Paediatric cardiac surgical mortality in England after Bristol: descriptive analysis of hospital episode statistics 1991-2002

Objective To describe trends in mortality of open cardiac surgery in children in Bristol and England since 1991.Design Retrospective analysis of hospital episode statistics data.Setting All open cardiac surgery of children in England.Population Patients younger than 16 undergoing open cardiac surgical procedures in England between April 1991 and March 2002. Three time periods were defined: epoch 3 (April 1991 to March 1995), epoch 5 (April 1996 to March 1999), epoch 6 (April 1999 to March 2002).Main outcome measure Mortality in hospital within 30 days of a cardiac procedure.Results We identified 5221 open operations between April 1996 and March 2002 in children under 1 year and 6385 in children aged 1-15 years. Mortality for all centres combined fell from 12% in epoch 3 to 4% in epoch 6. Mortality in children under 1 year at Bristol fell from 29% (95% confidence interval 21% to 37%) in epoch 3 to 3% (1% to 6%) in epoch 6, below the national average. The reduction in mortality did not seem to be due to fewer high risk procedures or an increase in the numbers of low risk cases. Oxford had a significantly higher mortality than the national average in all three epochs (11% (5% to 18%) in epoch 6), which was not affected by adjusting for procedure or the inclusion of cases with missing outcomes.Conclusions At Bristol, mortality for open operations in children aged under 1 year has fallen markedly, to below the national average. Nationwide mortality has also fallen. Improved quality of care may account for the drop in mortality, through new technologies or improved perioperative and postoperative care, or both.     

Surgeon specific mortality in adult cardiac surgery: comparison between crude and risk stratified data

Objective As a result of recent failures in clinical governance the government has made a commitment to bring individual surgeons'' mortality data into the public domain. We have analysed a database to compare crude mortality after coronary artery bypass surgery with outcomes that were stratified by risk.Design Retrospective analysis of prospectively collected data.Setting All NHS centres in the geographical north west of England that undertake cardiac surgery in adults.Participants All patients undergoing isolated bypass graft surgery for the first time between April 1999 and March 2002.Main outcome measures Surgeon specific postoperative mortality and predicted mortality by EuroSCORE.Results 8572 patients were operated on by 23 surgeons. Overall mortality was 1.7%. Observed mortality between surgeons ranged from 0% to 3.7%; predicted mortality ranged from 2% to 3.7%. Eighty five per cent (7286) of the patients had a EuroSCORE of 5 or less; 49% of the deaths were in this lower risk group. A large proportion of the variability in predicted mortality between surgeons was due to a small but differing number of high risk patients.Conclusions It is possible to collect risk stratified data on all patients undergoing coronary bypass surgery. For most the predicted mortality is low. The small proportion of high risk patients is responsible for most of the differences in predicted mortality between surgeons. Crude comparisons of death rates can be misleading and may encourage surgeons to practise risk averse behaviour. We recommend a comparison of death rates that is stratified by risk and based on low risk cases as the national benchmark for assessing consultant specific performance.     

Homologous blood use and conservation techniques for cardiac surgery in the United Kingdom.

The transfusion laboratories of 32 cardiothoracic surgical centres for adults were surveyed to determine the donor blood requirement for open heart surgery in the United Kingdom. Details of the transfusion practice and the use of blood conservation techniques were sought from a representative senior cardiac anaesthetist at each centre. Suitable data were received from 24 transfusion laboratories (75%) and 29 anaesthetists (90%). The mean (SD) blood use was 5.07 (1.53) units per operation. Seven centres routinely transfused fresh frozen plasma to all patients postoperatively. Experience with autologous deposit (three centres), "cell separators" (four centres), and the reinfusion of shed mediastinal blood (four centres) was limited. Prebypass phlebotomy for postbypass reinfusion (14 centres) and the infusion of residual oxygenator blood (27 centres) were the conservation techniques most commonly applied. In only nine centres was a postoperative normovolaemic anaemia to a haemoglobin concentration of less than 100 g/l accepted. Applying blood conservation techniques more widely would help to maintain blood supplies and reduce morbidity and mortality related to transfusion.     

