Deletion of 22q11.2 (del22q11) is associated with adverse outcomes in patients with tetralogy of Fallot (TOF). We sought to investigate its contribution to perioperative outcome in patients with a severe form of TOF characterized by pulmonary atresia (PA) or severe pulmonary stenosis (PS) and major aortopulmonary collateral arteries (MAPCAS). We conducted a retrospective review of patients with TOF/MAPCAS who underwent staged surgical reconstruction between 1995 and 2006. Groups were compared according to 22q11.2 deletion status using t-tests or the Wilcoxon Rank sum test. We included 26 subjects, 24 of whom survived the initial operation. Of those, 21 subjects had known deletion status and constitute the group for this analysis [15 with no deletion present (ND) and 6 del22q11 subjects]. There was no difference with respect to occurrence of palliative procedure prior to initial operation, or to timing of closure of the ventricular septal defect (VSD). Other than higher prevalence of prematurity (50%) in the del22q11 group versus no prematurity in the ND, the groups were comparable in terms of pre-operative characteristics. The intra- and post-operative course outcomes (length of cardiopulmonary bypass, use of vasopressors, duration of intensive care and length of hospital stay, tube-feeding) were also comparable. Although the del22q11 had longer mechanical ventilation than the ND, this difference was not significant [68 h (range 4-251) vs. 45 h (range 3-1005), p = 0.81]. In this detailed comparison of a small patient cohort, 22q11.2 deletion syndrome was not associated with adverse perioperative outcomes in patients with TOF, PA, and MAPCAS when compared to those without 22q11.2 deletion syndrome. These results are relevant to prenatal and neonatal pre-operative counseling and planning.

Tetralogy of Fallot (TOF) often carries long-term seqüelae following surgical intervention. We hypothesized that early perioperative factors are associated with long-term adverse right ventricular (RV) remodeling, diminished exercise capacity, and increased morbidity. We conducted a retrospective cohort study of patients operated for TOF that underwent cardiac magnetic resonance imaging study (CMR), exercise stress test (EST), and detailed review of past medical history. Outcome variables included measures of RV size, and function, maximal work rate, and oxygen consumption, and interim hospitalizations, surgeries, and catheterizations. Thirty-nine subjects were included. Age at surgical repair was 0.3 ± 0.3 years and age at testing was 9.7 ± 1.4 years. On CMR, there was borderline RV dilation with moderate pulmonary insufficiency (PI) [RF 32% (8; 43)] and normal RV ejection fraction [60% (55; 67)]. On EST, there was low percent-predicted maximal oxygen consumption (77 ± 20%), and percent-predicted maximal work rate (84 ± 23%). On multivariable analysis, mechanical ventilation and Blalock-Taussig (BT) shunt prior to complete surgical repair were associated with the number of future hospitalizations. Duration of cardiopulmonary bypass and prior BT shunt were associated with future catheterizations. Prior BT shunt was a predictor of worse RVEF, while duration of mechanical ventilation and use of transannular patch were predictors of worse PI. Longer duration of mechanical ventilation (or LOS) was associated with worse maximal work rate. Surgical and perioperative factors may portend long-term RV remodeling and outcome in TOF. Further studies are warranted to explore these associations and potential underlying mechanisms.

Background

Patients with repaired tetralogy of Fallot experience variable outcomes for reasons that are incompletely understood. We hypothesize that genetic variants contribute to this variability. We sought to investigate the association of 22q11.2 deletion status with clinical outcome in patients with repaired tetralogy of Fallot.

Methods and results

We performed a cross-sectional study of tetralogy of Fallot subjects who were tested for 22q11.2 deletion, and underwent cardiac magnetic resonance, exercise stress test, and review of medical history. We studied 165 subjects (12.3±3.1 years), of which 30 (18%) had 22q11.2 deletion syndrome (22q11.2DS). Overall, by cardiac magnetic resonance the right ventricular ejection fraction was 60±8%, pulmonary regurgitant fraction was 34±17%, and right ventricular end-diastolic volume was 114±39 cc/m(2). On exercise stress test, maximum oxygen consumption was 76±16% predicted. Despite comparable right ventricular function and pulmonary regurgitant fraction, on exercise stress test the 22q11.2DS had significantly lower percent predicted: forced vital capacity (61.5±16 versus 80.5±14; P<0.0001), maximum oxygen consumption (61±17 versus 80±12; P<0.0001), and work (64±18 versus 86±22, P=0.0002). Similarly, the 22q11.2DS experienced more hospitalizations (6.5 [5-10] versus 3 [2-5]; P<0.0001), saw more specialists (3.5 [2-9] versus 0 [0-12]; P<0.0001), and used ≥1 medications (67% versus 34%; P<0.001).

