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To define the incidence of definitive necrotising enterocolitis in term infants with CHD and identify risk factors for morbidity/mortality.
We performed a 20-year (2000–2020) single-institution retrospective cohort study of term infants with CHD admitted to the Boston Children’s Hospital cardiac ICU with necrotising enterocolitis (Bell’s stage ≥ II). The primary outcome was a composite of in-hospital mortality and post-necrotising enterocolitis morbidity (need for extracorporeal membrane oxygenation, multisystem organ failure based on the paediatric sequential organ failure assessment score, and/or need for acute gastrointestinal intervention). Predictors included patient characteristics, cardiac diagnosis/interventions, feeding regimen, and severity measures.
Of 3933 term infants with CHD, 2.1% (n = 82) developed necrotising enterocolitis, with 67% diagnosed post-cardiac intervention. Thirty (37%) met criteria for the primary outcome. In-hospital mortality occurred in 14 infants (17%), of which nine (11%) deaths were attributable to necrotising enterocolitis. Independent predictors of the primary outcome included moderate to severe systolic ventricular dysfunction (odds ratio 13.4,confidence intervals 1.13–159) and central line infections pre-necrotising enterocolitis diagnosis (odds ratio 17.7, confidence intervals 3.21–97.0) and mechanical ventilation post-necrotising enterocolitis diagnosis (odds ratio 13.5, confidence intervals 3.34–54.4). Single ventricle, ductal dependency, and feeding related factors were not independently associated with the primary outcome.
The incidence of necrotising enterocolitis was 2.1% in term infants with CHD. Adverse outcomes occurred in greater than 30% of patients. Presence of systolic dysfunction and central line infections prior to diagnosis and need for mechanical ventilation after diagnosis of necrotising enterocolitis can inform risk triage and prognostic counseling for families.
In single-ventricle patients undergoing staged-bidirectional Glenn, 36–59% have aorto-pulmonary collateral flow, but risk factors and clinical outcomes are unknown. We hypothesise that shunt type and catheter haemodynamics may predict pre-bidirectional Glenn aorto-pulmonary collateral burden, which may predict death/transplantation, pulmonary artery or aorto-pulmonary collateral intervention.
Retrospective cohort study of patients undergoing a Norwood procedure for single-ventricle anatomy. Covariates included clinical and haemodynamic characteristics up to/including pre-bidirectional Glenn catheterisation and aorto-pulmonary collateral burden at pre-bidirectional Glenn catheterisation. Multivariable models used to evaluate relationships between risk factors and outcomes.
From January 2011 to March 2016, 104 patients underwent Norwood intervention. Male sex (odds ratio 3.36, 95% confidence interval 1.17–11.4), age at pre-bidirectional Glenn assessment (2.12, 1.33–3.39 per month), and pulmonary to systemic flow ratio (1.23, 1.08–1.41 per 0.1 unit) were associated with aorto-pulmonary collateral burden. Aorto-pulmonary collateral burden was not associated with death/transplantation (hazard ratio 1.19, 95% confidence interval 0.37–3.85), pulmonary artery (sub-hazard ratio 1.38, 0.32–2.61), or aorto-pulmonary collateral interventions (sub-hazard ratio 1.11, 0.21–5.76). Longer post-Norwood length of stay was associated with greater risk of death/transplantation (hazard ratio 1.22 per week, 95% confidence interval 1.08–1.38), but lower risk of aorto-pulmonary collateral intervention (sub-hazard ratio 0.86 per week, 95% confidence interval 0.75–0.98). Time to pre-bidirectional Glenn catheterisation was associated with lower risk of pulmonary artery (sub-hazard ratio 0.80 per month, 95% confidence interval 0.65–0.98) and aorto-pulmonary collateral intervention (sub-hazard ratio 0.79, 0.63–0.99). Probability of moderate/severe aorto-pulmonary collateral burden increased with left-to-right shunt (22.5% at <1.0, 57.6% at >1.4) and the age at pre-bidirectional Glenn catheterisation (10.6% at <2 months, 56.9% at >5 months).
Aorto-pulmonary collateral burden is common after Norwood procedure and increases as age at bidirectional Glenn increases. As expected, higher pulmonary to systemic flow ratio is a marker for greater aorto-pulmonary collateral burden pre-bi-directional Glenn; aorto-pulmonary collateral burden does not confer risk of death/transplantation or pulmonary artery intervention.