Necrotizing enterocolitis in neonates with congenital heart disease

Both necrotizing enterocolitis (NEC) and congenital heart disease (CHD) are causes of significant morbidity and mortality in the neonatal population. While two distinct disease processes, NEC and CHD are inter-related as the incidence of NEC is greater in neonates with CHD than the normal newborn population. It is likely that circulatory perturbations, especially those seen in infants with left ventricular outflow tract lesions and single ventricle physiology, the stress of cardiac surgery and cardiopulmonary bypass, and the underlying baseline elevation of circulating endotoxin and proinflammatory cytokines all play a role in the pathogenesis of NEC in this uniquely susceptible population. The neurodevelopmental impairment in infants requiring surgery for NEC and in infants with complex congenital heart disease is alarming and requires further investigation. As medical and surgical advances allow for the palliation and correction of complex lesions at an earlier gestational age and lower birth weight, the already high risk of NEC in this population is likely to increase. This will require more aggressive study of the etiology of NEC in patients with CHD and the development of preventative therapies in order to decrease the impressive morbidity and mortality associated with the combination of these disease processes. In this article, we review the pathogenesis of NEC and CHD including associated mortality and morbidities and discuss possible mechanisms linking these two disease states.     

How to evaluate and improve the quality and credibility of an outcomes database: validation and feedback study on the UK Cardiac Surgery Experience

ObjectivesTo assess the quality and completeness of a database of clinical outcomes after cardiac surgery and to determine whether a process of validation, monitoring, and feedback could improve the quality of the database.DesignStratified sampling of retrospective data followed by prospective re-sampling of database after intervention of monitoring, validation, and feedback.SettingTen tertiary care cardiac surgery centres in the United Kingdom.InterventionValidation of data derived from a stratified sample of case notes (recording of deaths cross checked with mortuary records), monitoring of completeness and accuracy of data entry, feedback to local data managers and lead surgeons.ResultsThe database was incomplete, with a mean (SE) of 24.96% (0.09%) of essential data elements missing, whereas only 1.18% (0.06%) were missing in the patient records (P<0.0001). Intervention was associated with (a) significantly less missing data (9.33% (0.08%) P<0.0001); (b) marginal improvement in reliability of data and mean (SE) overall centre reliability score (0.53 (0.15) v 0.44 (0.17)); and (c) improved accuracy of assigned Parsonnet risk scores (κ 0.84 v 0.70). Mortality scores (actual minus risk adjusted mortality) for all participating centres fell within two standard deviations of the mean score.ConclusionA short period of independent validation, monitoring, and feedback improved the quality of an outcomes database and improved the process of risk adjustment, but with substantial room for further improvement. Wider application of this approach should increase the credibility of similar databases before their public release.

What is already know in this topic

Release of healthcare outcomes into the public domain has altered referral patterns and has led to improvement in some centres and elimination of othersThe tacit assumption is that such outcomes data are accurate and can be relied on by the public and by healthcare providers to guide improvements

What this study adds

Sampling of a published national cardiac surgery database in England revealed it to be both incomplete and unreliable in its ability to yield accurate, risk adjusted outcomes dataAn independent short process of monitoring, validation, and feedback improved the quality of the databaseSuch databases probably require an ongoing process of monitoring in order to allow data of adequate quality to be generated for the purpose of improving healthcare outcomes     

The UK cardiac surgical register, 1977-82.

