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Studies have suggested 5–20% of paediatric ICU patients may receive care felt to be futile. No data exists on the prevalence and impact of futile care in the Paediatric Cardiac ICU. The aim is to determine the prevalence and economic impact of futile care.
Materials and method:
Retrospective cohort of patients with congenital cardiac disease 0–21 years old, with length of stay >30 days and died (2015–2018). Documentation of futility by the medical team was retrospectively and independently reviewed.
Of the 127 deaths during the study period, 51 (40%) had hospitalisation >30 days, 13 (25%) had received futile care and 26 (51%) withdrew life-sustaining treatment. Futile care comprised 0.69% of total patient days with no difference in charges from patients not receiving futile care. There was no difference in insurance, single motherhood, education, income, poverty, or unemployment in families continuing futile care or electing withdrawal of life-sustaining treatment. Black families were less likely than White families to elect for withdrawal (p = 0.01), and Hispanic families were more likely to continue futile care than non-Hispanics (p = 0.044).
This is the first study to examine the impact of futile care and characteristics in the paediatric cardiac ICU. Black families were less likely to elect for withdrawal, while Hispanic families more likely to continue futile care. Futile care comprised 0.69% of bed days and little burden on resources. Cultural factors should be investigated to better support families through end-of-life decisions.
Objectives: As the number of adolescents and young adults (AYAs) surviving congenital heart disease (CHD) grows, studies of long-term outcomes are needed. CHD research documents poor executive function (EF) and cerebellum (CB) abnormalities in children. We examined whether AYAs with CHD exhibit reduced EF and CB volumes. We hypothesized a double dissociation such that the posterior CB is related to EF while the anterior CB is related to motor function. We also investigated whether the CB contributes to EF above and beyond processing speed. Methods: Twenty-two AYAs with CHD and 22 matched healthy controls underwent magnetic resonance imaging and assessment of EF, processing speed, and motor function. Volumetric data were calculated using a cerebellar atlas (SUIT) developed for SPM. Group differences were compared with t tests, relationships were tested with Pearson’s correlations and Fisher’s r to z transformation, and hierarchical regression was used to test the CB’s unique contributions to EF. Results: CHD patients had reduced CB total, lobular, and white matter volume (d=.52–.99) and poorer EF (d=.79–1.01) compared to controls. Significant correlations between the posterior CB and EF (r=.29–.48) were identified but there were no relationships between the anterior CB and motor function nor EF. The posterior CB predicted EF above and beyond processing speed (ps<.001). Conclusions: This study identified a relationship between the posterior CB and EF, which appears to be particularly important for inhibitory processes and abstract reasoning. The unique CB contribution to EF above and beyond processing speed alone warrants further study. (JINS, 2018, 24, 939–948)
Children with hypoplastic left heart syndrome are at a risk for neurodevelopmental delays. Current guidelines recommend systematic evaluation and management of neurodevelopmental outcomes with referral for early intervention services. The Single Ventricle Reconstruction Trial represents the largest cohort of children with hypoplastic left heart syndrome ever assembled. Data on life events and resource utilisation have been collected annually. We sought to determine the type and prevalence of early intervention services used from age 1 to 4 years and factors associated with utilisation of services.
Data from 14-month neurodevelopmental assessment and annual medical history forms were used. We assessed the impact of social risk and geographic differences. Fisher exact tests and logistic regression were used to evaluate associations.
Annual medical history forms were available for 302 of 314 children. Greater than half of the children (52–69%) were not receiving services at any age assessed, whereas 20–32% were receiving two or more therapies each year. Utilisation was significantly lower in year 4 (31%) compared with years 1–3 (with a range from 40 to 48%) (p<0.001). Social risk factors were not associated with the use of services at any age but there were significant geographic differences. Significant delay was reported by parents in 18–43% of children at ages 3 and 4.
Despite significant neurodevelopmental delays, early intervention service utilisation was low in this cohort. As survival has improved for children with hypoplastic left heart syndrome, attention must shift to strategies to optimise developmental outcomes, including enrolment in early intervention when merited.
Neonates undergoing heart surgery for CHD are at risk for postoperative gastrointestinal complications and aspiration events. There are limited data regarding the prevalence of aspiration after neonatal cardiothoracic surgery; thus, the effects of aspiration events on this patient population are not well understood. This retrospective chart review examined the prevalence and effects of aspiration among neonates who had undergone cardiac surgery at the time of their discharge.
This study examined the prevalence of aspiration among neonates who had undergone cardiac surgery. Demographic data regarding these patients were analysed in order to determine risk factors for postoperative aspiration. Post-discharge feeding routes and therapeutic interventions were extracted to examine the time spent using alternate feeding routes because of aspiration risk or poor caloric intake. Modified barium swallow study results were used to evaluate the effectiveness of the test as a diagnostic tool.