Surgical care for CHD is increasingly available in low- and middle-income countries, and efforts to optimise outcomes are growing. This study characterises cardiac imaging and prenatal diagnosis infrastructure in this setting.
An infrastructure survey was administered to sites participating in the International Quality Improvement Collaborative for CHD. Questions regarding transthoracic, transesophageal and epicardial echocardiography, cardiac CT, cardiac magnetic resonance, prenatal screening and fetal echocardiography were included. Associations with in-hospital and 30-day mortality were assessed.
Thirty-seven sites in 17 countries responded. Programme size and geography varied considerably: < 250 cases (n = 13), 250–500 cases (n = 9), > 500 cases (n = 15); Americas (n = 13), Asia (n = 18), and Eastern Europe (n = 6). All had access to transthoracic echo. Most reported transesophageal and epicardial echocardiography availability (86 and 89%, respectively). Most (81%) had cardiac CT, but only 54% had cardiac magnetic resonance. A third reported impediments to imaging, including lack of portable machines, age/size-appropriate equipment and advanced cardiac imaging access and training. Only 19% of centres reported universal prenatal CHD screening in their catchment area, and only 46% always performed fetal echocardiography if screening raised concern for CHD. No statistically significant associations were identified between imaging modality availability and surgical outcomes.
Although access to echocardiography is available in most middle-income countries; advanced imaging modalities (cardiac CT and magnetic resonance) are not always accessible. Prenatal screening for CHD is low, and availability of fetal echocardiography is limited. Imaging infrastructure in low- and middle-income countries and associations with outcomes merits additional study.
Exercise capacity is a modifiable factor in patients with CHD that has been related to surgical outcomes in adults. We hypothesised that this was true for children undergoing surgical pulmonary valve replacement; therefore, the relationship of preoperative percent predicted peak oxygen consumption to surgical outcomes as measured by total hospital length of stay was explored.
Single centre retrospective cohort study of patients aged 8–18 years who underwent surgical pulmonary valve replacement. The primary predictor was preoperative percent predicted peak oxygen consumption, and primary outcome was total hospital length of stay. Clinical, imaging, and cardiopulmonary exercise test data were reviewed and compared to total hospital length of stay. Cox proportional hazards regression was used to examine the association between total hospital length of stay and percent predicted peak oxygen consumption.
Three-hundred and seventy patients undergoing pulmonary valve replacement/conduit change between 2003 and 2017 at Boston Children’s Hospital were identified. Ninety had preoperative cardiopulmonary exercise tests within 6 months of surgery. Exclusion for inadequate exercise data (n = 3) and imaging data (n = 1) left 86 patients for review. Patients with percent predicted peak oxygen consumption ≥ 70% (n = 46, 53%) had shorter total hospital length of stay (4.4 days) than the 40 with percent predicted peak oxygen consumption <70% (5.4 days, p = 0.007). Median percent predicted peak oxygen consumption increased over sequential surgical eras (p < 0.001), but total hospital length of stay did not correlate with surgical era, preoperative left ventricular function, or preoperative right ventricular dilation.
Children undergoing surgical pulmonary valve replacement with better preoperative exercise capacity had shorter total hospital length of stay. Exercise capacity is a potentially modifiable factor prior to and after pulmonary valve replacement. Until more patients systematically undergo cardiopulmonary exercise tests, the full impact of optimisation of exercise capacity will not be known.
Growth failure is prevalent among infants with CHD. A Standardized Clinical Assessment and Management Plan was introduced at Boston Children’s Hospital’s cardiac medical ward to identify patients with growth failure, evaluate relevant contributing conditions, and recommend a management plan including collaboration with nutrition physicians.
The objective of this study was to determine whether enrolled patients had improved growth compared with historical controls.
A total of 29 patients were enrolled in the period July, 2013–June, 2014. In all, 42 historical controls who met eligibility criteria for enrolment were selected for comparison from patients admitted to the same ward in the period June, 2010–June, 2011. Patients with CHD aged <1 year , with growth failure defined as weight-for-age z-score <−2, or failure to sustain adequate weight gain were eligible for participation. Primary outcome was change in weight-for-age z-score from enrolment to most recent weight measurement among patients with at least 6 months of follow-up.
