The management of hypoplastic left heart syndrome (HLHS) has changed substantially over the past four decades. In the 1970s, children with HLHS could only be provided with supportive care. As a ...result, most of these unfortunate children died within the neonatal period. The advent of the Norwood procedure in the early 1980s has changed the prognosis for these children, and the majority now undergoing a series of three surgical stages that can support survival beyond the neonatal period and into early adulthood. This review will focus on the Norwood procedure and the other important innovations of the last half century that have improved our outlook toward children born with HLHS.
Full text
Available for:
EMUNI, FIS, FZAB, GEOZS, GIS, IJS, IMTLJ, KILJ, KISLJ, MFDPS, NLZOH, NUK, OILJ, PNG, SAZU, SBCE, SBJE, SBMB, SBNM, UKNU, UL, UM, UPUK, VKSCE, ZAGLJ
We sought to describe characteristics and operative outcomes of children who underwent repair of truncus arteriosus and identify risk factors for the occurrence of major adverse cardiac events (MACE) ...in the immediate postoperative period in a contemporary multicenter cohort.
We conducted a retrospective review of children who underwent repair of truncus arteriosus between 2009 and 2016 at 15 centers within the United States. Patients with associated interrupted or obstructed aortic arch were excluded. MACE was defined as the need for postoperative extracorporeal membrane oxygenation, cardiopulmonary resuscitation, or operative mortality. Risk factors for MACE were identified using multivariable logistic regression analysis and reported as odds ratios (ORs) with 95% confidence intervals (CIs).
We reviewed 216 patients. MACE occurred in 44 patients (20%) and did not vary significantly over time. Twenty-two patients (10%) received postoperative extracorporeal membrane oxygenation, 26 (12%) received cardiopulmonary resuscitation, and 15 (7%) suffered operative mortality. With multivariable logistic regression analysis (which included adjustment for center effect), factors independently associated with MACE were failure to diagnose truncus arteriosus before discharge from the nursery (OR, 3.1; 95% CI, 1.3-7.4), cardiopulmonary bypass duration >150 minutes (OR, 3.5; 95% CI, 1.5-8.5), and right ventricle-to-pulmonary artery conduit diameter >50 mm/m2 (OR, 4.7; 95% CI, 2.0-11.1).
In a contemporary multicenter analysis, 20% of children who underwent repair of truncus arteriosus experienced MACE. Early diagnosis, shorter duration of cardiopulmonary bypass, and use of smaller diameter right ventricle-to-pulmonary artery conduits represent potentially modifiable factors that could decrease morbidity and mortality in this fragile patient population.
Full text
Available for:
GEOZS, IJS, IMTLJ, KILJ, KISLJ, NLZOH, NUK, OILJ, PNG, SAZU, SBCE, SBJE, UILJ, UL, UM, UPCLJ, UPUK, ZAGLJ, ZRSKP
Abstract Objective In a multicenter cohort of neonates recovering from cardiac surgery, we sought to describe the epidemiology of extubation failure and its variability across centers, identify risk ...factors, and determine its impact on outcomes. Methods We analyzed prospectively collected clinical registry data on all neonates undergoing cardiac surgery in the Pediatric Cardiac Critical Care Consortium database from October 2013 to July 2015. Extubation failure was defined as reintubation less than 72 hours after the first planned extubation. Risk factors were identified using multivariable logistic regression with generalized estimating equations to account for within-center correlation. Results The cohort included 899 neonates from 14 Pediatric Cardiac Critical Care Consortium centers; 14% were premature, 20% had genetic abnormalities, 18% had major extracardiac anomalies, and 74% underwent surgery with cardiopulmonary bypass. Extubation failure occurred in 103 neonates (11%), within 24 hours in 61%. Unadjusted rates of extubation failure ranged from 5% to 22% across centers; this variability was unchanged after adjusting for procedural complexity and airway anomaly. After multivariable analysis, only airway anomaly was identified as an independent risk factor for extubation failure (odds ratio, 3.1; 95% confidence interval, 1.4-6.7; P = .01). Neonates who failed extubation had a greater median postoperative length of stay (33 vs 23 days, P < .001) and in-hospital mortality (8% vs 2%, P = .002). Conclusions This multicenter study showed that 11% of neonates recovering from cardiac surgery fail initial postoperative extubation. Only congenital airway anomaly was independently associated with extubation failure. We observed a 4-fold variation in extubation failure rates across hospitals, suggesting a role for collaborative quality improvement to optimize outcomes.