Conclusions

22q11.2DS is associated with restrictive lung disease, worse aerobic capacity, and increased morbidity, and may explain some of the clinical variability seen in tetralogy of Fallot. These findings may provide avenues for intervention to improve outcomes, and should be re-evaluated longitudinally because these associations may become more pronounced with time.

Previous studies of outcome after operative correction of interrupted aortic arch (IAA) have focused on mortality and rates of reintervention. We sought to investigate the clinical status of children and adolescents after surgery for IAA. A cross-sectional study of subjects with IAA between the ages of 8 and 18 years was performed with the subjects undergoing simultaneous genetic testing, electrocardiogram, cardiac magnetic resonance imaging, cardiopulmonary exercise testing, and assessment of health status and health-related quality of life as well as concurrent retrospective cohort study reviewing their postoperative use of medical care, including operative and transcatheter reinterventions, noncardiac surgeries, and hospitalizations. Twenty-one subjects with IAA with median age of 9 years were studied. Reintervention rates were 38% for left-ventricular outflow tract, 33% for AA, and 24% for both. Rates of reintervention were highest in the first year of life and decreased in subsequent years. Left-ventricular ejection fraction was preserved (72 ± 6%). Maximal oxygen consumption, maximal work, and forced vital capacity were both significantly decreased from age and sex norms (p < 0.0001). Health status and quality of life were both severely decreased. Subjects with IAA demonstrate a significant burden of operative and transcatheter intervention and large magnitude deficits in exercise performance, health status, and health-related quality of life.

Background

Studies of outcome after operative correction of truncus arteriosus communis (TA) have focused on mortality and rates of reintervention. We sought to investigate the clinical status of children and adolescents with surgically corrected TA.

Methods and results

A cross-sectional study of subjects with TA was performed. Subjects underwent concurrent genetic testing, electrocardiogram, cardiac magnetic resonance imaging, cardiopulmonary exercise testing, and completed questionnaires assessing health status and health-related quality of life. Review of their medical history provided retrospective information on cardiac reintervention and use of medical care. Twenty-five subjects with a median age of 11.8 (8.1-18.99) years were enrolled. The prevalence of 22q11.2 deletion was 32%. Incidence of hospitalization, cardiac reintervention, and noncardiac operations was highest in the first year of life. Combined catheter-based and operative reintervention rates were 52% on the conduit and 56% on the pulmonary arteries. Right ventricular ejection fraction and end-diastolic volume were normal. Moderate or greater truncal valve insufficiency was seen in 11% of subjects, and truncal valve replacement occurred in 8% of subjects. Maximal oxygen consumption (P = .0002), maximal work (P < .0001), and forced vital capacity (P < .0001) were all lower than normal for age and sex. Physical health status and health-related quality of life were both severely diminished.

Conclusion

Patients with TA demonstrate significant comorbid disease throughout childhood, significant burden of operative and catheter-based reintervention, and deficits in exercise performance, functional status, and health-related quality of life.

Objective

The effect of genotype on the outcomes of infant cardiac operations has not been well established. The purpose of the present study was to investigate the effect of 22q11.2 deletion (22q11del) on infants with truncus arteriosus communis (TA) and interrupted aortic arch (IAA) undergoing operative correction during infancy.

Methods

We conducted a retrospective cohort study of all infants who had undergone operative correction of TA or IAA at the Children's Hospital of Philadelphia from 1995 to 2007, comparing the perioperative outcomes (hospital length of stay, intensive care, mechanical ventilation, risk of cardiac and noncardiac events, number of consultations, and number of discharge medications) by 22q11del status.

Results

A total of 104 patients were studied (55 with TA and 49 with IAA), of whom 40 (38%) were 22q11del positive. The 22q11del status was unknown in 9 (7 with TA and 2 with IAA). In patients with known deletion status, those with 22q11del had a longer hospital and intensive care length of stay. Subjects with 22q11del also required more frequent operative reintervention and more consultations and were prescribed more medications at discharge. No significant difference was found in method of feeding between those with and without 22q11del at discharge.

Conclusions

In this study, 22q11del is associated with perioperative outcomes in infants undergoing operative correction of TA and IAA, with longer hospital stays and greater resource utilization in the perioperative period. These findings should inform counseling and risk stratification and warrant additional study to identify genotype-specific management strategies to improve outcomes.

Background

Postoperative length of stay (LOS) is an important quality metric and is known to vary widely across hospitals after congenital heart surgery. Whether this variability is explained by factors associated with the intensive care unit (ICU) or acute care unit (ACU) remains unclear. We evaluated the relationship between ICU and ACU LOS and the impact of ACU characteristics on postoperative LOS.