Control patients were older at baseline admission weight (118 versus 95 days, p=0.33), and had a higher weight-for-age z-score, −2.9 (−3.1, −2.6) versus −3.7 (−4.3, −3.0) (p=0.02), compared with enrolled patients. Enrolled patients had greater gain in weight-for-age z-score, 2.7 (2.0, 3.4) versus 1.8 (1.5, 2.2) (p=0.03), from baseline to most recent follow-up.
Patients enrolled in a nutrition-focused protocol had greater weight improvement than historical controls. Identification of growth failure and collaboration with a nutrition support team was associated with improved weight gain among CHD patients experiencing growth failure. CHD programmes should consider a structural approach, including nutrition expertise to address growth failure.
Following stage 1 palliation, delayed sternal closure may be used as a technique to enhance thoracic compliance but may also prolong the length of stay and increase the risk of infection.
We reviewed all neonates undergoing stage 1 palliation at our institution between 2010 and 2017 to describe the effects of delayed sternal closure.
During the study period, 193 patients underwent stage 1 palliation, of whom 12 died before an attempt at sternal closure. Among the 25 patients who underwent primary sternal closure, 4 (16%) had sternal reopening within 24 hours. Among the 156 infants who underwent delayed sternal closure at 4 [3,6] days post-operatively, 11 (7.1%) had one or more failed attempts at sternal closure. Patients undergoing primary sternal closure had a shorter duration of mechanical ventilation and intensive care unit length of stay. Patients who failed delayed sternal closure had a longer aortic cross-clamp time (123±42 versus 99±35 minutes, p=0.029) and circulatory arrest time (39±28 versus 19±17 minutes, p=0.0009) than those who did not fail. Failure of delayed sternal closure was also closely associated with Technical Performance Score: 1.3% of patients with a score of 1 failed sternal closure compared with 18.9% of patients with a score of 3 (p=0.0028). Among the haemodynamic and ventilatory parameters studied, only superior caval vein saturation following sternal closure was different between patients who did and did not fail sternal closure (30±7 versus 42±10%, p=0.002). All patients who failed sternal closure did so within 24 hours owing to hypoxaemia, hypercarbia, or haemodynamic impairment.
When performed according to our current clinical practice, sternal closure causes transient and mild changes in haemodynamic and ventilatory parameters. Monitoring of SvO2 following sternal closure may permit early identification of patients at risk for failure.
It is universally agreed that efforts to improve quality benefit from the analysis of outcomes. Yet, it is challenging to compare results across institutions because factors other than performance also impact outcomes. Two factors that complicate the analysis of outcomes after congenital cardiac surgery are case-mix and random statistical variation. Case-mix refers to differences in the mix of patients and their risk-factors at different institutions that may cause some centres to have more frequent complications and lower survival regardless of their true performance. Random statistical variation refers to fluctuations in outcomes that occur at random and follow the laws of probability. A variety of statistical methods exist to address these issues and make provider comparisons more fair. We explain a few common approaches including stratification, regression analysis, and confidence intervals. Concepts are illustrated using artificial data from two hypothetical hospitals, as well as real data from a multi-institution registry.
Meaningful evaluation of quality of care must account for variations in the population of patients receiving treatment, or “case-mix”. In adult cardiac surgery, empirical clinical data, initially from tens of thousands, and more recently hundreds of thousands of operations, have been used to develop risk-models, to increase the accuracy with which the outcome of a given procedure on a given patient can be predicted, and to compare outcomes on non-identical patient groups between centres, surgeons and eras.
In the adult cardiac database of The Society of Thoracic Surgeons, algorithms for risk-adjustment are based on over 1.5 million patients undergoing isolated coronary artery bypass grafting and over 100,000 patients undergoing isolated replacement of the aortic valve or mitral valve. In the pediatric and congenital cardiac database of The Society of Thoracic Surgeons, 61,014 operations are spread out over greater than 100 types of primary procedures. The problem of evaluating quality of care in the management of pediatric patients with cardiac diseases is very different, and in some ways a great deal more challenging, because of the smaller number of patients and the higher number of types of operations.
In the field of pediatric cardiac surgery, the importance of the quantitation of the complexity of operations centers on the fact that outcomes analysis using raw measurements of mortality, without adjustment for complexity, is inadequate. Case-mix can vary greatly from program to program. Without stratification of complexity, the analysis of outcomes for congenital cardiac surgery will be flawed. Two major multi-institutional efforts have attempted to measure the complexity of pediatric cardiac operations: the Risk Adjustment in Congenital Heart Surgery-1 method and the Aristotle Complexity Score. Both systems were derived in large part from subjective probability, or expert opinion. Both systems are currently in wide use throughout the world and have been shown to correlate reasonably well with outcome.
