Differences in Quality of Life in Children Across the Spectrum of Congenital Heart Disease.

OBJECTIVE: To create complexity groups based upon a patient's cardiac medical history and to test for group differences in health-related quality of life (HRQOL). METHODS: Patients 8-18 years with congenital heart disease (CHD) and parent-proxies from the Pediatric Cardiac Quality of Life Inventory (PCQLI) Testing Study were included. Outcome variables included PCQLI Total, Disease Impact, and Psychosocial Impact scores. Using a patient's medical history (cardiac, neurologic, psychological, and cognitive diagnosis), latent class analysis (LCA) was used to create CHD complexity groups. Covariates included demographics and burden of illness (number of: school weeks missed, physician visits in the past year, and daily medications). Generalized estimation equations tested for differences in burden of illness and patient and parent-proxy PCQLI scores. RESULTS: Using 1482 CHD patients (60% male; 84% white; age 12.3 ± 3.0 years), latent class analysis (LCA) estimates showed 4 distinct CHD complexity groups (Mild, Moderate 1, Moderate 2, and Severe). Increasing CHD complexity was associated with increased risk of learning disorders, seizures, mental health problems, and history of stroke. Greater CHD complexity was associated with greater burden of illness (P < .01) and lower patient- and parent-reported PCQLI scores (P < .001). CONCLUSIONS: LCA identified 4 congenital heart disease (CHD) complexity groupings. Increasing CHD complexity was associated with higher burden of illness and worse patient- and parent-reported HRQOL.

Long-term survival and center volume for functionally single-ventricle congenital heart disease in England and Wales.

OBJECTIVES: Long-term survival is an important metric for health care evaluation, especially in functionally single-ventricle (f-SV) congenital heart disease (CHD). This study's aim was to evaluate the relationship between center volume and long-term survival in f-SV CHD within the centralized health care service of England and Wales. METHODS: This was a retrospective cohort study of children born with f-SV CHD between 2000 and 2018, using the national CHD procedure registry, with survival ascertained in 2020. RESULTS: Of 56,039 patients, 3293 (5.9%) had f-SV CHD. Median age at first intervention was 7 days (interquartile range [IQR], 4, 27), and median follow-up time was 7.6 years (IQR, 1.0, 13.3). The largest diagnostic subcategories were hypoplastic left heart syndrome, 1276 (38.8%); tricuspid atresia, 440 (13.4%); and double-inlet left ventricle, 322 (9.8%). The survival rate at 1 year and 5 years was 76.8% (95% confidence interval [CI], 75.3%-78.2%) and 72.1% (95% CI, 70.6%-73.7%), respectively. The unadjusted hazard ratio for each 5 additional patients with f-SV starting treatment per center per year was 1.04 (95% CI, 1.02-1.06), P < .001. However, after adjustment for significant risk factors (diagnostic subcategory; antenatal diagnosis; younger age, low weight, acquired comorbidity, increased severity of illness at first procedure), the hazard ratio for f-SV center volume was 1.01 (95% CI, 0.99-1.04) P = .28. There was strong evidence that patients with more complex f-SV (hypoplastic left heart syndrome, Norwood pathway) were treated at centers with greater f-SV case volume (P < .001). CONCLUSIONS: After adjustment for case mix, there was no evidence that f-SV center volume was linked to longer-term survival in the centralized health service provided by the 10 children's cardiac centers in England and Wales.

Transfer of congenital heart patients from paediatric to adult services in England.

