Atrial septal interventions during and after hybrid stage I palliation of hypoplastic left heart syndrome.

BACKGROUND: Hybrid stage I palliation (HS1P) is an alternative approach for initial palliation in hypoplastic left heart syndrome (HLHS) patients. Unlike surgical stage I palliation where atrial septectomy is routinely performed, atrial septal intervention (ASI) during HS1P is variable. In this study, we described our experience with ASI in single ventricle (SV) patients who underwent HS1P and identified factors associated with need for ASI after HS1P. METHODS: Data were retrospectively collected for all HLHS patients who underwent HS1P at our center over the past 12 years. We evaluated ASIs performed during the HS1P (intra-HS1P ASI) and ASIs performed during the period from HS1P to the subsequent surgical stage, either interval Norwood stage I or comprehensive stage II (post-HS1P ASI). Patient factors and procedural data were compared to identify factors associated with undergoing post-HS1P ASI and the impact of ASI on patient outcomes was evaluated. RESULTS: Of 50 SV patients included, 23 (46%) underwent intra-HS1P ASI and 26 (52%) underwent post-HS1P ASI. Need for post-HS1P ASI was lower among patients who had an intra-HS1P ASI as compared to those who did not (30% vs. 70%; p = 0.005). There were no significant differences in short or Midterm outcomes between patients who underwent intra-HS1P ASI or post-HS1P ASI and their counterparts. CONCLUSIONS: ASI is common both during and after HS1P but is generally well tolerated and type of ASI does not significantly impact overall patient outcomes. Our findings suggest that the current approach of individualizing management of ASI in the HS1P population is effective and safe.

Reducing Hyperoxia Exposure in Infants Requiring Veno-Arterial Extracorporeal Membrane Oxygenation after Cardiac Surgery.

Recent studies have suggested worse outcomes in patients exposed to hyperoxia while supported on veno-arterial extracorporeal membrane oxygenation (VA-ECMO). However, there are no data regarding the effect of reducing hyperoxia exposure in this population by adjusting the fraction of inspired oxygen (FiO2) of the sweep gas of the ECMO circuit. A retrospective review of 143 patients less than 1 year of age requiring VA-ECMO following cardiac surgery from 2007 to 2018 was completed. 64 patients had a FiO2 of the sweep gas < 100% with an average PaO2 of 210 mm Hg in the first 48 h of support [vs 405 mm Hg in the group with a FiO2 = 100% (p < 0.0001)]. There was no difference in mortality at 30 days after surgery or other markers of end-organ injury with respect to whether the FiO2 was adjusted. At least one PaO2 value < 200 mm Hg in the first 24 h on ECMO in patients with a FiO2 < 100% trended toward a significant association (OR = 0.45, 95% CI = 0.21-1.01) with decreased risk of 30-day mortality when compared to those patients with a FiO2 = 100% and all PaO2 values > 200 mm Hg. Only 47% of patients with a FiO2 < 100% had an average PaO2 less than 200 mm Hg which indicates that the intervention of reducing the FiO2 of the sweep gas was not entirely effective at reducing hyperoxia exposure. Future research is needed for developing clinical protocols to avoid hyperoxia and to identify mechanisms for hyperoxia-induced injury on VA-ECMO.

Failed Extubation in Neonates After Cardiac Surgery: A Single-Center, Retrospective Study.

OBJECTIVES: To describe factors associated with failed extubation (FE) in neonates following cardiovascular surgery, and the relationship with clinical outcomes. DESIGN: Retrospective cohort study. SETTING: Twenty-bed pediatric cardiac ICU (PCICU) in an academic tertiary care children's hospital. PATIENTS: Neonates admitted to the PCICU following cardiac surgery between July 2015 and June 2018. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Patients who experienced FE were compared with patients who were successfully extubated. Variables associated with FE ( p < 0.05) from univariate analysis were considered for inclusion in multivariable logistic regression. Univariate associations of FE with clinical outcomes were also examined. Of 240 patients, 40 (17%) experienced FE. Univariate analyses revealed associations of FE with upper airway (UA) abnormality (25% vs 8%, p = 0.003) and delayed sternal closure (50% vs 24%, p = 0.001). There were weaker associations of FE with hypoplastic left heart syndrome (25% vs 13%, p = 0.04), postoperative ventilation greater than 7 days (33% vs 15%, p = 0.01), Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery (STAT) category 5 operations (38% vs 21%, p = 0.02), and respiratory rate during spontaneous breathing trial (median 42 vs 37 breaths/min, p = 0.01). In multivariable analysis, UA abnormalities (adjusted odds ratio [AOR] 3.5; 95% CI, 1.4-9.0), postoperative ventilation greater than 7 days (AOR 2.3; 95% CI, 1.0-5.2), and STAT category 5 operations (AOR 2.4; 95% CI, 1.1-5.2) were independently associated with FE. FE was also associated with unplanned reoperation/reintervention during hospital course (38% vs 22%, p = 0.04), longer hospitalization (median 29 vs 16.5 d, p < 0.0001), and in-hospital mortality (13% vs 3%, p = 0.02). CONCLUSIONS: FE in neonates occurs relatively commonly following cardiac surgery and is associated with adverse clinical outcomes. Additional data are needed to further optimize periextubation decision-making in patients with multiple clinical factors associated with FE.