Efforts are underway to develop the next generation of these systems. The next generation will be based more on objective data, but will continue to utilize subjective probability where objective data is lacking. A goal, going forward, is to re-evaluate and further refine these tools so that, they can be, to a greater extent, derived from empirical data. During this process, ideally, the mortality elements of both the Aristotle Complexity Score and the Risk Adjustment in Congenital Heart Surgery-1 methodology will eventually unify and become one and the same. This review article examines these two systems of stratification of complexity and reviews the rationale for the development of each system, the current use of each system, the plans for future enhancement of each system, and the potential for unification of these two tools.
Introduction: We sought to establish a modern understanding of the safety and efficacy of trans-catheter pulmonary arterial angioplasty. Methods: A review of records in a sample of 104 dilations, out of a total of 711 procedures undertaken between January, 1996 and December, 2000, provided descriptive information regarding technique, adverse events, and changes achieved in luminal diameter. Because evidence during the review of angiograms suggested substantial differences according to whether the stenotic lesions were positioned proximally or distally within the pulmonary arterial tree, all analyses incorporated this classification. Results: We reviewed stenoses in 203 pulmonary arteries, 38% located proximally and 62% distally, with follow-up available concerning dilation in 92 vessels. Proximal dilations frequently involved a prior surgical site, and appeared more compliant and amenable to conventional angioplasty, as evidenced by more common elimination of the waist, but also more recoil, then requiring placement of stents. In contrast, distal lesions frequently required balloons capable of sustaining high pressures of inflation, and larger balloons relative to the size of the vessels. The proportional increase in diameter was greater for distal sites, at 90 plus or minus 77%, compared to proximal, at 64 plus or minus 70%, p equal to 0.002. Serious adverse events occurred in 3 of 104 procedures, giving a rate of serious adverse events of 2.9%. At follow-up, 9 of 92 vessels (10%), 95% confidence intervals from 5% to 18%, returned to their diameters prior to dilation, with no difference in the rate of restenosis according to the site of dilation. Conclusion: Our findings indicate the need to distinguish, and to consider, the important differences in technical issues and outcomes, when performing dilations at proximal as opposed to distal sites. Although angioplasty is effective therapy for pulmonary arterial stenosis, a subset of vessels, more often distal, remain resistant to conventional techniques.
Background: We sought to determine if the acute gains in pulmonary arterial diameter achieved by using Cutting Balloons to dilate stenoses in small arteries are maintained at follow-up. Methods: A search of our database identified all patients who underwent dilation with Cutting Balloons for pulmonary arterial stenosis between June 2001 and September 2003. We reviewed the procedural and angiographic data obtained at the initial and follow-up procedures. Results: Over the period of study, 29 patients with a median age of 3.7 years, and a range from 0.4 to 46.6, underwent treatment with Cutting Balloons at 41 procedures for one or more pulmonary arterial obstructions resistant to conventional angioplasty. At the initial procedure, we enlarged 79 vessels, with an initial minimal luminal diameter of 1.5 plus or minus 0.8 millimetres, to a diameter of 3.0 plus or minus 1.1 millimetres (p smaller than 0.001). Angiographic data indicated that 49% of the vessels showed evidence of vascular damage not requiring intervention, while stents were placed in 9 vessels. Follow-up angiography was available for 39 of the vessels, evaluated at a median of 6 months, with a range from 3 to 24 months, after the initial procedure. In these vessels, there was a mean loss of 10 plus or minus 25% in luminal diameter, p not significant. Of the 39 vessels, 8 had returned to a diameter within half of the initial diameter, giving a failure rate at follow-up of 21%, with 95% confidence intervals from 9% to 36%. All complications were identified within 24 hours of the catheterization procedure, and there were no late complications or deaths during the period of follow-up. Placement of stents produced both greater increases in diameter at the initial procedure (p smaller than 0.001), and greater losses at follow-up (p equal to 0.01). Conclusions: Despite the frequent identification of intravascular trauma, initial gains in luminal diameter after use of Cutting Balloons to dilate stenotic pulmonary arteries are maintained at follow-up.
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