OBJECTIVE: This study assessed the transfer of patients from paediatric cardiac to adult congenital heart disease (ACHD) services in England and the factors impacting on this process. METHODS: This retrospective cohort study used a population-based linked data set (LAUNCHES QI data set: 'Linking Audit and National datasets in Congenital Heart Services for Quality Improvement') including all patients born between 1987 and 2000, recorded as having a congenital heart disease (CHD) procedure in childhood. Hospital Episode Statistics data identified transfer from paediatric to ACHD services between the ages of 16 and 22 years. RESULTS: Overall, 63.8% of a cohort of 10 298 patients transferred by their 22nd birthday. The estimated probability of transfer by age 22 was 96.5% (95% CI 95.3 to 97.7), 86.7% (95% CI 85.6 to 87.9) and 41.0% (95% CI 39.4 to 42.6) for severe, moderate and mild CHD, respectively. 166 patients (1.6%) died between 16 and 22 years; 42 of these (0.4%) died after age 16 but prior to transfer. Multivariable ORs in the moderate and severe CHD groups up to age 20 showed significantly lower likelihood of transfer among female patients (0.87, 95% CI 0.78 to 0.97), those with missing ethnicity data (0.31, 95% CI 0.18 to 0.52), those from deprived areas (0.84, 95% CI 0.72 to 0.98) and those with moderate (compared with severe) CHD (0.30, 95% CI 0.26 to 0.35). The odds of transfer were lower for the horizontal compared with the vertical care model (0.44, 95% CI 0.27 to 0.72). Patients who did not transfer had a lower probability of a further National Congenital Heart Disease Audit procedure between ages 20 and 30 compared with those who did transfer: 12.3% (95% CI 5.1 to 19.6) vs 32.5% (95% CI 28.7 to 36.3). CONCLUSIONS: Majority of patients with moderate or severe CHD in England transfer to adult services. Patients who do not transfer undergo fewer elective CHD procedures over the following decade.

The brain in pediatric critical care: unique aspects of assessment, monitoring, investigations, and follow-up.

As survival after pediatric intensive care unit (PICU) admission has improved over recent years, a key focus now is the reduction of morbidities and optimization of quality of life for survivors. Neurologic disorders and direct brain injuries are the reason for 11-16% of admissions to PICU. In addition, many critically ill children are at heightened risk of brain injury and neurodevelopmental difficulties affecting later life, e.g., complex heart disease and premature birth. Hence, assessment, monitoring and protection of the brain, using fundamental principles of neurocritical care, are crucial to the practice of pediatric intensive care medicine. The assessment of brain function, necessary to direct appropriate care, is uniquely challenging amongst children admitted to the PICU. Challenges in assessment arise in children who are unstable, or pharmacologically sedated and muscle relaxed, or who have premorbid abnormality in development. Moreover, the heterogeneity of diseases and ages in PICU patients, means that high caliber evidence is harder to accrue than in adult practice, nonetheless, great progress has been made over recent years. In this 'state of the art' paper about critically ill children, we discuss (1) patient types at risk of brain injury, (2) new standardized clinical assessment tools for age-appropriate, clinical evaluation of brain function, (3) latest evidence related to cranial imaging, non-invasive and invasive monitoring of the brain, (4) the concept of childhood 'post intensive are syndrome' and approaches for neurodevelopmental follow-up. Better understanding of these concepts is vital for taking PICU survivorship to the next level.

Cohort study of intervened functionally univentricular heart in England and Wales (2000-2018).

OBJECTIVE: Given the paucity of long-term outcome data for complex congenital heart disease (CHD), we aimed to describe the treatment pathways and survival for patients who started interventions for functionally univentricular heart (FUH) conditions, excluding hypoplastic left heart syndrome. METHODS: We performed a retrospective cohort study using all procedure records from the National Congenital Heart Diseases Audit for children born in 2000-2018. The primary outcome was mortality, ascertained from the Office for National Statistics in 2020. RESULTS: Of 53 615 patients, 1557 had FUH: 55.9% were boys and 67.4% were of White ethnic groups. The largest diagnostic categories were tricuspid atresia (28.9%), double inlet left ventricle (21.0%) and unbalanced atrioventricular septal defect (AVSD) (15.2%). The ages at staged surgery were: initial palliation 11.5 (IQR 5.5-43.5) days, cavopulmonary shunt 9.2 (IQR 6.0-17.1) months and Fontan 56.2 (IQR 45.5-70.3) months. The median follow-up time was 10.8 (IQR 7.0-14.9) years and the 1, 5 and 10-year survival rates after initial palliation were 83.6% (95% CI 81.7% to 85.4%), 79.4% (95% CI 77.3% to 81.4%) and 77.2% (95% CI 75.0% to 79.2%), respectively. Higher hazards were present for unbalanced AVSD HR 2.75 (95% CI 1.82 to 4.17), atrial isomerism HR 1.75 (95% CI 1.14 to 2.70) and low weight HR 1.65 (95% CI 1.13 to 2.41), critical illness HR 2.30 (95% CI 1.67 to 3.18) or acquired comorbidities HR 2.71 (95% CI 1.82 to 4.04) at initial palliation. CONCLUSION: Although treatment pathways for FUH are complex and variable, nearly 8 out of 10 children survived to 10 years. Longer-term analyses of outcome based on diagnosis (rather than procedure) can inform parents, patients and clinicians, driving practice improvements for complex CHD.