Data for 1977-82 obtained from the Cardiac Surgical Register, established by the Society of Thoracic and Cardiovascular Surgeons of Great Britain and Northern Ireland in 1977, were analysed for trends in incidence and mortality of cardiac surgery and regional workload in the United Kingdom. Operative mortality for most types of cardiac operation showed a general decline. The numbers of operations performed for valvular and congenital heart disease had remained unchanged, but a striking increase had occurred in the number of coronary bypass graft operations: 2297 in 1977 to 6008 in 1982. The figure for 1982, representing an annual rate of 107 operations per million population, was still well below that for other countries such as Australia (410 per million in 1982) and the United States (750 per million in 1981). A wide variation was seen in the regional provision of cardiac surgical services within the United Kingdom. This was particularly appreciable for coronary bypass graft surgery, in which there was a 10-fold difference in numbers of operations performed between the various regions. The UK Cardiac Surgical Register provides an important source of information on trends within the specialty that could well be followed by other surgical specialties.     

Associations between multimorbidity and adverse health outcomes in UK Biobank and the SAIL Databank: A comparison of longitudinal cohort studies

BackgroundCohorts such as UK Biobank are increasingly used to study multimorbidity; however, there are concerns that lack of representativeness may lead to biased results. This study aims to compare associations between multimorbidity and adverse health outcomes in UK Biobank and a nationally representative sample.Methods and findingsThese are observational analyses of cohorts identified from linked routine healthcare data from UK Biobank participants (n = 211,597 from England, Scotland, and Wales with linked primary care data, age 40 to 70, mean age 56.5 years, 54.6% women, baseline assessment 2006 to 2010) and from the Secure Anonymised Information Linkage (SAIL) databank (n = 852,055 from Wales, age 40 to 70, mean age 54.2, 50.0% women, baseline January 2011). Multimorbidity (n = 40 long-term conditions [LTCs]) was identified from primary care Read codes and quantified using a simple count and a weighted score. Individual LTCs and LTC combinations were also assessed. Associations with all-cause mortality, unscheduled hospitalisation, and major adverse cardiovascular events (MACEs) were assessed using Weibull or negative binomial models adjusted for age, sex, and socioeconomic status, over 7.5 years follow-up for both datasets.Multimorbidity was less common in UK Biobank than SAIL (26.9% and 33.0% with ≥2 LTCs in UK Biobank and SAIL, respectively). This difference was attenuated, but persisted, after standardising by age, sex, and socioeconomic status. The association between increasing multimorbidity count and mortality, hospitalisation, and MACE was similar between both datasets at LTC counts of ≤3; however, above this level, UK Biobank underestimated the risk associated with multimorbidity (e.g., mortality hazard ratio for 2 LTCs 1.62 (95% confidence interval 1.57 to 1.68) in SAIL and 1.51 (1.43 to 1.59) in UK Biobank, hazard ratio for 5 LTCs was 3.46 (3.31 to 3.61) in SAIL and 2.88 (2.63 to 3.15) in UK Biobank). Absolute risk of mortality, hospitalisation, and MACE, at all levels of multimorbidity, was lower in UK Biobank than SAIL (adjusting for age, sex, and socioeconomic status). Both cohorts produced similar hazard ratios for some LTCs (e.g., hypertension and coronary heart disease), but UK Biobank underestimated the risk for others (e.g., alcohol-related disorders or mental health conditions). Hazard ratios for some LTC combinations were similar between the cohorts (e.g., cardiovascular conditions); however, UK Biobank underestimated the risk for combinations including other conditions (e.g., mental health conditions). The main limitations are that SAIL databank represents only part of the UK (Wales only) and that in both cohorts we lacked data on severity of the LTCs included.ConclusionsIn this study, we observed that UK Biobank accurately estimates relative risk of mortality, unscheduled hospitalisation, and MACE associated with LTC counts ≤3. However, for counts ≥4, and for some LTC combinations, estimates of magnitude of association from UK Biobank are likely to be conservative. Researchers should be mindful of these limitations of UK Biobank when conducting and interpreting analyses of multimorbidity. Nonetheless, the richness of data available in UK Biobank does offers opportunities to better understand multimorbidity, particularly where complementary data sources less susceptible to selection bias can be used to inform and qualify analyses of UK Biobank.