Contemporary Outcomes of Tracheostomy in Patients With Single Ventricle Heart Lesions.

OBJECTIVES: Approximately 0.2% to 2.7% of children with congenital heart disease require a tracheostomy after cardiac surgery with the majority having single ventricle (SV) type heart lesions. Tracheostomy in SV patients is reported to be associated with high mortality. We hypothesized that short- and long-term survival of patients with SV heart disease would vary according to tracheostomy indication. METHODS: This is a single center, 20-year, retrospective review of all patients with SV heart disease who underwent tracheostomy. Demographic, cardiac anatomy, surgical, intensive care unit, and hospital course data were collected. The primary outcome was survival following tracheostomy. Secondary outcome was the completion of staged palliation to Fontan. RESULTS: In total, 25 patients with SV heart disease who underwent tracheostomy were included. Indications for tracheostomy included one or more of the following: tracheobronchomalacia (n = 8), vocal cord paralysis (n = 7), tracheal/subglottic stenosis (n = 6), primary respiratory insufficiency (n = 4), diaphragm paralysis (n = 3), suboptimal hemodynamics (n = 2), and other upper airway issues (n = 1). Survival at six months, one year, five years, and ten years was 76%, 68%, 63%, and 49%, respectively. Most patients completed Fontan palliation (64%). Patients who underwent tracheostomy for suboptimal hemodynamics and/or respiratory insufficiency had a higher mortality risk compared to those with indications of upper airway obstruction or diaphragm paralysis (hazard ratio 4.1, 95% confidence interval 1.2-13.7; P = .02). CONCLUSIONS: Mortality risk varies according to tracheostomy indication in patients with SV heart disease. Tracheostomy may allow staged surgical palliation to proceed with acceptable risk if it was indicated for anatomic or functional airway dysfunction.

Protein-losing enteropathy recurrence after pediatric heart transplantation: Multicenter case series.

BACKGROUND: Protein-losing enteropathy (PLE) is a devastating complication of the Fontan circulation. Although orthotopic heart transplantation (HTx) typically results in resolution of PLE symptoms, isolated cases of PLE relapse have been described after HTx. METHODS: Patients with Fontan-related PLE who had undergone HTx at participating centers and experienced relapse of PLE during follow-up were retrospectively identified. Available data related to pre- and post-HTx characteristics and PLE events were collected. RESULTS: Eight patients from four different centers were identified. Median time from Fontan procedure to the development of PLE was 8 years, and median age at HTx was 17 years (range 7.7-21). In all patients, PLE resolved at a median time of 1 month after HTx (0.3-5). PLE recurrences occurred at a median time of 7.5 months after HTx (2-132). Each occurrence was associated with one or more significant clinical events; most commonly cellular- or antibody-mediated rejection; and less commonly graft dysfunction, infection, thrombosis, and posttransplant lymphoproliferative disease. PLE recurrences resolved after the successful treatment of the concomitant event, after a median time of 2 months in seven cases, while persisted and recurred in one patient in association with atypical mycobacterium infection and subsequent PTLD onset and relapses. Six patients were alive during follow-up at a median time of 4 years (1.3-22.5) after HTx. CONCLUSIONS: This is the largest series of PLE recurrence after HTx. All cases were associated with one or more concomitant and significant clinical events. PLE typically resolved after resolution of the inciting clinical event.

Oral Aversion in Infants With Congenital Heart Disease: A Single-Center Retrospective Cohort Study.

