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Background Due to increasing success with repair or palliation in childhood, there is a rapidly growing population of adult patients with complex congenital heart disease who may require transplantation. There remains little data on outcomes of cardiac transplantation in this group. Methods 38 orthotopic cardiac transplants were performed in 37 patients (18 men) ≥18 years of age with congenital heart disease (CHD) from 1988 to 2009 in our institution. Outcomes were reviewed using medical records and transplant databases. Results 15 patients (41%) had univentricular and 22 (59%) biventricular physiology. The biggest group was transposition of the great arteries following atrial switch in eight patients (22%). Six (16%) had no previous surgical intervention. Mean age at transplant was 33.5 years (range 19.1–59.9 years). 11 patients (30%) required additional surgical procedures at transplant. 16 (43%) died, 12 early and 4 late deaths (1.8, 2.4, 2.7 and 7 years). Survival was 70% at 30 days, 68% at 1 year, 58% at 5 years and 53% at 10 and 15 years. Outcome improved in later eras with reduction in 30-day mortality from 50% to 18% and increase in 5-year survival from 50% to 69%. Two patients developed post-transplant lymphoproliferative disease. None required long-term renal replacement therapy. One patient was re-transplanted for cardiac allograft vasculopathy. Conclusions While operative mortality following cardiac transplantation for adult congenital heart disease is higher than for other diagnostic groups, long-term survival is good and comparable to patients without CHD. Disappointing early results are improved with increasing experience.

We sought to investigate the accuracy of dobutamine stress echocardiography to predict the degree and timing of recovery in resting function and contractile reserve (CR) after revascularization of the hibernating myocardium.In all, 24 patients with ischemic cardiomyopathy (ejection fraction40%) underwent dobutamine stress echocardiography 1 week before and 6 weeks, 3 months, and 6 months after coronary artery bypass grafting.Recovery rates at 6 weeks, 3 months, and 6 months postoperation were 21%, 33%, and 45% (P.01) for resting function and 55%, 65%, and 74% (P.01) for CR. Positive and negative predictive values for recovery of resting function and CR at 6 months postrevascularization were 66% vs 97% (P.001) and 78% vs 48% (P.001), respectively. Positive and negative predictive values were both high for recovery of CR at 6 weeks postrevascularization (89% and 78%).Dobutamine stress echocardiography can predict early recovery in CR postrevascularization with an excellent accuracy but may underestimate the degree of late recovery in CR.

Incompatibility of the major blood groups A, B, and O has been an absolute contraindication for heart transplantation. However, because of immunologic immaturity, infants may have relative protection from hyperacute rejection and thus could undergo transplantation with ABO-mismatched organs.Since January 2000, the authors have adopted a policy of considering infants for ABO-incompatible heart transplantation. Serum isohemagglutinin titers were measured before, during, and after transplantation. Two infants (3 and 2 months old) and a 21-month-old child underwent ABO-incompatible heart transplantation. During cardiopulmonary bypass, plasma exchange was performed. No other antibody-removal procedures were performed. A routine immunosuppressive regimen was used, and rejection was monitored by endomyocardial biopsies. An additional two patients (31 and 18 months old) were worked up but were unsuitable for ABO-incompatible transplantation because of high isohemagglutinin titers. They were successfully bridged to transplantation and received heart transplants from ABO-compatible donors.All three infants with ABO-incompatible heart transplants are fit and well, 40 months, 30 months, and 12 months postoperatively. All three had serum antibodies to antigens of the donor's blood group before transplantation. No hyperacute rejection occurred. No morbidity attributable to the ABO incompatibility has been observed.ABO-mismatched heart transplantation may be undertaken safely and without any short-term adverse consequences in infants and young children in whom isohemagglutinin production is not yet established.