Methods

Hospitalizations for congenital heart surgery within the Pediatric Cardiac Critical Care Consortium (PC⁴) registry (August 2014 to February 2018) were included. Models were developed for ICU, ACU, and postoperative LOS by adjusting for differences in case-mix across hospitals. PC⁴ hospitals participating in the Pediatric Acute Care Cardiology Collaborative (PAC³) were also surveyed on ACU organizational factors and practice patterns.

Results

Overall, 19,674 hospitalizations across 27 hospitals were included. There was significant variation in ICU and ACU LOS. Postperative LOS appeared to be most closely related to ICU LOS; 75% (6 of 8) of hospitals with shorter than expected postoperative LOS also had shorter than expected ICU LOS. A clear relationship between postoperative and ACU LOS was not observed. Hospitals with an ACU able to provide higher-acuity care as indexed according to the PAC³ survey were more likely to have shorter postoperative LOS (P < .01).

Conclusions

For hospitals that achieve shorter than expected postoperative LOS after congenital heart surgery, ICU LOS appears to be the primary driver. Higher-acuity resources in the ACU may be an important factor facilitating earlier transfer from the ICU. These data are key to informing quality improvement initiatives geared toward reducing postoperative LOS.

Background The objective of this international multicenter study was to investigate both early and late outcomes of cardiac resynchronization therapy (CRT) in patients with a systemic right ventricle (SRV) and to identify predictors for congestive heart failure readmissions and mortality. Methods and Results This retrospective international multicenter study included 13 centers. The study population comprised 80 adult patients with SRV (48.9% women) with a mean age of 45±14 (range, 18-77) years at initiation of CRT. Median follow-up time was 4.1 (25th-75th percentile, 1.3-8.3) years. Underlying congenital heart disease consisted of congenitally corrected transposition of the great arteries and dextro-transposition of the great arteries in 63 (78.8%) and 17 (21.3%) patients, respectively. CRT resulted in significant improvement in functional class (before CRT: III, 25th-75th percentile, II-III; after CRT: II, 25th-75th percentile, II-III; P=0.005) and QRS duration (before CRT: 176±27; after CRT: 150±24 milliseconds; P=0.003) in patients with pre-CRT ventricular pacing who underwent an upgrade to a CRT device (n=49). These improvements persisted during long-term follow-up with a marginal but significant increase in SRV function (before CRT; 30%, 25th-75th percentile, 25-35; after CRT: 31%, 25th-75th percentile, 21-38; P=0.049). In contrast, no beneficial change in the above-mentioned variables was observed in patients who underwent de novo CRT (n=31). A quarter of all patients were readmitted for heart failure during follow-up, and mortality at latest follow-up was 21.3%. Conclusions This international experience with CRT in patients with an SRV demonstrated that CRT in selected patients with SRV dysfunction and pacing-induced dyssynchrony yielded consistent improvement in QRS duration and New York Heart Association functional status, with a marginal increase in SRV function.

Background

Nearly every child undergoing congenital heart surgery has chest tubes placed intraoperatively. Center variation in removal practices and impact on outcomes has not been well described. This study evaluated variation in chest tube management practices and outcomes across centers.

Methods

The study included patients undergoing any of 10 benchmark operations from June 2017 to May 2018 at participating Pediatric Acute Care Cardiology Collaborative (PAC³) and Pediatric Cardiac Critical Care Consortium (PC⁴) centers. Clinical data from PC⁴ centers were merged with chest tube data from PAC³ centers. Practices and outcomes were compared across centers in univariate and multivariable analysis.

Results

The cohort included 1029 patients (N = 9 centers). Median chest tube duration varied significantly across centers for 9 of 10 benchmark operations (all P ≤ .03), with a "model" center noted to have the shortest duration for 9 of 10 operations (range, 27.9% to 87.4% shorter duration vs other centers across operations). This effect persisted in multivariable analysis (P < .0001). The model center had higher volumes of chest tube output before removal (median, 8.5 mL/kg/24 h [model] vs 2.2 mL/kg/24 h [other centers]; P < .001], but it did not have higher rates of chest tube reinsertion (model center 1.3% vs 2.1%; P = .59) or readmission for pleural effusion (model center 4.4% vs 3.0%; P = .31), and had the shortest length of stay for 7 of 10 operations.

Conclusions

This study suggests significant center variation in chest tube removal practices and associated outcomes after congenital heart surgery. Best practices used at the model center have informed the design of an ongoing collaborative learning project aimed at reducing chest tube duration and length of stay.