and safe in the treatment of small infants. Methods We assessed our experience of a new miniaturised multiplane micro-TOE probe (Phillips Medical Systems) in small infants below 5.5 kg undergoing cardiac intervention. Results 21 patients, 11 male (median age 112 days, range 3–214 days), median weight 4.4 kg range 3.0–5.4 kg) underwent cardiac intervention with micro-TOE guidance. The type of intervention is summarised in table 1. Probe insertion was successful in all 21 patients. No immediate haemodynamic or clinical complications related to probe insertion or removal occurred. Diagnostic images were possible in all cases (figure 1). There was a learning curve with more structures identified in the later part of the study, in particular coronary arteries. Conclusions Infants under 5.5 kg having cardiac intervention were able to undergo intra-operative micro-TOE without complication. These patients need no longer be excluded from advanced high quality intra-operative imaging during cardiac surgery.

Summary Background Traditionally, after prenatal diagnosis of hypoplastic left-heart syndrome (HLHS) couples have been offered termination of pregnancy or comfort care. Success of postnatal surgical options such as the Norwood procedure have been associated with survival of up to 60%. Whether survival is affected by the congenital anomaly being identified prenatally or postnatally remains uncertain. Methods We reviewed all cases of prenatally diagnosed HLHS referred to the Fetal Medicine Unit at Birmingham Women's Hospital over 6 years between 1994 and 1999. Findings 87 cases of HLHS were referred at a median gestational age (95% CI) of 23 (19–37) weeks. Of these, 53 (61%) chose prenatal karyotyping. The overall frequency of abnormal karyotype was found in seven of 59 cases (12%) and associated structural anomalies in 18 of 87 (21%). After counselling, 38 of 87 couples (44%) chose termination of pregnancy. Of the remaining 49 fetuses, 11 (23%) were not considered for postnatal surgery because of parental choice and they died after compassionate care. Of the 36 babies who had surgery postnatally, 12 survived (33%). We recorded a survival rate of 38% for the stage-1 Norwood procedure in the prenatally diagnosed HLHS in our centre. These data suggest that at the point of prenatal detection, the overall survival rate for fetuses with HLHS is 25% (if terminated pregnancies are excluded). Interpretation Fetal echocardiography allows early diagnosis of HLHS and gives clinicians the opportunity to triage this group dependent on prenatal findings, including karyotyping and the exclusion of other structural anomalies. These prospective data provide up-to-date information on the basis of which parents can make decisions.

OBJECTIVES: To assess the current results and outcome of surgery in infants and children with right atrial isomerism and complex congenital heart disease. SETTING: Tertiary referral centre. METHODS: 20 consecutive children with right atrial isomerism and complex congenital heart disease underwent surgery over a 6 year period between August 1987 and July 1993. The results and outcome were analysed according to age, presentation, and surgical procedures. RESULTS: Patients were divided into two groups depending on age at presentation and initial surgery: group A comprised 11 patients who required surgical intervention in the first month of life (mean age 5 days); and group B comprised nine patients who required initial surgical intervention after the first month of life (mean age 6.8 months). Seven (64%) of the 11 patients in group A had obstructed pulmonary venous drainage and ten (91%) had pulmonary atresia. There were seven early deaths (64%), including the five patients who required systemic to pulmonary artery shunt and simultaneous repair of obstructed pulmonary veins. The long-term survival rate in this group was 18% (two of 11). Pulmonary venous obstruction was present in two (22%) of the nine patients in group B and four (44%) had pulmonary atresia. There were no early deaths. One patient died after a second palliative procedure. There was one late sudden death. Four patients had a Fontan operation with no deaths. Two of the remaining three patients meet the Fontan criteria. The long-term survival rate in this group was 78% (seven of nine). CONCLUSIONS: Surgical management of patients with right atrial isomerism who have complex congenital heart disease carries a high mortality and remains palliative. The overall survival rate was 45% (nine of 20); 18% in patients requiring surgery in the first month of life (group A) and 78% in patients requiring surgery after the first month of life (group B) (P < 0.001). Of the total of 20 patients, nine were potential candidates for a Fontan operation. Seven of these have undergone a Fontan procedure with five survivors.