Peter Hanlon and colleagues compare the associations between multimorbidity and adverse health outcomes in UK Biobank and the SAIL Databank.     

Modelling Survival and Mortality Risk to 15 Years of Age for a National Cohort of Children with Serious Congenital Heart Defects Diagnosed in Infancy

Background

Congenital heart defects (CHDs) are a significant cause of death in infancy. Although contemporary management ensures that 80% of affected children reach adulthood, post-infant mortality and factors associated with death during childhood are not well-characterised. Using data from a UK-wide multicentre birth cohort of children with serious CHDs, we observed survival and investigated independent predictors of mortality up to age 15 years.

Methods

Data were extracted retrospectively from hospital records and death certificates of 3,897 children (57% boys) in a prospectively identified cohort, born 1992–1995 with CHDs requiring intervention or resulting in death before age one year. A discrete-time survival model accounted for time-varying predictors; hazards ratios were estimated for mortality. Incomplete data were addressed through multilevel multiple imputation.

Findings

By age 15 years, 932 children had died; 144 died without any procedure. Survival to one year was 79.8% (95% confidence intervals [CI] 78.5, 81.1%) and to 15 years was 71.7% (63.9, 73.4%), with variation by cardiac diagnosis. Importantly, 20% of cohort deaths occurred after age one year. Models using imputed data (including all children from birth) demonstrated higher mortality risk as independently associated with cardiac diagnosis, female sex, preterm birth, having additional cardiac defects or non-cardiac malformations. In models excluding children who had no procedure, additional predictors of higher mortality were younger age at first procedure, lower weight or height, longer cardiopulmonary bypass or circulatory arrest duration, and peri-procedural complications; non-cardiac malformations were no longer significant.

Interpretation

We confirm the high mortality risk associated with CHDs in the first year of life and demonstrate an important persisting risk of death throughout childhood. Late mortality may be underestimated by procedure-based audit focusing on shorter-term surgical outcomes. National monitoring systems should emphasise the importance of routinely capturing longer-term survival and exploring the mechanisms of mortality risk in children with serious CHDs.     

Allogenic heart valve bank in the Department of Cardiovascular Surgery and Transplantology of Jagiellonian University in Cracow – 23 years experience in the treatment of aortic valve or aortic root diseases.

Allogenic aortic valves are widely used in case of native aortic valve or root disease as well as failed prosthetic valves with great success. At the Department of Cardiovascular Surgery and Transplantology of the Jagiellonian University in Cracow, aortic valve or aortic root replacement with allogenic aortic valve has been performed for 23 years. Allogenic heart valve bank was founded in 1980. In the bank we prepare both aortic allografts for adult cardiac surgical procedures and pulmonary allografts that are mostly used for repair of congenital heart disease.Allogenic aortic valves implantation was usually considered in our clinic for older patients, patients with infective endocarditis of the native or prosthetic valve, young women in reproductive age and patients with Marfan syndrome. Allografts exhibit excellent clinical performance and acceptable durability with no early failure if properly inserted. Between 1980 and 1992, allografts were obtained only from cadavers during routine autopsies. More than 10% of prepared allografts were exported to other cardiac surgery centres in Poland and foreign countries.Aortic valve replacement using allogenic aortic valves can be performed with acceptable mortality and good long-term results. The procedure although surgically more challenging has the advantage of not requiring anticoagulation therapy, hemodynamic performance of the allogenic valve is excellent, it demonstrates freedom from thromboembolism and infective endocarditis. We would like to emphasize the importance and advantages of the fact that allogenic heart valve bank is placed in the department of cardiovascular surgery and it is able to supply the department in heart valve allografts 24 h a day.     