OBJECTIVES: Neonates undergoing cardiac surgery are at risk for oral aversion (OA). OA is not well described outside of the index hospitalization and impacts patients and families. We evaluated the prevalence of OA at 1 year old after neonatal cardiopulmonary bypass (CPB) surgery. DESIGN: Retrospective cohort study. SETTING: Single quaternary care hospital. SUBJECTS: Our cohort included 157 neonates who underwent CPB surgery from 2014 to 2017 and had follow-up data available at 1 year old. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Three feeding experts reviewed the medical record to define children with OA; 30% of charts were evaluated in triplicate for validation. Neonates with and without OA were compared in univariate analysis, and risk factors for OA were explored in a limited multivariable analysis. OA was present at 1 year in 37 patients (23.6%) and other feeding difficulties were present in an additional 29 patients (18.5%). Thirty-eight patients (24.2%) had a feeding tube, including 12 (7.6%) with a gastrostomy tube. Factors associated with OA at 1 year included total ICU days, duration of mechanical ventilation, total number of nil per os days, and number of postoperative days (PODs) until oral feeding initiation (all p < 0.0001). Number of POD until oral feeding initiation remained independently associated with OA at 1 year in multivariable analysis (adjusted odds ratio, 1.08; 95% CI, 1.04-1.12; p < 0.0001). Infants with any oral intake at discharge had lower odds of OA at 1 year (0.21; 95% CI, 0.08-0.5; p = 0.0003). At hospital discharge, 132 patients (84.1%) were taking some oral feeds, and 128 patients (81.5%) received tube feeding. CONCLUSIONS: OA and other feeding difficulties are common at 1 year old in neonates undergoing CPB surgery. Delayed exposure to oral intake may be a modifiable risk factor for OA and efforts to improve early oral feeding could lead to better functional outcomes.

Ventricular Function in Physiologically Repaired and Unrepaired Congenitally Corrected Transposition of the Great Arteries.

In patients with congenitally corrected transposition of the great arteries (ccTGA) and hemodynamically significant concomitant lesions, physiologic repair may be undertaken, in which the circulation is septated but the morphologic right ventricle (RV) remains the systemic ventricle. Patients without significant concomitant lesions may be observed without surgery, with a similar physiologic result. We compared cardiovascular magnetic resonance measures of ventricular size and function in patients with physiologically repaired and unrepaired ccTGA. Patients with ccTGA who underwent cardiovascular magnetic resonance at our center between September 2007 and July 2019 were analyzed. In 38 patients identified (12, physiologically repaired; 26, unrepaired; mean age 34.5 [18.7 to 52.0] years), there was a higher proportion of RV ejection fraction ≤45% in physiologically repaired (75% vs unrepaired 35%, p = 0.02). Physiologically repaired patients had worse left ventricle global longitudinal strain (-14.9% ± 5.0% vs unrepaired patients -18.4% ± 2.7%, p = 0.04). The difference in tricuspid regurgitant fraction between groups did not achieve statistical significance (physiologically repaired 27.4 ± 11.1% vs unrepaired patients 19.2 ± 13.0%, p = 0.08). Evaluation for late gadolinium enhancement was more commonly undertaken in physiologically repaired patients (8 of 12 vs unrepaired 7 of 26, p = 0.03) and present more frequently in the left ventricle in physiologically repaired patients in patients evaluated (6 of 8 vs unrepaired 0 of 7, p = 0.01). In conclusion, ventricular function is decreased in patients with ccTGA undergoing physiologic repair compared with those without previous surgery. These cohorts should be considered separately when using ventricular function as an outcome. RV dysfunction is concerning for long-term outcomes following physiologic repair.

Kommerell Diverticulum: Distinctions Between Arch Side and Evaluation of Morphology, Size, And Risk.

BACKGROUND: Kommerell diverticulum (KD) is a dilated proximal aberrant right or left subclavian artery associated with either right or left aortic arches (RAA-ARSA or LAA-ALSA). Although case series suggest that KD may be a liability for vascular complications, the risk, pattern of dilation throughout the life span, and differences between arch sides are not known. METHODS: This study was a single-center retrospective review of patients of all ages with KD on cross-sectional imaging. Maximal short-axis diameter of KD (KDmax), absolute and indexed to descending aortic diameter (DAo), was correlated with age. Comparisons were made between arch sides. Patients with vascular complications were described. RESULTS: A total of 104 patients with KD were included: 68 (65%) with RAA-ALSA, 36 (35%) with LAA-ARSA, 43 (41%) asymptomatic. Although KDmax was correlated with age (RAA-ALSA r = 0.84; [P< .0001]; LAA-ARSA r = 0.51 [P = .001]), KDmax indexed to DAo was not (RAA-ALSA r = 0.14 [P = .27]; LAA-ARSA r = -0.22 [P = .21]). Patients with RAA-ALSA had larger KDmax indexed to DAo (1.02 ± 0.20 mm/mm vs 0.89 ± 0.18 mm/mm; P = .002) and more symptoms (75% vs 28%; P < .0001), and they were younger (median, 9.5 years vs 61.7 years; P < .0001). Six patients (58 to 80 years of age) had vascular complications, and all 6 had LAA-ARSA and risk factors for acquired aneurysms. CONCLUSIONS: In older patients, KDmax indexed to DAo was not larger, thus arguing against isolated KD dilation with age. Diverticula from RAA-ALSA and LAA-ARSA demonstrated different phenotypes, a finding suggesting different disease processes and likely different risk. The incidence of vascular complications was lower than in previous reports, and these complications occurred exclusively in patients with LAA-ARSA and aneurysm risk factors. This finding suggests that conservative management of asymptomatic KD is often reasonable, especially in patients with RAA-ALSA.