Interventional treatments and risk factors in patients born with hypoplastic left heart syndrome in England and Wales from 2000 to 2015.

OBJECTIVE: To describe the long-term outcomes, treatment pathways and risk factors for patients diagnosed with hypoplastic left heart syndrome (HLHS) in England and Wales. METHODS: The UK's national audit database captures every procedure undertaken for congenital heart disease and updated life status for resident patients in England and Wales. Patients with HLHS born between 2000 and 2015 were identified using codes from the International Paediatric and Congenital Cardiac Code. RESULTS: There were 976 patients with HLHS. Of these, 9.6% had a prepathway intervention, 89.5% underwent a traditional pathway of staged palliation and 6.4% of infants underwent a hybrid pathway. Patients undergoing prepathway procedures or the hybrid pathway were more complex, exhibiting higher rates of prematurity and acquired comorbidity. Prepathway intervention was associated with the highest in-hospital mortality (34.0%).44.6% of patients had an off-pathway procedure after their primary procedure, most frequently stenting or dilation of residual or recoarctation and most commonly occurring between stage 1 and stage 2.The survival rate at 1 year and 5 years was 60.7% (95% CI 57.5 to 63.7) and 56.3% (95% CI 53.0 to 59.5), respectively. Patients with an antenatal diagnosis (multivariable HR (MHR) 1.63 (95% CI 1.12 to 2.38)), low weight (<2.5 kg) (MHR 1.49 (95% CI 1.05 to 2.11)) or the presence of an acquired comorbidity (MHR 2.04 (95% CI 1.30 to 3.19)) were less likely to survive. CONCLUSION: Treatment pathways among patients with HLHS are complex and variable. It is essential that the long-term outcomes of conditions like HLHS that require serial interventions are studied to provide a fuller picture and to inform quality assurance and improvement.

Impact of Surgical Complexity on Health-Related Quality of Life in Congenital Heart Disease Surgical Survivors.

BACKGROUND: Surgical complexity and related morbidities may affect long-term patient quality of life (QOL). Aristotle Basic Complexity (ABC) score and Risk Adjustment in Congenital Heart Surgery (RACHS-1) category stratify the complexity of pediatric cardiac operations. The purpose of this study was to examine the relationship between surgical complexity and QOL and to investigate other demographic and clinical variables that might explain variation in QOL in pediatric cardiac surgical survivors. METHODS AND RESULTS: Pediatric Cardiac Quality of Life (PCQLI) study participants who had undergone cardiac surgery were included. The PCQLI database provided sample characteristics and QOL scores. Surgical complexity was defined by the highest ABC raw score or RACHS-1 category. Relationships among surgical complexity and demographic, clinical, and QOL variables were assessed using ordinary least squares regression. A total of 1416 patient-parent pairs were included. Although higher ABC scores and RACHS-1 categories were associated with lower QOL scores (P<0.005), correlation with QOL scores was poor to fair (r=-0.10 to -0.29) for all groups. Ordinary least squares regression showed weak association with R(2)=0.06 to R(2)=0.28. After accounting for single-ventricle anatomy, number of doctor visits, and time since last hospitalization, surgical complexity scores added no additional explanation to the variance in QOL scores. CONCLUSIONS: ABC scores and RACHS-1 categories are useful tools for morbidity and mortality predictions prior to cardiac surgery and quality of care initiatives but are minimally helpful in predicting a child's or adolescent's long-term QOL scores. Further studies are warranted to determine other predictors of QOL variation.