Increased inpatient mortality in patients hospitalized for atrial fibrillation and atrial flutter with concomitant amyloidosis: Insight from National Inpatient Sample (NIS) 2016-2017

PurposeUsing National Inpatient Database (NIS), comparison of clinical outcomes for patients primarily admitted for atrial fibrillation/flutter with and without a secondary diagnosis of amyloidosis was done. Inpatient mortality was the primary outcome and hospital length of stay (LOS), mean total hospital charges, odds of undergoing cardiac ablation, pharmacologic cardioversion, having a secondary discharge diagnosis of heart block, cardiogenic shock and cardiac arrest were secondary outcomes.MethodsNIS database of 2016, 2017 was used for only adult hospitalizations with atrial fibrillation/flutter as principal diagnosis with and without amyloidosis as secondary diagnosis using ICD-10 codes. Multivariate logistic with linear regression analysis was used to adjust for confounders.Results932,054 hospitalizations were for adult patients with a principal discharge diagnosis of atrial fibrillation/flutter. 830 (0.09%) of these hospitalizations had amyloidosis.Atrial fibrillation/flutter hospitalizations with co-existing amyloidosis have higher inpatient mortality (4.22% vs 0.88%, AOR: 3.92, 95% CI 1.81–8.51, p = 0.001) and likelihood of having a secondary discharge diagnosis of cardiac arrest (2.40% vs 0.51%, AOR: 4.80, 95% CI 1.89–12.20, p = 0.001) compared to those without amyloidosis.ConclusionsHospitalizations of atrial fibrillation/flutter with co-existing amyloidosis have higher inpatient mortality and odds of having a secondary discharge diagnosis of cardiac arrest compared to those without amyloidosis. However, LOS, total hospital charges, likelihood of undergoing cardiac ablation, pharmacologic cardioversion, having a secondary discharge diagnosis of heart block and cardiogenic shock were similar between both groups.     

Congenital cardiology: recent advances emphasise the need for collaboration

The population of adult patients with congenital heart disease is steadily growing, due to the developments in cardiac surgery and thereby decreased mortality. However, morbidity in these patients is substantial. Patients with repaired lesions often need reoperations later in life. Most congenital heart defects, operated or not, have the potential to lead to clinical heart failure. Arrhythmias affect up to 50% of patients with congenital heart disease. The prevalence of pulmonary hypertension due to a left-to-right shunt among patients with a congenital heart defect is estimated at 4 to 10%. Advances in diagnostics, interventional and surgical therapy will result in new populations of adult survivors with even more complex disease. Collaboration of cardiologists with expertise in different areas of modern cardiology, such as electrophysiology, imaging and percutaneous interventions, is necessary for optimal care and management of these patients.     

Increased mortality of preterm infants transferred between tertiary perinatal centres.

Over 18 months almost one quarter of infants born before 30 weeks'' gestation in a tertiary perinatal centre who required intensive care had to be transferred to other tertiary centres because intensive care facilities were fully occupied. When infants with lethal congenital malformations were excluded half of the 34 infants who were transferred died; this was twice the mortality (24%) in the 111 infants remaining. The difference between the groups was significant (relative odds = 3.1) and remained so after adjustment for any discrepancies in gestational age (relative odds = 4.0). After adjustment for potential confounding variables by logistic function regression the risk of dying for those transferred remained significantly higher than that for infants who remained (relative odds = 4.6, 95% confidence interval 1.8 to 12.1). As the requirement for neonatal intensive care is episodic and unpredictable more flexibility has to be built into the perinatal health care system to enable preterm infants delivered in tertiary perinatal centres to be cared for where they are born.     

What tissue bankers should know about the use of allograft heart valves

Heart valve allografts (usually referred to as ‘homografts’) have been used in cardiac surgery for over 45 years when they were amongst the first valves ever used. Today they remain an important part of valve replacement and reconstructive surgery, particularly in the field of congenital heart disease. There are currently seven tissue banks on the UK and Eire that procure, prepare and store these homografts for surgical implantation, currently providing around 700 grafts per year. This article reviews the history and applications of homografts and compares their performance and outcomes with current prosthetic alternatives. It also describes the processes of valve procurement and storage and describes their clinical applications, hopefully providing tissue bankers with the surgeon’s insight into what is required. Homograft degeneration and the natural history of these tissues is discussed, together with future expectations and developments in homograft valve technology.     