Materials and methods
A retrospective study was undertaken of neonates who had undergone heart surgery from July, 2013 to January, 2014. Data describing patient demographics, feeding methods, and follow-up visits were recorded and compared using a χ2 test for goodness of fit and a Kaplan–Meier graph.
The patient population included 62 infants – 36 of whom were male, and 10 who were born with single-ventricle circulation. The median age at surgery was 6 days (interquartile range=4 to 10 days). Modified barium swallow study results showed that 46% of patients (n=29) aspirated or were at risk for aspiration, as indicated by laryngeal penetration. In addition, 48% (n=10) of subjects with a negative barium swallow or no swallow study demonstrated clinical aspiration events. Tube feedings were required by 66% (n=41) of the participants. The median time spent on tube feeds, whether in combination with oral feeds or exclusive use of a nasogastric or gastric tube, was 54 days; 44% (n=27) of patients received tube feedings for more than 120 days. Premature infants were significantly more likely to have aspiration events than infants delivered at full gestational age (OR p=0.002). Infants with single-ventricle circulation spent a longer time on tube feeds (median=95 days) than infants with two-ventricle defects (median=44 days); the type of cardiac defect was independent of prevalence of an aspiration event.
Aspiration is common following neonatal cardiac surgery. The modified barium swallow study is often used to identify aspiration events and to determine an infant’s risk for aspirating. This leads to a high proportion of infants who require tube feedings following neonatal cardiac surgery.
White matter disruptions have been identified in individuals with congenital heart disease (CHD). However, no specific theory-driven relationships between microstructural white matter disruptions and cognition have been established in CHD. We conducted a two-part study. First, we identified significant differences in fractional anisotropy (FA) of emerging adults with CHD using Tract-Based Spatial Statistics (TBSS). TBSS analyses between 22 participants with CHD and 18 demographically similar controls identified five regions of normal appearing white matter with significantly lower FA in CHD, and two higher. Next, two regions of lower FA in CHD were selected to examine theory-driven differential relationships with cognition: voxels along the left uncinate fasciculus (UF; a tract theorized to contribute to verbal memory) and voxels along the right middle cerebellar peduncle (MCP; a tract previously linked to attention). In CHD, a significant positive correlation between UF FA and memory was found, r(20)=.42, p=.049 (uncorrected). There was no correlation between UF and auditory attention span. A positive correlation between MCP FA and auditory attention span was found, r(20)=.47, p=.027 (uncorrected). There was no correlation between MCP and memory. In controls, no significant relationships were identified. These results are consistent with previous literature demonstrating lower FA in younger CHD samples, and provide novel evidence for disrupted white matter integrity in emerging adults with CHD. Furthermore, a correlational double dissociation established distinct white matter circuitry (UF and MCP) and differential cognitive correlates (memory and attention span, respectively) in young adults with CHD. (JINS, 2015, 21, 22–33)
Diseases of the tricuspid and pulmonary valve are common in childhood. These include congenital anomalies, acquired lesions, and secondary valve compromise due to left heart disease. A comprehensive and methodical approach to the echocardiographic assessment of these diseases of the tricuspid and pulmonary valve is necessary for best care of children with these conditions.
The successful diagnosis, surgical planning, and long-term care of children with transposition of the great arteries require high-quality cardiac imaging with echocardiography. Echocardiography must identify the relevant anatomic variants of transposition of the great arteries, such as of ventricular septal defects and aortic arch anomalies. Methodical and detailed imaging of the coronary arteries is particularly important, as translocation of the coronary arteries is a critical component of the arterial switch procedure. Familiarity with the potential coronary artery variants and the ideal imaging planes is essential for an echocardiographer. Knowledge of both the early and late complications following the arterial switch procedure is essential to optimise post-operative echocardiography. These complications can include residual lesions leading to haemodynamic compromise or progressive late phenomena, such as aortic root dilatation and aortic insufficiency. Echocardiography will continue to be the cornerstone to the lifelong management of transposition of the great arteries, and improvements in technology and increased familiarity with modalities such as stress echocardiography will enhance the role of advanced imaging even further.
On recognising poor growth following neonatal palliation with a systemic-to-pulmonary shunt, we sought to determine how patient- and procedure-related factors impact growth, paying attention to the role of the primary cardiologist in this process.
In a retrospective review, neonates (133 patients) receiving modified systemic-to-pulmonary artery shunts from 2002 to 2009 were studied and outpatient visits were reviewed. Patients with single- and two-ventricle circulations after shunt takedown were compared using weight-for-age z-score.