Full text
Available for:
GEOZS, IJS, IMTLJ, KILJ, KISLJ, NLZOH, NUK, OILJ, PNG, SAZU, SBCE, SBJE, UILJ, UL, UM, UPCLJ, UPUK, ZAGLJ, ZRSKP
Literature describing morbidity and mortality after truncus arteriosus repair is predominated by single-center reports. We created and analyzed a multicenter dataset to identify risk factors for late ...mortality and right ventricle-to-pulmonary artery (RV-PA) conduit reintervention for this patient population.
We retrospectively collected data on children who underwent repair of truncus arteriosus without concomitant arch obstruction at 15 centers between 2009 and 2016. Cox regression survival analysis was conducted to determine risk factors for late mortality, defined as death occurring after hospital discharge and greater than 30 days after operation. Probability of any RV-PA conduit reintervention was analyzed over time using Fine-Gray modeling.
We reviewed 216 patients with median follow-up of 2.9 years (range, 0.1 to 8.8). Operative mortality occurred in 15 patients (7%). Of the 201 survivors there were 14 (7%) late deaths. DiGeorge syndrome (hazard ratio HR, 5.4; 95% confidence interval CI, 1.6 to 17.8) and need for postoperative tracheostomy (HR, 5.9; 95% CI, 1.8 to 19.4) were identified as independent risk factors for late mortality. At least one RV-PA conduit catheterization or surgical reintervention was performed in 109 patients (median time to reintervention, 23 months; range, 0.3 to 93). Risk factors for reintervention included use of pulmonary or aortic homografts versus Contegra (Medtronic, Inc, Minneapolis, MN) bovine jugular vein conduits (HR, 1.9; 95% CI, 1.2 to 3.1) and smaller conduit size (HR per mm/m2, 1.05; 95% CI, 1.03 to 1.08).
In a multicenter dataset DiGeorge syndrome and need for tracheostomy postoperatively were found to be independent risk factors for late mortality after repair of truncus arteriosus, whereas risk of conduit reintervention was independently influenced by both initial conduit type and size.
Display omitted
We sought to report characteristics and outcomes of children with complex anomalous left coronary artery from the pulmonary artery (ALCAPA) and to compare the outcomes to children with isolated ...ALCAPA. We performed a retrospective data analysis of children with ALCAPA who underwent cardiac surgery between 1/2009 and 3/2018 at 21 centers. Characteristics and outcomes of patients with complex ALCAPA are provided using descriptive statistics. Outcomes were compared between complex ALCAPA and isolated ALCAPA using Fisher's exact test. We reviewed 258 patients who underwent surgical repair of ALCAPA at 21 centers. We identified 10 patients (3.9%) with complex ALCAPA. Median age at initial cardiac surgery was 49 days (range: 4 days, 12.8 years). Cardiac lesions associated with ALCAPA were HLHS (n = 3); scimitar syndrome (n = 2); VSD with aortic coarctation (n = 2); VSD with right pulmonary artery discontinuity (1); DORV with mitral atresia (n = 1); and ToF (n = 1). ALCAPA was diagnosed prior to surgical intervention in 1 patient; during the initial cardiac surgery in 4 patients; in the early postoperative period via cardiac catheterization in 3 patients; and later in childhood after initial surgical repair in 2 patients. Following ALCAPA repair, patients with complex ALCAPA, as compared to patients with isolated ALCAPA, were more likely to be placed on ECMO (50% vs 12%, p = 0.002), receive CPR (30% vs 6%, p = 0.017), or suffer operative mortality (50% vs 3%, p < 0.001). Complex ALCAPA is uncommon. All but one with complex ALCAPA was not diagnosed preoperatively and postoperative morbidity and mortality were significantly greater in these complex patients compared to patients with isolated ALCAPA.