Fifty one years have passed since grossand Crafoord independently reported successful repair of aortic coarctation. One could be forgiven for assuming that all the surgical controversies have now been settled. This is far from the case, as the numerous publications (frequently with opposing view points) related to timing, technique and complications bear testament. Perhaps the passage of the golden anniversary of this operation should stimulate a degree of reflection among surgeons and cardiologists.

There are no data comparing patient attitudes to sternotomy and thoracotomy scars following surgery for congenital heart disease (CHD).Two hundred and one patients with a scar from CHD surgery (105 sternotomy, 36 thoracotomy, and 60 both scars) had a structured interview to explore attitudes to their scar.Comparable proportions of each group reported that they did not like or hated their scar (23/105 [22 %] sternotomy, 9/36 [25 %] thoracotomy, 17/60 [28 %] both scars). Significantly more patients stated that they where embarrassed by and/or their choice of clothing was affected by a thoracotomy scar (20/36, 56 %) than those with a sternotomy scar (36/105, 34 %), p = 0.04. This was also seen when comparing sternotomy alone with both scars (36/105 [34 %] vs. 34/60 [57 %], p = 0.008).Adults who have undergone surgery for CHD are more likely to have a negative attitude to a thoracotomy than a sternotomy scar. Before a change in surgical approach is considered based on patient preferences, the acceptability and psychological impact of the different scars following surgery needs formal study.

Anomalous origin of the left coronary artery from the pulmonary artery requires surgical correction. A surgical technique is presented. Two infants underwent re-implantation of their anomalous left coronary arteries by creating a tunnel using autogenous aortic and pulmonary arterial walls. The advantage of this technique is that the new left coronary artery lies in the horizontal plane and in an anatomically correct axis running in the groove behind the pulmonary artery. It also provides a tension free endothelialized autogenous arterial walls with normal growth anticipated.

The Ross procedure for aortic valve replacement was introduced at two paediatric cardiac centres (Freeman Hospital, Newcastle, and Royal Liverpool Children's Hospital) where surgeons had no previous experience of this technique. A structured programme of training and cooperation between the two centres enabled surgeons to minimise the learning curve for this procedure. We share our experience and suggest options for surgeons who wish to undertake new procedures in the current era of surgical practice and clinical governance. Issues of informed consent and risk stratification remain. #### Summary points Surgeons have always recognised the concept of a learning curve when undertaking a new procedure Recent events mean that there is a lack of professional and public tolerance for suboptimal results due to a learning curve Learning a new technique, even for an established consultant, requires some sort of learning curve Introducing a new procedure in a structured way that incorporates formal training courses, cadaveric resection, and assistance from expert practitioners can reduce the learning curve The recent General Medical Council (GMC) inquiry into the Bristol Paediatric Cardiac Surgical Unit highlighted many concerns common to all surgical specialties. At the end of the judgment, the president of the GMC listed 13 issues that the professions should address, and the senate of surgery recently published its response.1 The concept of a learning curve, which has long been recognised by surgeons, was of particular consequence. However, the senate responded that “there should be no learning curve as far as patient safety is concerned.”1 To learn to perform a new procedure without having a learning curve is a dilemma that we, as established consultants in paediatric cardiac surgery, faced when we wanted to introduce the Ross procedure into our surgical practice. We discussed strategies which we hoped would reduce the steepness of our learning curve, and …

Over a 2-year period (1987-1988), 31 children (3% of 960 operations) were found to have a paralysed diaphragm following cardiac surgery. The median age was 5 months with 65% less than 1 year of age. In the 31 patients, 38 paralysed diaphragms were identified. The phrenic nerve injury was on the right in 8 cases, the left in 16, bilateral in 7 and 40% were related to modified Blalock Taussig shunts. The time to extubation was analysed for each patient in two separate groups: Group A: (greater than 1 year): 11 children of whom 5 were extubated without difficulty, 4 had non-respiratory reasons for prolonged ventilation and only 2 were plicated--both were extubated within 4 days. Group B: (less than 1 year): 20 infants of whom 16 were plicated and 4 were not. Three of the children who were plicated died from cardiac causes. Of the 13 survivors, there were 3 who had other reasons for prolonged ventilation. Thus 10 infants required prolonged ventilation (mean 11 days) because of respiratory difficulties. All underwent plication and were extubated at a mean of 2.4 days postoperatively. The 4 who were not plicated were extubated at a mean of 11 days postoperatively. In infants in whom there is no cardiac cause for failure to wean from ventilation, diaphragmatic paralysis should be suspected and plication performed if not extubated 2 weeks after operation.