"Inflammatory response to colloids compared to crystalloid priming in cardiac surgery patients with cardiopulmonary bypass"

"Cardiac surgery with cardiopulmonary bypass (CPB) induces a systemic inflammatory response syndrome that may contribute to postoperative morbidity and mortality. We investigated the in-flammatory responses to colloids compared to crystalloid priming in cardiac surgery patients with cardiopulmonary bypass. Thirty patients undergoing coronary artery bypass grafting (CABG) preparing for CPB were randomized into Ringer's solution (RS), 10% hydroxyethyl starch (HES) or 25% human albumin (HA) group. Serum concentrations of tumor necrosis factor-α (TNF-α), interleukin-1 β (IL-1β ), interleukin-6 (IL-6) and interleukin-10 (IL-10) were measured before CPB, at the end of CPB and 1, 6 and 12 h after CPB. Serum C-reactive protein (CRP) was determined pre-operatively and then daily for 2 days. Body-weight gain was significantly decreased on the day after surgery in the HES group than in the RS group. Volume priming in CPB for CABG patients using HA or HES preparation had less tendency for intense inflammatory response with lower levels of TNF-α, IL-1 β , IL-6 and higher levels of IL-10 compared to patients treated with RS. HES prime had lower levels of circulating CRP than in patients treated with HA or Ringer prime on the second post-operative day. Our data indicate that volume priming using colloid during CPB in CABG patients might exert beneficial effects on inflammatory responses."     

婴幼儿先天性心脏病术后床边胸片的临床应用评价

评价床边胸片在婴幼儿先天性心脏病术后的临床应用价值,总结其常见并发症以提高阅片正确率。方法:55例先天性心脏病术后婴幼儿患者,男34例,女24例,年龄1月至36月(平均12．8月),其术后均使用移动X线机摄取床边胸片,总结分析术后胸片新出现的异常表现。结果:55例先天性心脏病惠儿术后床边胸片中,发现31例新出现异常X线表现,发生率为56．4%。其中胸腔积液12例;气胸9例,其中4例合并皮下气肿;肺不张5例;左下肺炎3例;肺水肿1例;膈肌抬高1例。婴幼儿先天性心脏病术后最常见的并发症中前三位的依次为:胸腔积液、气胸和肺不张,分别占38．7%、29．0%、16．1%。结论:床边胸片是及时全面了解先天性心脏病患儿术后胸部出现新异常的重要和有效的检查手段,且简便易行,能将婴幼儿不能主诉的影响减至最低,从而为临床提供及时的诊疗依据。     

Phospholipid composition of myocardium in children with normoxemic and hypoxemic congenital heart diseases

Samples of myocardial tissue were obtained during cardiac surgery from children operated for different types of normoxemic and hypoxemic congenital heart diseases. The phospholipid composition was analyzed by thin layer chromatography. The concentration of total phospholipids (PL), phosphatidylcholine and phosphatidylethanolamine (PE) was found lower in atrial tissue of both normoxemic and hypoxemic groups in comparison with the ventricles. When comparing the difference between hypoxemic and normoxemic defects, hypoxemia was found to increase the concentration of total PL, PE and phosphatidylserine in ventricles and total PL and PE in the atria. The increased level of particular phospholipid species may represent adaptive mechanisms to hypoxemia in children with congenital heart diseases.     

