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

Arterial Switch for Transposition of the Great Arteries

Background Reports of long-term mortality and reintervention after transposition of the great arteries with intact ventricular septum treatment, although favorable, are mostly limited to single-center studies. Even less is known about hospital resource utilization (days at hospital) and the impact of treatment choices and timing on outcomes. Objectives The purpose of this study was to describe survival, reintervention and hospital resource utilization after arterial switch operation (ASO) in a national dataset. Methods Follow-up and life status data for all patients undergoing ASO between 2000 and 2017 in England and Wales were collected and explored using multivariable regressions and matching. Results A total of 1,772 patients were identified, with median ASO age of 9.5 days (IQR: 6.5-14.5 days). Mortality and cardiac reintervention at 10 years after ASO were 3.2% (95% CI: 2.5%-4.2%) and 10.7% (95% CI: 9.1%-12.2%), respectively. The median time spent in hospital during the ASO spell was 19 days (IQR: 14, 24). Over the first year after the ASO patients spent 7 days (IQR: 4-10 days) in hospital in total, decreasing to 1 outpatient day/year beyond the fifth year. In a subgroup with complete risk factor data (n = 652), ASO age, and balloon atrial septostomy (BAS) use were not associated with late mortality and reintervention, but cardiac or congenital comorbidities, low weight, and circulatory/renal support at ASO were. After matching for patient characteristics, BAS followed by ASO and ASO as first procedure, performed within the first 3 weeks of life, had comparable early and late outcomes, including hospital resource utilization. Conclusions Mortality and hospital resource utilization are low, while reintervention remains relatively frequent. Early ASO and individualized use of BAS allows for flexibility in treatment choices and a focus on at-risk patients

Hospital resource utilization in a national cohort of functionally single ventricle patients undergoing surgical treatment

Objective: To provide a detailed overview of health resource utilization from birth to 18 years old for functionally single ventricle (f-SV) patients and identify associated risk factors./ Methods: All f-SV patients treated between 2000-2017 in England and Wales were linked to hospital and outpatient records using data from the Linking AUdit and National datasets in Congenital HEart Services (LAUNCHES) project. Hospital stay was described in yearly age intervals and associated risk factors were explored using quantile regression./ Results: A total of 3,037 f-SV patients were included, 1409 (46.3%) undergoing a Fontan procedure. During the first year of life the median days spent in-hospital was 60 (IQR 37-102), mostly inpatient days, mirroring a mortality of 22.8%. This decreases to between 2-9 in-hospital days/year afterwards. Between 2-18 years most hospital days were outpatient, with a median of 1-5 days/year./ Lower age at the first procedure, hypoplastic left heart syndrome/mitral atresia, unbalanced atrioventricular septal defect, preterm birth, congenital/acquired comorbidities, additional cardiac risk factors and severity of illness markers were associated with fewer days-at-home and more ICU days in the first year of life. Only markers of early severe illness were associated with fewer days-at-home in the first 6 months post Fontan procedure./ Conclusions: Hospital resource utilization in f-SV is not uniform, decreasing tenfold during adolescence compared to the first year of life. There are subsets of patients with worse outcomes during their first year of life, or with persistently high hospital usage throughout their childhood, which could be the target of future research

Report of the Coding Committee of the Association for European Paediatric Cardiology

IN 1999 AND EARLY 2000, THE ASSOCIATION FOR European Paediatric Cardiology published the European Paediatric Cardiac Code as independent but linked Short and Long Lists, containing 650 and 3876 primary terms respectively. The historical background and rationale for development of this coding system has been previously detailed, but essentially it followed a series of meeting of the coding committee of the Association between 1997-1999, during which a pre-existing Long List was adopted and then used to create the condensed Short List. The system was published as the recommended standard coding system for use across Europe, covering the diagnosis and therapy of children with congenital and acquired cardiac disease. The scope of the lists was to encompass the needs of all those involved with such patients, from the fetal cardiologist through to the specialist in adult congenital heart disease; and from the general paediatric cardiologist and cardiac surgeon, to those specialising in transcatheter interventions, paediatric electrophysiology, and paediatric echocardiographers. In addition, the code was crossmapped to the 9th and 10th revisions of the International Classification of Diseases ("ICD-9” and "ICD-10”) provided by the World Health Organisation in order to facilitate returns to central government, a requirement in most countries. In so doing, it was hoped to address the concerns of many centres that such information submitted by professional coding staff was often inaccurate due to the complex nature of congenital cardiac disease, together with the limited scope and vague terminology of the International listing

Ethnic and socioeconomic variation in incidence of congenital heart defects

Introduction: Ethnic differences in the birth prevalence of congenital heart defects (CHDs) have been reported; however, studies of the contemporary UK population are lacking. We investigated ethnic variations in incidence of serious CHDs requiring cardiac intervention before 1 year of age. Methods: All infants who had a cardiac intervention in England and Wales between 1 January 2005 and 31 December 2010 were identified in the national congenital heart disease surgical audit and matched with paediatric intensive care admission records to create linked individual child records. Agreement in reporting of ethnic group by each audit was evaluated. For infants born 1 January 2006 to 31 December 2009, we calculated incidence rate ratios (IRRs) for CHDs by ethnicity and investigated age at intervention, antenatal diagnosis and area deprivation. Results: We identified 5350 infants (2940 (55.0%) boys). Overall CHD incidence was significantly higher in Asian and Black ethnic groups compared with the White reference population (incidence rate ratios (IRR) (95% CIs): Asian 1.5 (1.4 to 1.7); Black 1.4 (1.3 to 1.6)); incidence of specific CHDs varied by ethnicity. No significant differences in age at intervention or antenatal diagnosis rates were identified but affected children from non-White ethnic groups were more likely to be living in deprived areas than White children. Conclusions: Significant ethnic variations exist in the incidence of CHDs, including for specific defects with high infant mortality. It is essential that healthcare provision mitigates ethnic disparity, including through timely identification of CHDs at screening, supporting parental choice and effective interventions. Future research should explore the factors underlying ethnic variation and impact on longer-term outcomes

A Genome-Wide Association Study Identifies Variants Underlying the Arabidopsis thaliana Shade Avoidance Response

Shade avoidance is an ecologically and molecularly well-understood set of plant developmental responses that occur when the ratio of red to far-red light (R∶FR) is reduced as a result of foliar shade. Here, a genome-wide association study (GWAS) in Arabidopsis thaliana was used to identify variants underlying one of these responses: increased hypocotyl elongation. Four hypocotyl phenotypes were included in the study, including height in high R∶FR conditions (simulated sun), height in low R∶FR conditions (simulated shade), and two different indices of the response of height to low R∶FR. GWAS results showed that variation in these traits is controlled by many loci of small to moderate effect. A known PHYC variant contributing to hypocotyl height variation was identified and lists of significantly associated genes were enriched in a priori candidates, suggesting that this GWAS was capable of generating meaningful results. Using metadata such as expression data, GO terms, and other annotation, we were also able to identify variants in candidate de novo genes. Patterns of significance among our four phenotypes allowed us to categorize associations into three groups: those that affected hypocotyl height without influencing shade avoidance, those that affected shade avoidance in a height-dependent fashion, and those that exerted specific control over shade avoidance. This grouping allowed for the development of explicit hypotheses about the genetics underlying shade avoidance variation. Additionally, the response to shade did not exhibit any marked geographic distribution, suggesting that variation in low R∶FR–induced hypocotyl elongation may represent a response to local conditions