Prediction by clinicians of quality of life for children and adolescents with cardiac disease.

OBJECTIVE: To determine whether clinicians could reliably predict health-related quality of life (HRQOL) for children with cardiac disease, the level of agreement in predicted HRQOL scores between clinician sub-types, and agreement between clinician-predicted HRQOL scores and patient and parent-proxy reported HRQOL scores. STUDY DESIGN: In this multicenter, cross-sectional study, a random sample of clinical summaries of children with cardiac disease and related patient and parent-proxy reported HRQOL scores were extracted from the Pediatric Cardiac Quality of Life Inventory data registry. We asked clinicians to review each clinical summary and predict HRQOL. RESULTS: Experienced pediatric cardiac clinicians (n = 140), including intensive care physicians, outpatient cardiologists, and intensive care, outpatient, and advanced practice nurses, each predicted HRQOL for the same 21 pediatric cardiac patients. Reliability within clinician subspecialty groups for predicting HRQOL was poor (intraclass correlation coefficients, 0.34-0.38). Agreement between clinician groups was low (Pearson correlation coefficients, 0.10-0.29). When comparing the average clinician predicted HRQOL scores to those reported by patients and parent-proxies by Bland Altman plots, little systematic bias was present, but substantial variability existed. Proportional bias was found, in that clinicians tended to overestimate HRQOL for those patients and parent-proxies who reported lower HRQOL, and underestimate HRQOL for those reporting higher HRQOL. CONCLUSIONS: Clinicians perform poorly when asked to predict HRQOL for children with cardiac disease. Clinicians should be cognizant of these data when providing counseling. Incorporating reported HRQOL into clinical assessment may help guide individualized treatment decision-making.

Quality of life in pediatric patients affected by electrophysiologic disease.

BACKGROUND: Treatment of electrophysiologic (EP) disease in pediatric patients has improved; however, the effects on quality of life (QOL) are unknown. OBJECTIVE: The purpose of this study was to compare QOL within EP disease groups and to other congenital heart diseases, to evaluate the effects of cardiac rhythm devices on QOL, and to identify drivers of QOL in EP disease. METHODS: Cross-sectional study of patient/parent proxy-reported Pediatric Cardiac Quality of Life Inventory scores (Total, Disease Impact, Psychosocial Impact) in subjects aged 8 to 18 years from 11 centers with congenital complete heart block (CCHB), ventricular tachycardia (VT), supraventricular tachycardia (SVT), and long QT syndrome (LQTS). QOL was compared between EP disease groups and congenital heart disease groups [bicuspid aortic valve (BAV), tetralogy of Fallot (TOF), and Fontan]. General linear modeling was used to perform group comparisons and to identify predictors of QOL variation. RESULTS: Among 288 patient-parent pairs, mean age was 12.8 ± 3.0 years. CCHB (µ = 83) showed higher patient Total QOL than other EP disease cohorts (P ≤ .02; LQTS µ = 73; SVT µ = 74). SVT (µ = 75) and LQTS (µ = 75) had lower patient Total scores than BAV (µ = 81; P ≤ .008). Patient/parent-proxy QOL scores for all EP disease groups were not different than TOF and higher than Fontan. The presence of a cardiac rhythm device was associated with lower QOL scores in LQTS (µ = 66 vs µ = 76; P < .01). Predictors of lower patient/parent-proxy QOL included EP disease type (P ≤ .03), increased medical care utilization (P ≤ .04), and no parental college degree (P ≤ .001). CONCLUSION: Given the significant variation in QOL in EP disease type, stratification by EP disease type and increased medical care utilization may allow for targeted interventions to improve QOL.

Neither age at repair nor previous palliation affects outcome in tetralogy of Fallot repair.