Gender differences in adult congenital heart disease

Objective. To assess gender differences in morbidity, mortality and patient management among adults born with a heart defect. Methods and results. The database of the European Heart Survey on adult congenital heart disease was explored. This contains data on 4110 patients with one of eight congenital heart defects followed retrospectively for a median of 5.1 years. The existence of gender differences was assessed by considering mortality and a few ‘overall’ measures of morbidity. Adjusting for type of defect and age, it was found that cumulative mortality was greater in the male population (hazard ratio 1.63 (95% CI 1.12 to 2.38); p=0.011)). A significantly greater proportion of females had functional limitations (NYHA functional class >1; 37% vs. 29% of men; p=0.003). However, males were more likely to be on chronic medication during follow-up (59% vs. 55% of women; p=0.001), and males underwent diagnostic procedures more frequently (1.58/patient-year vs. 1.48/patient-year for women; p<0.02). There was no significant difference in the proportions of patients who underwent at least one intervention during follow-up, and rates of outpatient (re-)visits were not different between the sexes. Conclusion. This exploratory assessment of a large international database found evidence that gender differences exist in morbidity and mortality among adult patients with congenital heart disease, as well as in medical management. Future studies in adult congenital heart disease should always take into account the effects of gender. (Neth Heart J 2009; 17:414–7.)     

Using birth defects registry data to evaluate infant and childhood mortality associated with birth defects: an alternative to traditional mortality assessment using underlying cause of death statistics

BACKGROUND: Although birth defects are a leading cause of death in infancy and early childhood, the proportion of all deaths to children with clinically diagnosed birth defects is not well documented. The study is intended to measure the proportion of all deaths to infants and children under age 10 occurring to children with birth defects and how and why this proportion differs from the proportion of deaths due to an underlying cause of congenital anomalies using standard mortality statistics. METHODS: A linked file of Michigan livebirths and deaths was combined with data from a comprehensive multisource birth defects registry of Michigan livebirths born during the years 1992 through 2000. The data were analyzed to determine the mortality rate for infants and children with birth defects and for children with no reported birth defect. Mortality risk ratios were calculated. The underlying causes of death for children with birth defects were also categorized and compared to cause- specific mortality rates for the general population. RESULTS: Congenital anomalies were the underlying cause of death for 17.8% of all infant deaths while infants with birth defects were 33.7% of all infant deaths in the study. Almost half of all Michigan deaths to children aged 1 to 2 were within the birth defects registry, though only 15.0% had an underlying cause of death of a congenital anomaly based upon standard mortality statistics. The mortality experience among children with birth defects was significantly higher than other children throughout the first 9 years of life, ranging from 4.6 for 5 year olds to 12.8 for children 1 to 2. Mortality risk ratios examined by cause of death for infants with birth defects were highest for other endocrine (28.1), other CNS (28.1), and heart (21.9) conditions. For children 1 through 9, the highest differential risk was seen for other perinatal conditions (39.0), other endocrine (29.7), other CNS (24.5), and heart (21.4). CONCLUSIONS: Childhood mortality analyses that incorporate birth defects registry data provide a more comprehensive picture of the full burden of birth defects on mortality in infant and children and can provide an effective mechanism for monitoring the survival and mortality risks of children with selected birth defects on a population basis.     

Epidemiology of adult congenital heart disease: demographic variations worldwide

The population of adults with a congenital heart defect (CHD) is increasing, due to improved survival after cardiac surgery. To accommodate the specialised care for these patients, a profound interest in the epidemiology of CHD is required. The exact size of the current population of adults with CHD is unknown, but the best available evidence suggests that currently overall prevalence of CHD in the adult population is about 3000 per million. Regional differences in CHD prevalence have been described, due to both variations in incidence and in mortality. Knowledge of demographic variations of CHD may lead to new aetiological insights and may be useful for preventive therapies. Socioeconomic status, education, urbanisation, climatological factors, ethnicity and patient-related factors, such as comorbidity, lifestyle and healthcare-seeking behaviour, may play a role in CHD incidence and mortality. The higher risk of several major cardiac outcomes in males with CHD might well explain at least partly the increased mortality rate in men. Regional differences in quality of life among CHD patients have been reported and although methodological differences may play a role, sociocultural differences warrant further attention. Socioeconomic outcomes in CHD patients, such as lower education, more unemployment and less relationships, might have a different impact on quality of life in different cultures. To gain more insight into demographic differences around the world large international multicentre studies on the epidemiology of CHD are needed.