Although ABO blood group incompatible cardiac transplantation in neonates and infants reduces waiting list mortality without compromising outcome, the technique has not been adopted by all centers, and to date Toronto remains the only center to have published results from a large case series. We present a review of ABO-incompatible heart transplantation in the United Kingdom (UK) where current recipient selection criteria differ somewhat from those used in the United States (US) and Canada. Between February 2000 and November 2006, 21 ABO-incompatible cardiac transplants were performed in children aged 2-40 months (median 10.0). Immunosuppression followed standard regimens. Pretransplant donor-specific isohemagglutinins of >1:4, (the UNOS cutoff), were present in five patients and reduced by plasma exchange. After transplantation, 19/21 recipients demonstrated persisting deficiency of donor-specific isohemagglutinins. Significant donor-specific isohemagglutinins levels were detected repeatedly in 2/21 recipients who have shown no clinical or biopsy evidence of rejection. All recipients survive without retransplantation and there have been no episodes of humoral rejection. We conclude it is possible for other centers to replicate the excellent results achieved in Toronto and that ABO-incompatible transplantation may be performed successfully in some patients beyond infancy with established isohemagglutinin production providing preoperative antibody removing strategies are used.

Fig. 1. Transverse midesophageal plane at 458, demonstrating the short axis of the aortic valve in diastole. The ruptured aneurysm of the right coronary sinus protrudes into the right atrium just above the tricuspid valve producing the characteristic ‘windsock’ appearance described in textbooks. Colour-flow Successful surgical repair of a ruptured sinus of Valsalva aneurysm was performed in a 30-year-old man, presenting with acute onset dyspnoea. Transoesophageal echocardiography demonstrated the characteristic ‘windsock’ appearance of the aneurysm described in textbooks and the continuous flow between the aorta and the right atrium corresponding to a continuous murmur. During surgery an 8 mm opening was identified in the right coronary sinus leading into the right atrium, the aneurysm tissue in the right atrium was excised and the base oversewn, whilst a patch of pericardium was used to close the defect from the aortic end (Figs. 1 and 2). Doppler demonstrates continuous blood flow through the ruptured aneurysm between the aorta and the right atrium, corresponding to the continuous murmur. RA (right atrium), LA (left atrium), RV (right ventricle), TV (tricuspid valve), PV (pulmonary valve), PA (pulmonary artery), LCC (left coronary cusp), NCC (non-coronary cusp), RCC (right coronary cusp). Appendix. Supplementary material

To compare the outcome of life insurance and mortgage applications of adults with congenital heart disease (CHD) with controls and at different severities of CHD.Two hundred and ninety-nine adult CHD patients underwent a questionnaire-based interview by a trained nurse. They were asked to give an identical questionnaire to a friend to act as a control. One hundred and seventy-seven controls replied. CHD patients were classified into three categories based on severity. Comparisons were made between matched controls and between different severities of CHD.Similar proportions of the CHD group (59%) had applied for life insurance as matched controls (56%). Compared to controls, significantly more of the adults with CHD who had applied for life insurance have been refused (34 vs 4%, P0.0001) or asked to pay extra (37 vs 6%, P = 0.0002). Mortgage application rate was also similar in both groups with more of the CHD patients refused than matched controls (20 vs 3%, P = 0.0004). These differences in both life insurance and mortgage remain significant when the cases and controls are matched by employment status and NYHA functional class. There was no significant difference in life insurance and mortgage application outcome between the groups of mild, significant and complex CHD.Adults with CHD are significantly more likely to have difficulty obtaining life insurance or a mortgage than controls. Refusal rates appear to be independent of the severity of CHD. This suggests that the label of CHD may have a negative impact despite the lesion being minor and that the outcome of an individual application is difficult to predict based on the severity of the CHD. The increasing numbers of adults with CHD suggest that this problem is likely to increase and needs to be addressed as it can have a major impact on the patient's quality of life.