Evaluation of a "tummy time" intervention to improve motor skills in infants after cardiac surgery.

BACKGROUND: Infants who require open heart surgery are at increased risk for developmental delays including gross motor impairments which may have implications for later adaptive skills and cognitive performance. We sought to evaluate the feasibility and efficacy of a tummy time intervention to improve motor skill development in infants after cardiac surgery. METHODS: Infants <4 months of age who underwent cardiac surgery were randomly assigned to tummy time with or without outpatient reinforcement or standard of care prior to hospital discharge. The Alberta Infant Motor Scale (AIMS) was administered to each infant prior to and 3 months after discharge. Groups were compared, and the association between parent-reported tummy time at home and change in motor scores at follow-up was examined. RESULTS: Parents of infants (n = 64) who had cardiac surgery at a median age of 5 days were randomly assigned to tummy time instruction (n = 20), tummy time + outpatient reinforcement (n = 21) or standard of care (n = 23). Forty-nine (77%) returned for follow-up. At follow-up, reported daily tummy time was not significantly different between groups (p = 0.17). Fifteen infants had <15 minutes of tummy time daily. Infants who received >15 minutes of tummy time daily had a significantly greater improvement in motor scores than infants with <15 minutes of tummy time daily (p = 0.01). CONCLUSION: In infants following cardiac surgery, <15 minutes of tummy time daily is associated with increased motor skill impairment. Further research is needed to elucidate the best strategies to optimise parental compliance with tummy time recommendations.

Echocardiographic Findings Associated with Transplantation-Free Survival and Left Ventricular Systolic Function at Midterm Follow-Up after Ross Procedure in Infants with Critical Aortic Stenosis.

BACKGROUND: The Ross operation is an important option for children with critical aortic stenosis with residual disease, but operation in infancy is associated with significant morbidity and mortality. The aim of this study was to evaluate echocardiographic correlates of transplantation-free survival, reintervention, and left ventricular (LV) function in midterm follow-up. METHODS: This retrospective, single-center study included all infants with critical aortic stenosis who underwent Ross by 1 year of age from January 2000 to September 2018. Serial echocardiograms were analyzed for LV ejection fraction (LVEF) and systolic and diastolic longitudinal strain. The primary outcome was mortality or transplantation; secondary outcomes were reintervention and abnormal LVEF (≤55%). RESULTS: Among 40 infants (30 male [75%]; median age at Ross, 51 days) with median follow-up duration of 3.3 years (interquartile range, 1.0-9.4 years), the primary outcome was met in 11 (28%). Rates of transplantation-free survival was 79%, 77%, and 69% at 1, 5, and 10 years after Ross. Predictors of transplantation or death included neonatal surgery, cross-clamp time, and preoperative left atrial dilatation and lower LVEF. Median freedom from reintervention was 7.1 years after Ross, with no identified associations. LV longitudinal strain improved 1 year after Ross (-21.1 ± 3.8% vs -17.4 ± 5.1%, P = .02), although LVEF did not reach significance. Lower LVEF at 1 year was related to pre-Ross left atrial dilatation (P = .02), abnormal LVEF (P = .04), and lower early diastolic longitudinal strain rate (P = .03). LVEF remained stable 3 years after Ross. CONCLUSIONS: Both transplantation-free survival and normalization of LV function after Ross in infancy are associated with preoperative LV systolic and diastolic measures, highlighting the prognostic value of echocardiography in this population. Further data are necessary in a larger, multicenter cohort to allow more precise risk stratification.

Utilisation of early intervention services in infants with congenital heart disease following open-heart surgery.

