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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.

Objective: 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. Methods: 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. Results: 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%, P < 0.0001) 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 = 0.004). 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. Conclusions: 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.

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)

Background Posttransplantation Epstein-Barr virus-associated lymphoproliferative disease (PTLPD) occurs as a spectrum of disease ranging from benign, polyclonal, localized lymphoid hyperplasia to malignant, monoclonal, disseminated lymphoma, sometimes involving the bone marrow. To our knowledge, PTLPD has not been previously reported to present as acute lymphoblastic leukemia. Methods We report the case of a boy who developed PTLPD in the form of acute lymphoblastic leukemia 6 years after cardiac transplantation. He had greater than 90% bone marrow invasion by Epstein-Barr virus-positive B lymphoblasts with Burkitt-like features and a t(8;14) translocation. Results He was successfully treated with combination chemotherapy but unfortunately died, 6 months after completing treatment, from ischemic heart disease. Conclusions B lymphoblastic leukemia may occur as a manifestation of PTLPD and should be included in the classification of these diseases. Bone marrow examination should be an essential part of the investigation of patients suspected of having PTLPD.

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.