Objectives: Paraplegia following coarctation repair occurs in 0.4% of infants. However, for older children, adults and re-operations, the incidence can be as high as 2.6%. Yet there is no consensus on the need for spinal cord protection or the optimal method. This paper reports our experience with left heart bypass (LHB) in adult and re-do coarctation. Methods: Between 1997 and 2000, nine patients underwent elective resection of coarctation (three re-dos, two balloons) with a mean age of 17.9 years (range, 8–44) and weight of 52 kg (range, 17.3– 109). The mean trans-coarctation gradient was 29.6 mmHg (range, 20–45). Patients were placed on LHB using a centrifugal pump with full heparinization through a fourth-space thoracotomy. Patients were cooled to 31–348C for additional spinal cord protection. Repair was carried out with an inter-positional graft (5/9), a Gore-Tex patch (2/9) or end-to-end anastomosis (2/9). The mean cross-clamp and bypass times were 36.4 (range, 19–65) and 40.3 min (range, 22–70), respectively. Results: No patient developed transient or permanent paraplegia. The mean peak creatinine was 80 mmol/l (range, 51–123). Conclusions: LHB is simple, easy and safe to implement, and is the only technique capable of maintaining independent upper and lower body perfusion pressure. Potentially, it provides the best spinal cord protection, and extends the margin of safety and time to execute an accurate repair. q 2001 Elsevier Science B.V. All rights reserved.

Background . In the absence of reliable national data, we have collected results of all operations for congenital heart defects from five departments to assess mortality rates and compare them among surgeons and departments. Methods . Data relating to all operations (2,718) carried out at the five centers during a period from April 1, 1997 through March 31, 1999. Clearly defined criteria were agreed for the classification of patients into various subgroups. Results . The overall hospital mortality was 4.4% (95% confidence intervals 3.7%–5.3%). Mortality for open operations was 12.6% in neonates, 5.1% in infants, and 3.5% in children. Mortality rates were 1.1% for coarctation, 0.4% ventricular septal defect, 4.1% atrioventricular septal defect, 2.9% Fallot, 0.9% switch, and 15.6% truncus arteriosus. Although individual surgeons' mortality rates ranged from 1.8% to 7.5%, none of the 12 surgeons' data were above 95% confidence intervals. For individual surgeons, the change in mortality rates between the 2 years ranged between −3.3% and +3.8%. Conclusions . With 2 years' data available, estimates of mortality rates are more precise as reflected by tighter confidence intervals. There were relatively small data sets for individual hospitals and surgeons, which made statistical evaluation difficult. For setting standards, data from more departments for a longer period will be required. Statistical methods alone cannot be used as a sole arbiter of what is considered acceptable performance.

Summary Background A public inquiry into surgery for paediatric congenital heart defects in Bristol, UK, underscored the need for reliable data on overall mortality rates, which would allow assessment of individual surgeons' performance. We aimed to gather and report such data for 1 year to provide information for clinicians, researchers, policy makers, and the general public. Methods We collected data on all operations (1378) for congenital heart defects done by 11 surgeons in five departments in the UK between April 1, 1997, and March 31, 1998. These operations represented about 36% of all operations done in the UK during that time. Clearly defined criteria were agreed to classify operations into subgroups. Findings The overal mortality rate for all operations was 4·0% (95% Cl 3·0–5·2). No deaths occurred for 67 arterial-switch operations. Mortality rates for coarctation, ventricular septal defect, atrioventricular septal defect, Fallot, and truncus arteriosus operations were 1·1%, 0·6%, 3·6%, 2·3%, and 28·6%, respectively. Although overall mortality rates between surgeons varied (1·6–6·9%), no surgeon's were higher than the 95% Cl. The numbers of operations done by individual surgeons were small, which led to wide confidence intervals and made the detection of differences in performance difficult. Interpretation The participating departments seemed to reach high standards of care for children with congenital heart defects, although more data would be needed to assess performance of individual surgeons. The development of quality standards will be difficult because of the complexity of defects, the different types of operations, and few patients in each subgroup. Collection of larger sets of data for more patients and centres are needed.