Major associated cardiac anomalies are known to increase the risk of repair of complete atrioventricular septal defects (CAVSDs). The purpose of this study was to examine the effects of such anomalies on the current surgical management of CAVSDs and their influence on outcome following repair.We performed a retrospective review of a 100 consecutive non-isomeric patients undergoing repair of CAVSD at our institution, between January 1989 and December 1994; patients with partial or intermediate defects were excluded. Complete atrioventricular septal defect patients with other major cardiac abnormalities (complex) were then compared to those with isolated CAVSDs.There were 15 patients (15%) with associated anomalies; 3 had tetralogy of Fallot, 1 patient had pulmonary atresia, 6 had hypoplastic left or right ventricle, 1 had tetralogy of Fallot and hyperplastic right ventricle, 2 patients had double outlet right ventricle, 1 had hypoplastic aortic arch and 1 patient had aortic coarctation. The median age at operation was similar for both groups (4.2 months), while the median weight was not significantly different for isolated CAVSDs compared to complex (4.2 months vs 3.4 months, P = 0.89), but there was a higher incidence of trisomy 21 (70/85, 82% vs 8/15, 53.3%, P = 0.01). Two of the 85 isolated CAVSD patients (2.3%) had undergone palliative pulmonary artery banding, while 5 of the 15 complex patients (33.3%) had either banding or Blalock-Taussig shunts performed. The technique of CAVSD repair was identical in each group. All complex patients had standard repair of their associated anomalies. Hospital mortality was higher in the complex group (3/15, 20% vs 2/85, 2.3%, P = 0.004); all early deaths in the complex group occurred in patients with a hypoplastic ventricle. Reoperation for left atrioventricular valve regurgitation was required in six isolated CAVSD patients (7.1%) and in one complex (6.6%).In the absence of significant ventricular hypoplasia, the early results of surgical repair in patients with CAVSDs and associated cardiovascular anomalies are similar to those achieved in patients with isolated CAVSD.

In 24 consecutive infants (19 male and 5 female) with complex forms of single-ventricle physiology and systemic outflow obstruction, a modified Damus operation without the use of exogenous material was undertaken in conjunction with creation of an aortopulmonary shunt 3.5 mm in diameter. The median age at operation was 6 days (range, 1 to 170 days) and the median weight, 3.4 kg (range, 2.6 to 4.6 kg). There were nine early deaths. All 15 survivors (median follow-up, 6.5 months) were clinically well without major systemic ventricular dysfunction or atrioventricular or arterial valve regurgitation. Ten of them have undergone a superior vena cava—pulmonary shunt (one death), and 1 has required patch angioplasty of the aortic arch and innominate artery with revision of the aortopulmonary shunt. The 4 other survivors are awaiting a cavopulmonary shunt. Univariate analysis yielded the chronologic rank for an individual procedure (higher risk of death early in the series), presence of aortic arch atresia, and presence or absence of transposition of the great arteries as predictors of death. This aggressive surgical approach provides excellent early palliation, and because the operation prevents abnormal ventricular hypertrophy from pressure or volume overload, systemic ventricular function is optimally conserved for a future Fontan-type procedure.

OBJECTIVE--Prospective audit of the first year of implementation of a modified approach to palliation for infants with hypoplastic systemic ventricle and severe systemic outflow obstruction. SETTING--Tertiary referral centre for neonatal and infant cardiac surgery. PATIENTS AND METHODS--17 of 19 infants (aged < 35 days) presenting to Birmingham Children's Hospital in 1993 with hypoplastic systemic ventricle and severe outflow obstruction underwent surgery. This was performed using a new modification of the Norwood-type arch repair, without the use of exogenous material, and a 3.5 mm Gore-tex shunt between the innominate and right pulmonary arteries. The Gore-tex shunt was replaced by a cavopulmonary shunt between 3 and 5 months later. Clinical, morphological, and functional determinants of outcome were examined. RESULTS--10 (59%) infants survived initial surgery. All proceeded to cavopulmonary shunt without further loss. Significant atrioventricular valve regurgitation seemed to be the main risk factor for poor outcome. If this was excluded, the morphology of the dominant ventricle seemed to have little effect on the outcome of initial surgery. CONCLUSIONS--Early survival was achieved in 59% of patients in the first year of implementation of a protocol for surgery in infants with hypoplastic systemic ventricle and severe outflow obstruction. The construction of a neoaorta without the use of exogenous material may allow improved later growth of the neoaorta. Early cavopulmonary shunt can be performed safely and should reduce mid-term complications from cyanosis and systemic ventricular volume loading.