OBJECTIVES: The study aimed to evaluate the results following complete repair of tetralogy of Fallot (TOF) in relation to age at surgery and to assess the role of palliation in the current era. METHODS: A retrospective review of 251 consecutive patients with TOF repaired between 2003 and 2011 at the Great Ormond Street Hospital was performed. Children were divided into two groups: Group A, younger than 6 months (n = 78) and B, older than 6 months (n = 173). Early clinical outcomes and reoperation/reintervention rates were studied as well as indication for a palliation. RESULTS: There was 1 (0.4%) early and 1 (0.4%) late death after a median follow-up time of 4.5 years. Forty-three patients (17%) underwent repair after initial palliation with inter-stage mortality of 5%. Groups A and B were similar in terms of surgical approach, postoperative complications and length of stay. Significant differences were found in terms of more frequent use of a transannular patch (P = 0.05), longer surgeries (P = 0.02) and a greater proportion of palliated patients (P = 0.002) in older patients. There was no difference in rates of reoperation/reintervention between groups and following both primary and staged repair. Palliated patients were more symptomatic (duct-dependent pulmonary blood flow; P < 0.01, cyanotic spells; P < 0.01), had more extracardiac/genetic anomalies (P < 0.01), coronary anomalies (P = 0.015) and significantly smaller pulmonary annulus, right pulmonary artery (RPA) and left pulmonary artery (LPA) Z-scores (P < 0.01 for all). CONCLUSION: Age at complete repair was not linked to early clinical outcome or reoperation/reintervention rate. Palliative procedures postponed the timing of complete repair, but did not increase the reintervention rate.

Rapid 2-stage Norwood I for high-risk hypoplastic left heart syndrome and variants.

OBJECTIVES: Preoperative comorbidities (PCMs) are known risk factors for Norwood stage I (NW1). We tested the hypothesis that short-term bilateral pulmonary arterial banding (bPAB) before NW1 could improve the prognosis of these high-risk patients. METHODS: From January 2006 to October 2011, 17 high-risk patients with hypoplastic left heart syndrome (defined as having ≥4 of the following PCMs: prolonged mechanical ventilation; older age; sepsis; necrotizing enterocolitis; hepatic, renal, or heart failure; coagulopathy; pulmonary edema; high inotropic requirements; anasarca; weight <2.5 kg; and cardiac arrest) were identified. In addition to conventional treatment of PCMs, they underwent bPAB before NW1. bPAB was undertaken with Silastic slings and secured with ligaclips to a luminal diameter of approximately 3.5 to 4.0 mm. The patency of the ductus arteriosus was maintained with prostaglandin. NW1 was performed using a modified, right Blalock-Taussig shunt at a median interval of 8 days after bPAB. The data from these patients were retrospectively reviewed, and the 30-day mortality and 1-year survival were compared with the hypoplastic left heart syndrome population who underwent primary NW1 with <3 PCMs in the same period. RESULTS: Of the bPAB patients, 5 (29.4%) died before NW1. All had ≥5 PCMs. Twelve patients (70.6%) survived to undergo NW1. One early death occurred after NW1 (8.3%). The 1-year survival rate for high-risk patients who underwent NW1 was 66.7%. The early mortality and 1-year survival for the 130 patients with <3 PCMs was 10% and 80%, respectively. CONCLUSIONS: Optimizing the balance between the pulmonary and systemic blood flow with a short period of bPAB and ductal patency can improve the perioperative conditions of high-risk patients before NW1. Those who survived bPAB and underwent NW1 had early mortality and 1-year survival comparable to the standard risk category, despite the severity of their initial condition. A rapid 2-stage NW1 strategy with bPAB and prostaglandin to maintain ductal patency can avoid the risks of suboptimal palliation and vascular injuries associated with hybrid procedures.

Long-term survival outcomes and causes of late death in neonates, infants, and children treated with extracorporeal life support.