Single-ventricle patients had a higher weight-for-age z-score at neonatal surgery than two-ventricle patients (−0.4 ± 1.0 compared with −1.2 ± 0.9, with p < 0.001), but they had a greater drop in the weight-for-age z-score to the first outpatient visit (−1.1 ± 0.7 compared with −0.8 ± 0.7, with p = 0.02). After the first outpatient visit, the weight-for-age z-score was not significantly different between single-ventricle and two-ventricle patients. From multivariate analysis, a lower number of nutritional interventions by cardiologists was significantly associated with poor growth (p = 0.03). Poor growth was not associated with race, use of feeding tube, exclusive formula use, or proximity to surgical centre.
The significant drop in the weight-for-age z-score from neonatal surgery to first outpatient visit suggests that these patients may receive inadequate nutrition. The poorest performers received the least number of outpatient changes to their diet. This finding underscores the critical role of the primary cardiologist in optimising weight gain through adjustments in nutrition.
Necrotising enterocolitis is a rare, though catastrophic complication that may occur in term newborns with congenital heart disease. There is considerable controversy regarding the factors that lead to necrotising enterocolitis in this population. We sought to determine the incidence of necrotising enterocolitis among term and near-term newborns with congenital heart disease, focusing on the relationship of enteral feeding to this complication.
In this retrospective study, we identified the incidence of necrotising enterocolitis among 1551 newborns admitted to our cardiac intensive care unit between July 1, 2002 and July 1, 2010. In order to understand the impact of enteral feeding upon the development of necrotising enterocolitis, we undertook a nested 2:1 matched case–control analysis to compare feeding patterns in an age- and lesion-matched control population.
Necrotising enterocolitis developed in 45 term or near-term infants (3%). The majority of these cases, 27 (60%), occurred in the post-operative period after the introduction of enteral feeds. This subgroup was used for matched analysis. There were no differences in enteral feeding patterns among the patients who developed necrotising enterocolitis and their matched controls. The overall mortality rate for patients who developed necrotising enterocolitis was 24.4% (11 out of 45).
Despite numerous advances in the care of infants with congenital heart disease, necrotising enterocolitis remains a significant source of morbidity and mortality. In these infants, there is no clear relationship between enteral feeding patterns and the development of necrotising enterocolitis in the post-operative period. The benefits of graduated feeding advancements to avoid the development of necrotising enterocolitis remain unproven.
The combination of both right and left heart obstruction has only rarely been described in the medical literature. We present three cases of coarctation of the aorta in patients with variants of tetralogy of Fallot and hypothesise that this condition may be more common than previously suspected and could represent a hidden cause of morbidity in patients with pulmonary atresia/ventricular septal defect.
There is a collection of rare congenital cardiac defects that can produce significant haemodynamic embarrassment. Owing to their rarity, these lesions may be overlooked or mistaken for other more common congenital cardiac defects. Using careful segmental echocardiographic techniques, such as multiple planes of imaging and inferential Doppler findings, it is possible to identify these lesions and thus plan surgical management. Some of the lesions of importance reviewed include the aortopulmonary window, the aorto-ventricular tunnels, the aorto-atrial tunnels, and aneurysm of the sinuses of Valsalva. Some conditions such as the aortopulmonary window and aneurysm of the sinuses of Valsalva often occur in the setting of other congenital cardiac lesions. At times, the presence of coexisting lesions may mask some of the characteristic features of these defects. With prompt identification, all of these rare conditions are amenable to complete surgical repair. Newer modalities such as three-dimensional echocardiography can further enhance surgical planning.
Administrative databases are often used for congenital cardiac disease research and evaluation, with little validation of the accuracy of the diagnostic codes.
Metropolitan Atlanta Congenital Defects Program surveillance records were reviewed and classified using a version of the International Pediatric and Congenital Cardiac Code. Using this clinical nomenclature as the referent, we report the sensitivity and false positive fraction (1 – positive predictive value) of the International Classification of Diseases, Ninth Revision, Clinical Modification diagnosis codes for tetralogy of Fallot, transposition of the great arteries, and hypoplastic left heart syndrome.
We identified 4918 infants and foetuses with congenital cardiac disease from the surveillance records. Using only the International Classification of Diseases diagnosis codes, there were 280 records with tetralogy, 317 records with transposition, and 192 records with hypoplastic left heart syndrome. Based on the International Pediatric and Congenital Cardiac Code, 330 records were classified as tetralogy, 163 records as transposition, and 179 records as hypoplastic left heart syndrome. The sensitivity of International Classification of Diseases diagnosis codes was 83% for tetralogy, 100% for transposition, and 95% for hypoplastic left heart syndrome. The false positive fraction was 2% for tetralogy, 49% for transposition, and 11% for hypoplastic left heart syndrome.