Significant post-operative bleeding may occur following cardio-pulmonary bypass, sometimes necessitating early re-exploration. Two questions were asked by this study. Firstly, in children undergoing cardio-pulmonary bypass, can pre-operative, operative and immediately post-operative variables be used to predict post-operative blood loss? Secondly, in the same patient group, can the same variables be used to predict the surgical intervention of re-exploration for excessive bleeding within 12 h of surgery? In a prospective study of patients operated on in a 9-month period, bleeding fell rapidly in the first 3 h after surgery and early re-exploration for bleeding was necessary in 8 of the 206 patients. The only significant predictor of post-operative blood loss was minimum oesophageal temperature, low values being associated with greater blood loss (correlation r = -0.19 (P < 0.05)). With regard to re-exploration for bleeding, a long bypass time (P < 0.05) and abnormal pre-operative coagulation indices (P < 0.01) were significant and independent predictors. The data were also used to create a practical nomogram relating re-exploration for bleeding to hourly post-operative blood loss.

Objective: This study was undertaken to determine the outcomes of patients with congenitally corrected transposition of the great arteries after restoration of the morphologically left ventricle to the systemic circulation. Methods Between November 1991 and June 2001, a total of 54 patients (median age 3.2 years, range 7 weeks–40 years) with either congenitally corrected transposition of the great arteries (n = 51) or atrioventricular discordance with double-outlet right ventricle (n = 3) underwent anatomic repair. This comprised a Senning procedure in all cases plus arterial switch (double-switch group) in 29 cases (53.7%), plus a Rastelli procedure (Rastelli-Senning group) in 22 cases (40.7%), and plus intraventricular rerouting (Senning-tunnel group) in 3 cases (5.6%). Left ventricular training by PA banding was performed before the double-switch operation in 9 of 29 cases (31%). Follow-up is complete (median 4.4 years). Results Early mortality was 5.6% (n = 3), with 2 late deaths. Kaplan-Meier survivals (± SEM) were 94.4% ± 3.1% at 1 year and 89.7% ± 4.4% at 9 years. Survivals at 7 years were 84.9% ± 7.1% in the double-switch group and 95.5% ± 4.4% in the Rastelli-Senning group ( P = .32). Of the 49 survivors, 46 (94%) were in New York Heart Association functional class I. Six have acquired new left ventricular dilatation or impaired systolic ventricular function. Four patients in the double-switch group had moderate aortic valve regurgitation develop, and 2 of them required valve replacement. Overall freedoms from reoperation at 1 and 9 years were 94.2% ± 3.3% and 77.5% ± 9.0%, with no significant difference between the groups ( P = .60). Conclusions Anatomic repair of congenitally corrected transposition of the great arteries can be carried out with low early mortality. Excellent functional status can be achieved, with good midterm survival. Continued surveillance is necessary for patents with valved conduits and to determine the longer-term function of the aortic valve and the morphologically left ventricle in the systemic circulation.

Prior to 1970, most patients with tricuspid atresia (TA) and reduced pulmonary blood flow were palliated by means of a systemic to pulmonary artery shunt (SPAS). In Birmingham between 1967 and 1988, 19 patients with TA (aged 16 days to 23 years, mean 3.8 years) have undergone side-to-side cavopulmonary anastomosis (CPA) with preservation of pulmonary artery continuity. One had undergone a previous right Blalock-Taussig shunt and in 1 patient with left and right SVCs, bilateral CPAs were performed. Nine patients were less than 1 year old at the time of operation. There were no operative deaths. One late death occurred due to pneumonia. Immediate clinical improvement occurred in all cases. Six symptomatic patients subsequently required further surgery (mean interval between operations 8.9 years, range 4.6-14.2 years). The remaining 12 patients have recently been reviewed. Mean follow-up was 9.5 years (range 5 weeks to 21 years). Exercise tolerance remains good (9 in NY-HA Class 1 or 2) and resting transcutaneous oxygen saturations are higher (mean 85%, range 70%-89%) compared with preoperative values (P less than 0.01). In small children, CPA avoids the complications of SPAS and, in some patients, may itself achieve adequate long-term definitive palliation.

Contrast radiology has valuable applications in routine necropsy practice. Contrast radiology was used to study the vascular anatomy in four normal fetuses, in five children with cardiovascular malformations, and in two hearts prepared by the perfusion fixation method. A contrast radiograph provides permanent documentation of anatomical associations before they are disrupted by dissection and permits a planned approach to the necropsy. While these injection studies are seldom undertaken by pathologists because they are felt to be too difficult and time-consuming, contrast studies should form part of the post mortem investigation of children with congenital heart disease, even when angiography has been performed in life.