Full text
Available for:
EMUNI, FIS, FZAB, GEOZS, GIS, IJS, IMTLJ, KILJ, KISLJ, MFDPS, NLZOH, NUK, OBVAL, OILJ, PNG, SAZU, SBCE, SBJE, SBMB, SBNM, UKNU, UL, UM, UPUK, VKSCE, ZAGLJ
Objectives To describe the epidemiology of extubation failure and identify risk factors for its occurrence in a multicenter population of neonates undergoing surgery for congenital heart disease. ...Study design We conducted a prospective observational study of neonates ≤30 days of age who underwent cardiac surgery at 7 centers within the US in 2015. Extubation failure was defined as reintubation within 72 hours of the first planned extubation. Risk factors were identified with the use of multivariable logistic regression analysis and reported as OR with 95% CIs. Multivariable logistic regression analysis was conducted to examine the relationship between extubation failure and worse clinical outcome, defined as hospital length of stay in the upper 25% or operative mortality. Results We enrolled 283 neonates, of whom 35 (12%) failed their first extubation at a median time of 7.5 hours (range 1-70 hours). In a multivariable model, use of uncuffed endotracheal tubes (OR 4.6; 95% CI 1.8-11.6) and open sternotomy of 4 days or more (OR 4.8; 95% CI 1.3-17.1) were associated independently with extubation failure. Accordingly, extubation failure was determined to be an independent risk factor for worse clinical outcome (OR 5.1; 95% CI 2-13). Conclusions In this multicenter cohort of neonates who underwent surgery for congenital heart disease, extubation failure occurred in 12% of cases and was associated independently with worse clinical outcome. Use of uncuffed endotracheal tubes and prolonged open sternotomy were identified as independent and potentially modifiable risk factors for the occurrence of this precarious complication.
Full text
Available for:
GEOZS, IJS, IMTLJ, KILJ, KISLJ, NUK, OILJ, PNG, SAZU, SBCE, SBJE, UL, UM, UPCLJ, UPUK, ZRSKP
Respiratory complications are common in patients who require venoarterial (VA) extracorporeal membrane oxygenation (ECMO) for cardiac indications. We aimed to examine the frequency and ...characteristics of patients who develop multi-lobar atelectasis early in the course of VA ECMO and to identify factors associated with its occurrence.
We performed a single-center retrospective review of consecutive pediatric subjects on VA ECMO in the cardiovascular ICU from 2014 to 2019. Chest radiographs before VA ECMO initiation and daily for up to 5 d of VA ECMO support were reviewed. Multi-lobar atelectasis was defined as the collapse of ≥2 lobes of the same lung. Patients with multi-lobar atelectasis before or immediately after VA ECMO cannulation were excluded. Bivariate comparisons and multivariable logistic regression analyses were performed to identify factors independently associated with lung collapse. Results of the multivariable analysis are provided as odds ratio (OR) with 95% CI.
We reviewed 119 VA ECMO runs in 101 unique subjects. Multi-lobar atelectasis occurred in 36 runs (30%), with an isolated collapse of the left lung occurring most frequently (no. runs = 20). VA ECMO runs complicated by multi-lobar atelectasis were significantly longer and associated with lower hospital survival (51% vs 77%, survival in subjects without multi-lobar atelectasis;
= .01). Multivariable logistic regression analysis identified a subsequent ECMO run during the same admission to be independently associated with multi-lobar atelectasis (OR 5.4, 95% CI 1.2-21.5). Subanalysis of the subjects with isolated left lung collapse revealed male sex (OR 8.9, 95% CI 1.6-48.2) and subsequent ECMO run during the same admission (OR 4.0, 95% CI 1.2-13.6) to be independently associated with this complication, and mechanical ventilation at least 12 h before ECMO may be protective (OR 0.22, 95% CI 0.07-0.76).
Multi-lobar atelectasis commonly occurred in children who were receiving VA ECMO for cardiac failure and was associated with worse outcomes. Male patients, a subsequent VA ECMO run during the same hospitalization, and patients in whom mechanical ventilation was initiated shortly before ECMO cannulation may be at increased risk for this complication.