Analyses based on International Classification of Diseases diagnosis codes may have substantial misclassification of congenital heart disease. Isolating the major defect is difficult, and certain codes do not differentiate between variants that are clinically and developmentally different.
The most appropriate way of describing the congenital cardiac malformations unified because the atrial chambers are joined across the atrioventricular junctions to morphologically inappropriate ventricles has long been contentious. In the past, the lesions have been described in such arcane terms as mixed levocardia,1 while “ventricular inversion” still retains it currency in some circles. As we will show in this review, the abnormal arrangements at the atrioventricular junctions can be found with various patterns, but most frequently the patients also have the arterial trunks arising from morphologically inappropriate ventricles. This combination is best described as congenitally corrected transposition, and will form the focus of our review. It is salutary to note that, when von Rokitansky gave the first description of this combination,2 one of his illustrations was ideally suited to aid the understanding of modern-day echocardiographers (Fig. 1). We hope to emulate von Rokitansky in our own review.
It is now well recognized that patients fulfilling the diagnostic criterions for the group of hearts usually described as atrioventricular canal malformations, or atrioventricular septal defects, can present with shunting at atrial level, at both atrial and ventricular levels, and on occasion, with shunting only at ventricular level.1,2 It is also well recognized that, in most instances, the patients with shunting exclusively at atrial level have separate atrioventricular valvar orifices for the right and left ventricles, this arrangement often described as the “ostium primum” variant of atrial septal defect.3 Morphological and echocardiographic studies, however, have shown that, in this variant presumed to represent deficient atrial septation, it is the atrioventricular septal structures, rather than the atrial septum, which are deficient, the phenotypic feature being the presence of a common atrioventricular junction.4,5 In this review, we will show how, using modern day echocardiographic techniques, particularly the newly developed potential for three-dimensional display, it is an easy matter to identify the presence or absence of the common atrioventricular junction, and then to demonstrate the various relationships between the valvar leaflets, the septal structures, and the common junction itself which determine the options for clinical presentation within the group.
Background: Performance of the functionally single right ventricle may deteriorate over time. Quantitative assessment of this chamber, however, is complicated by its asymmetric geometry. Automatic detection of borders, and the Doppler-derived index of myocardial performance, are echocardiographic techniques that allow for quantitative assessment regardless of ventricular shape. We sought to evaluate the mechanics of contraction and relaxation in the functionally single right ventricle using these parameters. Methods: We evaluated systemic ventricular function in 35 asymptomatic patients with functionally single right ventricle, having a mean age of 7.8 ± 3.1 years, who had undergone the Fontan procedure. We compared them with 32 age-matched normal controls using both automatic detection of borders and Doppler indexes. Results: When compared with the controls, the group with a functionally single right ventricle demonstrated diminished systolic function as evidenced by a lower fractional change in area (42.7 ± 10.1% vs. 54.6 ± 10.5%, p = 0.001), and diminished diastolic function, as demonstrated by a greater reliance on atrial contraction to achieve ventricular filling (32.0 ± 4.4% vs. 22.2 ± 4.1%, p = 0.001). The mean index of myocardial performance in those with functionally single right ventricles was also greater than in controls (0.41 ± 0.12 vs. 0.30 ± 0.05, p = 0.001), and the indexed ejection time was shorter (0.35 ± 0.05 vs. 0.39 ± 0.05, p = 0.01), suggesting less efficient ventricular mechanics. Conclusions: These data demonstrate that the systolic and diastolic properties of the functionally single right ventricle differ significantly from those of the normal systemic left ventricle. Use of the echocardiographic techniques provide insight into ventricular mechanics in patients with functionally single ventricles, and may be valuable tools for serial quantitative follow-up.
It has long been known that the most complex combinations of cardiac malformations are those found in the setting of the so-called “splenic syndromes”.1 Many aspects of these syndromes have been controversial over recent years, not least the presence or absence of features of isomerism within the heart.2,3 Recent experience with genetic manipulation of mice, nonetheless, has now shown that it is possible to generate unequivocal evidence of cardiac isomerism, particularly in those animals which show features of right isomerism when the genes responsible for morphologically leftness are knocked out.4 Furthermore, when the crucial philosophical principle known as the “morphological method”5 is applied to the hearts of patients known to have visceral heterotaxy, it is equally clear that patients falling within these groups, when judged on the extent of the pectinate muscles relative to the atrioventricular junctions, exhibit isomerism of either the morphologically right or left atrial appendages.3
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