OBJECTIVES: Extracorporeal life support is a resource-intense treatment offered to the sickest patients. We aimed to investigate long-term survival rates and late deaths. DESIGN: Retrospective cohort study. SETTING: Tertiary referral center for extracorporeal life support. PATIENTS: All patients who required extracorporeal life support from 1992 to 2010 at our center. The U.K. National Health Service number was used to trace survival status of all patients who received extracorporeal life support at our center, grouped by diagnosis. Death more than 90 days after extracorporeal life support was defined as late, and these medical records were reviewed. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: A total of 741 children with 272 early deaths (36.7%) and 46 late deaths (6.2%) were included. Median follow-up time in survivors was 7.1 (interquartile range, 3.0-11.9) years. Five-year survival estimates were highest for meconium aspiration syndrome 88.0% (95% CI, 80.6-92.7%) and lowest for congenital heart disease 32.3% (95% CI, 25.1-39.8%). Five-year survival estimates conditional on being alive at 90 days were highest for meconium aspiration syndrome 97.9% (95% CI, 92.0-99.5%) and lowest for congenital diaphragmatic hernia 73.6% (52.3-86.5%). There was increased risk of late death in congenital diaphragmatic hernia, congenital heart disease, and acquired heart disease (p < 0.001, p < 0.01, p = 0.01) in comparison with the risk in meconium aspiration syndrome. For 46 late deaths, 17 had a cardiac cause, 16 had a respiratory cause, 10 had a comorbid cause, one died of sepsis, and in two, causation was unknown. CONCLUSIONS: Although the majority of deaths were early, late mortality was observed following extracorporeal life support. Late deaths were more prevalent in children with underlying complex long-term conditions, particularly heart disease and congenital diaphragmatic hernia. Evaluation of longer term survival is an important component of audit for extracorporeal life support outcomes.

Outcomes following extracorporeal membrane oxygenation in children with cardiac disease.

Extracorporeal membrane oxygenation is a commonly used form of mechanical circulatory support in children with congenital or acquired heart disease and cardiac failure refractory to conventional medical therapies. In children with heart disease who suffer cardiac arrest, extracorporeal membrane oxygenation has been successfully used to provide cardiopulmonary support when conventional resuscitation has failed to establish return of spontaneous circulation. Survival to hospital discharge for children with heart disease support is approximately 40% but varies widely based on age, indication for support, and underlying cardiac disease. Although extracorporeal membrane oxygenation is lifesaving in many instances, it is associated with many complications and is expensive. Thus, a clear understanding of survival to discharge and long-term functional and neurologic outcomes are essential to guide the use of extracorporeal membrane oxygenation now and in the future. This review, part of the Pediatric Cardiac Intensive Care Society/Extracorporeal Life Support Organization Joint Statement on Mechanical Circulatory Support, summarizes current knowledge on short- and long-term outcomes for extracorporeal membrane oxygenation used to support children with cardiac disease.

Development of a diagnosis- and procedure-based risk model for 30-day outcome after pediatric cardiac surgery.

OBJECTIVE: The study objective was to develop a risk model incorporating diagnostic information to adjust for case-mix severity during routine monitoring of outcomes for pediatric cardiac surgery. METHODS: Data from the Central Cardiac Audit Database for all pediatric cardiac surgery procedures performed in the United Kingdom between 2000 and 2010 were included: 70% for model development and 30% for validation. Units of analysis were 30-day episodes after the first surgical procedure. We used logistic regression for 30-day mortality. Risk factors considered included procedural information based on Central Cardiac Audit Database "specific procedures," diagnostic information defined by 24 "primary" cardiac diagnoses and "univentricular" status, and other patient characteristics. RESULTS: Of the 27,140 30-day episodes in the development set, 25,613 were survivals, 834 were deaths, and 693 were of unknown status (mortality, 3.2%). The risk model includes procedure, cardiac diagnosis, univentricular status, age band (neonate, infant, child), continuous age, continuous weight, presence of non-Down syndrome comorbidity, bypass, and year of operation 2007 or later (because of decreasing mortality). A risk score was calculated for 95% of cases in the validation set (weight missing in 5%). The model discriminated well; the C-index for validation set was 0.77 (0.81 for post-2007 data). Removal of all but procedural information gave a reduced C-index of 0.72. The model performed well across the spectrum of predicted risk, but there was evidence of underestimation of mortality risk in neonates undergoing operation from 2007. CONCLUSIONS: The risk model performs well. Diagnostic information added useful discriminatory power. A future application is risk adjustment during routine monitoring of outcomes in the United Kingdom to assist quality assurance.