Abstract Objectives We aimed to further validate the vasoactive-ventilation-renal score as a predictor of outcome in patients recovering from surgery for congenital heart disease. We also sought to ...determine the optimal time point within the early recovery period at which the vasoactive-ventilation-renal score should be measured. Methods We prospectively reviewed consecutive patients recovering from cardiac surgery within our intensive care unit between January 2015 and June 2015. The vasoactive-ventilation-renal score was calculated at 6, 12, 24, and 48 hours postoperatively as follows: vasoactive-ventilation-renal score = ventilation index + vasoactive-inotrope score + Δ creatinine change in serum creatinine from baseline*10. Primary outcome of interest was prolonged hospital length of stay, defined as length of stay in the upper 25%. Receiver operating characteristic curves were generated, and areas under the curve with 95% confidence intervals were calculated for all time points. Multivariable logistic regression modeling also was performed. Results We reviewed 164 patients with a median age of 9.25 months (interquartile range, 2.6-58 months). Median length of stay was 8 days (interquartile range, 5-17.5 days). The area under the curve value for the vasoactive-ventilation-renal score as a predictor of prolonged length of stay (>17.5 days) was greatest at 12 hours postoperatively (area under the curve = 0.93; 95% confidence interval, 0.89-0.97). On multivariable regression analysis, after adjustment for potential confounders, the 12-hour vasoactive-ventilation-renal score remained a strong predictor of prolonged hospital length of stay (odds ratio, 1.15; 95% confidence interval, 1.10-1.20). Conclusions In a heterogeneous population of patients undergoing surgery for congenital heart disease, the novel vasoactive-ventilation-renal score calculated in the early postoperative recovery period can be a strong predictor of prolonged hospital length of stay.
Full text
Available for:
GEOZS, IJS, IMTLJ, KILJ, KISLJ, NLZOH, NUK, OILJ, PNG, SAZU, SBCE, SBJE, UILJ, UL, UM, UPCLJ, UPUK, ZAGLJ, ZRSKP
Early extubation following pediatric cardiac surgery is common, but debate exists whether location affects outcome, with some centers performing routine early extubations in the operating room (odds ...ratio) and others in the cardiac ICU. We aimed to define early extubation practice variation across hospitals and assess impact of location on hospital length-of-stay and other outcomes.
Secondary analysis of the Pediatric Cardiac Critical Care Consortium registry.
Twenty-eight Pediatric Cardiac Critical Care Consortium hospitals.
Patients undergoing Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery mortality category 1-3 operations between August 2014 and February 2018.
None.
We defined early extubation as extubation less than 6 hours after postoperative admission. Hospitals were categorized based on the proportion of their early extubation patients who underwent an odds ratio extubation. Categories included low- (< 50% of early extubation, n = 12), medium- (50%-90%, n = 8), or high- (> 90%, n = 8) frequency odds ratio early extubation centers. The primary outcome of interest was postoperative hospital length-of-stay. We analyzed 16,594 operations (9,143 early extubation, 55%). Rates of early extubation ranged from 16% to 100% across hospitals. Odds ratio early extubation rates varied from 16% to 99%. Patient characteristics were similar across hospital odds ratio early extubation categories. Early extubation rates paralleled the hospital odds ratio early extubation rates-77% patients underwent early extubation at high-frequency odds ratio extubation centers compared with 39% at low-frequency odds ratio extubation centers (p < 0.001). High- and low-frequency odds ratio early extubation hospitals had similar length-of-stay, cardiac arrest rates, and low mortality. However, high-frequency odds ratio early extubation hospitals used more noninvasive ventilation than low-frequency hospitals (15% vs. 9%; p < 0.01), but had fewer extubation failures (3.6% vs. 4.5%; p = 0.02).
Considerable variability exists in early extubation practices after low- and moderate-complexity pediatric cardiac surgery. In this patient population, hospital length-of-stay did not differ significantly between centers with different early extubation strategies based on location or frequency.
PDF