Cyclosporin A is the primary immunosuppressive agent used in cardiac transplantation to maintain chronic immunosuppression. Absorption may be erratic and major side-effects include nephrotoxicity. A recently introduced formulation (Neoral) improves absorption in cystic fibrosis heart-lung transplant recipients, but episodes of rejection have been reported on conversion from the oil-based formulation. A retrospective analysis of 21 paediatric cardiac transplant recipients who had been converted from the oil-based formulation to the micro-emulsified formulation was performed. No clinical rejection episodes occurred following conversion. There was a significant reduction in dose following conversion (p < 0.001). The mean trough level was less on the new formulation (p = 0.04), but there was no difference in coefficient of variation or standard deviation. Mean glomerular filtration rate (GFR) was significantly less, but there was no difference in the rate of change of GFR on the new formulation. Erratic absorption and deteriorating renal function remain as significant problems in paediatric cardiac transplant recipients despite conversion to the new formulation.

Epstein-Barr virus (EBV)-associated post-transplant lymphoproliferative disease (PTLD) is a serious complication of organ and bone marrow transplantation. The importance of EBV matching between recipient and donor remains unclear. Between October 1987 and December 1997, 64 pediatric cardio-pulmonary transplants were performed at this center (58 hearts, two heart/lungs, four sequential single lungs). The EBV viral capsid antigen (VCA) immunoglobulin G (IgG) status of both donor and recipient was determined at the time of transplant. Of 56 patients from whom paired sera was available for analysis, 27 (50%) were recipient and donor EBV IgG positive, four (7%) were recipient EBV IgG positive and donor EBV IgG negative, and 12 (20%) were recipient EBV IgG negative and donor EBV IgG negative. The remaining 13 (23%) patients were EBV IgG negative but received organs from EBV IgG-positive donors. The EBV immunoglobulin M (IgM) status was determined from 6 weeks post-transplant in the 11 mismatches who survived for longer than 28 d. Seven became EBV IgM positive, two remained EBV IgM negative; the status of the remaining two remains unknown. The EBV IgM status was also determined retrospectively in patients who were EBV IgG negative pretransplant and received organs from EBV IgG-negative donors. Nine became EBV IgM positive; the rest remained negative. PTLD was diagnosed in two of 56 patients from whom paired sera was available; one was donor and recipient EBV IgG negative; the other was donor and recipient EBV IgG positive. No cases of PTLD were diagnosed in the remaining eight patients from whom paired sera was not available. Our experience suggests that PTLD does not occur with any greater frequency in the 'mismatch' group, and does not justify EBV matching in pediatric thoracic transplantation where there is a higher proportion of EBV-negative recipients than in adults.