Use of diagnostic information submitted to the United Kingdom Central Cardiac Audit Database: development of categorisation and allocation algorithms.

OBJECTIVE: To categorise records according to primary cardiac diagnosis in the United Kingdom Central Cardiac Audit Database in order to add this information to a risk adjustment model for paediatric cardiac surgery. DESIGN: Codes from the International Paediatric Congenital Cardiac Code were mapped to recognisable primary cardiac diagnosis groupings, allocated using a hierarchy and less refined diagnosis groups, based on the number of functional ventricles and presence of aortic obstruction. SETTING: A National Clinical Audit Database. Patients Children undergoing cardiac interventions: the proportions for each diagnosis scheme are presented for 13,551 first patient surgical episodes since 2004. RESULTS: In Scheme 1, the most prevalent diagnoses nationally were ventricular septal defect (13%), patent ductus arteriosus (10.4%), and tetralogy of Fallot (9.5%). In Scheme 2, the prevalence of a biventricular heart without aortic obstruction was 64.2% and with aortic obstruction was 14.1%; the prevalence of a functionally univentricular heart without aortic obstruction was 4.3% and with aortic obstruction was 4.7%; the prevalence of unknown (ambiguous) number of ventricles was 8.4%; and the prevalence of acquired heart disease only was 2.2%. Diagnostic groups added to procedural information: of the 17% of all operations classed as "not a specific procedure", 97.1% had a diagnosis identified in Scheme 1 and 97.2% in Scheme 2. CONCLUSIONS: Diagnostic information adds to surgical procedural data when the complexity of case mix is analysed in a national database. These diagnostic categorisation schemes may be used for future investigation of the frequency of conditions and evaluation of long-term outcome over a series of procedures.

Total donor ischemic time: relationship to early hemodynamics and intensive care morbidity in pediatric cardiac transplant recipients.

OBJECTIVE: Single-center studies have failed to link modest increases in total donor ischemic time to mortality after pediatric orthotopic heart transplant. We aimed to investigate whether prolonged total donor ischemic time is linked to pediatric intensive care morbidity after orthotopic heart transplant. DESIGN: Retrospective cohort review. SETTING: Tertiary pediatric transplant center in the United Kingdom. PATIENTS: Ninety-three pediatric orthotopic heart transplants between 2002 and 2006. METHODS: Total donor ischemic time was investigated for association with early post-orthotopic heart transplant hemodynamics and intensive care unit morbidities. RESULTS: Of 43 males and 50 females with median age 7.2 (interquartile range 2.2, 13.0) yrs, 62 (68%) had dilated cardiomyopathy, 20 (22%) had congenital heart disease, and nine (10%) had restrictive cardiomyopathy. The mean total donor ischemic time was 225.9 (sd 65.6) mins. In the first 24 hrs after orthotopic heart transplant, age-adjusted mean arterial blood pressure increased (p < .001), mean pulmonary arterial pressure fell (p = .012), but central venous pressure (p = .58) and left atrial pressure (p = .20) were unchanged. After adjustment for age, primary diagnosis, pre-orthotopic heart transplant mechanical support, and marginal donor factors, longer total donor ischemic time was significantly associated with lower mean arterial blood pressure (p < .001) in the first 24 hrs after orthotopic heart transplant, longer post-orthotopic heart transplant mechanical ventilation (p = .03), longer post-orthotopic heart transplant stay in the intensive care unit (p = .004), and longer post-orthotopic heart transplant stay in hospital (p = .02). Total donor ischemic time was not related to levels of mean pulmonary arterial pressure (p = .62), left atrial pressure (p = .38), or central venous pressure (p = .76) early after orthotopic heart transplant. CONCLUSIONS: Prolonged total donor ischemic time has an adverse effect on the donor organ, contributing to lower mean arterial blood pressure, as well as more prolonged ventilation and intensive care unit and hospital stays post-orthotopic heart transplant, reflecting increased morbidity.