Background —The technical aspects of ductal stenting have been reported, but little is known of the fate of the duct after stent implantation. Methods and Results —Nineteen patients underwent stent implantation to maintain ductal patency. Eight had hypoplastic left heart (HLH) syndrome, 10 had pulmonary atresia, and 1 had tricuspid atresia. Median survival with HLH was 57 (12 to 907) days. Stent implantation was successful in all cases of HLH, but there were no long-term survivors. Two well-palliated infants died at transplantation. Median survival with duct-dependant pulmonary flow was 183 (0 to 1687) days, with 3 patients well at latest follow-up (56, 55, and 9 months, respectively). There were 2 operative deaths due to ductal spasm and 4 late deaths, 1 due to duct thrombosis, 1 due to chronic lung disease, and 2 of unknown cause. Stent implantation failed in 4 of the 11 cases. Assessment of endothelialization was possible in 13 cases; the stent was partially covered in 3 and fully endothelialized in all 10 cases assessed >8 weeks after implantation. In patients stented for inadequate pulmonary flow, ductal intimal hyperplasia occurred by 9 months in all 3 survivors but responded to repeated dilation. Conclusions —Ductal stenting cannot be recommended. In patients with HLH, it provides only short-term palliation even when combined with pulmonary artery banding. With duct-dependent pulmonary blood flow, the procedure carries high risk, and duration of palliation is poor. In patients with bilateral ducts and absent central pulmonary arteries, good palliation may be achieved, but repeated angioplasty is necessary to counteract intimal hyperplasia.

Heart transplantation for congenital heart disease poses unique problems because of structural anomalies and previous corrective and palliative surgery. In the period between May 1985 and February 1992 a total of 231 orthotopic heart transplants were performed at our hospital -22 of these procedures were carried out in patients with congenital heart disease. The patient's ages ranged from 1 month to 51 years (median 10 years). There were 13 patients in the paediatric group (under 16 years) and 9 patients in the adult group (16 years or older). The diagnoses included univentricular connections (8 patients), complex morphology (4 patients), congenitally corrected transposition of the great arteries (TGA) (3 patients), hypoplastic left heart syndrome (2 patients), Fallot's tetralogy, tricuspid atresia, TGA, pulmonary atresia with intact ventricular septum and atrial septal defect (ASD) and ventricular septal defect (VSD). These patients had protected pulmonary circulation due to previous surgery or as a result of intrinsic pulmonary stenosis. Seventeen patients (77%) had undergone 29 prior operations (21 palliative and 8 corrective) including Blalock-Taussig shunts, pulmonary artery (PA) banding, ASD and VSD repair, Fontan procedure and Mustard operation. There were 2 early deaths in the paediatric group and 4 early deaths in the adult group, but no late deaths. The cause of death was multi-organ failure following uncontrollable haemorrhage in 3 patients, sepsis in 1 patient and donor organ failure in the 2 paediatric patients. Specific surgical manoeuvres were required to create the normal anatomical configuration. These included the rerouting of venous circulation, pulmonary artery reconstruction and atrial septation. Adequate donor tissue was taken to permit satisfactory reconstruction.(ABSTRACT TRUNCATED AT 250 WORDS)

A 34 year old woman presented with increasing shortness of breath, and a secundum atrial septal defect (ASD) was diagnosed by transthoracic echocardiography. A preoperative transoesophageal echocardiogram demonstrated a large secundum ASD with left to right shunt, and a dilated right ventricle (RV) with a pulmonary artery systolic pressure …

In six infants with pulmonary atresia and intact ventricular septum operative balloon dilatation was used to achieve continuity between the right ventricle and the main pulmonary artery as the initial procedure. Two of the six subsequently needed an aortico pulmonary shunt. All six are alive and well.

A case of chylopericardium occurring after a Waterston shunt was successfully managed by pericardial drainage of chyle and substitution of dietary fats with medium chain triglycerides.

Recent legislation has led to increasing numbers of car occupants wearing seat belts and presumably the number of injuries attributable to seat belts will also increase. The commonest such injury is a fracture of the sternum, and blunt myocardial damage is a logical sequel although often unsuspected and therefore unsought. Confirmation is difficult and ECG changes are usually absent or not specific. In three consecutive patients admitted with a fractured sternum we found raised blood levels of CK-MB (creatine kinase-myocardial band) and believe these indicate myocardial contusion. We therefore feel that an isolated fracture of the sternum should be considered a more serious injury than is initially apparent, as these patients are at risk of developing cardiac complications.