OBJECTIVE: To examine the use of early intervention services in infants with CHD after open-heart surgery and identify factors associated with receipt of services. STUDY DESIGN: Surveys were administered to caregivers of infants who underwent open-heart surgery before 1 year of age at a single institution between July, 2017 and July, 2018. Information regarding the infant's use of early intervention services and the caregiver's experience with the programme was obtained. Clinical data were retrieved from the medical record review. Logistic regression identified factors associated with receipt of services. RESULTS: The study included 158 eligible infants. Ninety-eight caregivers (62%) completed the surveys. Of those surveyed, 53.1% of infants were currently or previously enrolled in early intervention services. Infants most frequently received physical therapy (76.9%). The majority of caregivers found services to be moderately/very helpful (92.3%) and sufficient for their child (76.9%). In the univariate analysis, single-ventricle disease, known syndrome/genetic abnormality, extracardiac anomaly, and longer intensive care and hospital length of stay were associated with receipt of services. Single-ventricle disease (p = 0.004) and known syndrome/genetic abnormality (p < 0.0001) remained independently associated with receipt of services in the multivariable analysis. CONCLUSION: Amongst infants at risk for neurodevelopmental deficits, approximately half received services after open-heart surgery. Caregivers expressed satisfaction with the programme. While infants with single-ventricle disease and a known syndrome/genetic abnormality were more likely to receive early intervention services, many at-risk infants with CHD failed to receive services. Further research is needed to identify barriers to early intervention services and promote developmental outcomes.

Early and Midterm Outcomes in High-risk Single-ventricle Patients: Hybrid Vs Norwood Palliation.

BACKGROUND: Although overall outcomes have improved for single-ventricle patients, substantial morbidity and mortality remain for certain high-risk groups. The hybrid stage I procedure is an alternative to the Norwood operation for stage I palliation, but it remains unclear whether it is associated with improved outcomes in high-risk patients. METHODS: This single-center, nested, case-control study included high-risk patients with a systemic right ventricle who underwent hybrid stage I or Norwood palliation from January 2000 to December 2016. High-risk features included prematurity < 34 weeks, birth weight < 2.5 kg, restrictive/intact atrial septum, at least moderate atrioventricular valve regurgitation or right ventricular dysfunction, genetic or extracardiac anomalies, or left ventricular sinusoids. Patients were matched by presence of genetic anomaly, restrictive/intact atrial septum, and prematurity/weight < 2 kg. Early and midterm outcomes were compared in the matched hybrid vs Norwood groups. RESULTS: The study included 96 patients (35 hybrid, 61 Norwood). Despite improved 30-day survival in hybrid patients (91% vs 66%, P < .01), 1-year survival was similar between the hybrid and Norwood groups (46% vs 48%, P = .9). No hybrid patients required dialysis or extracorporeal membrane oxygenation after stage I palliation as compared with 19% and 22% of Norwood patients, respectively (both P < .01). Hybrid patients, however, required more unplanned reinterventions (43% vs 21%, P = .02). CONCLUSIONS: There remains significant morbidity and mortality among high-risk single-ventricle infants. Despite an early survival benefit, hybrid stage I palliation has not been associated with improved midterm outcomes at our center.

Differential Myocardial Mechanics in Volume and Pressure Loaded Right Ventricles Demonstrated by Cardiac Magnetic Resonance.

In patients with D-looped transposition of the great arteries (D-TGA) status post atrial switch operation, the systemic right ventricle (RV) shifts to predominantly circumferential (CS) rather than longitudinal strain (LS), which may represent adaptation or dysfunction. We aimed to evaluate myocardial mechanics in pressure loaded, volume-loaded, and normal RVs by cardiac magnetic resonance (CMR). Patients with D-TGA post atrial switch operation with CMR from 2008 to 2015 were matched 1:1 for age and RV ejection fraction (EF) with repaired tetralogy of Fallot (TOF) patients (volume-loaded RVs), and 1:1 for age with control patients. RV free wall LS and CS were measured using feature tracking software (TomTec, Unterscleissheim, Germany). A total of 32 D-TGA (median age 32 years, 56% male), 32 TOF, and 32 control patients were included. D-TGA patients had less dilatation than TOF patients (125 ± 35 ml/m2 vs. 149 ± 44 ml/m2, p = 0.02) and lower RVEF than controls (42.9 ± 7.7% vs. 56.3 ± 5.6%, p < 0.0001). RV LS was similar in D-TGA and TOF ( - 13.2 ± 4.5% vs. - 14.5 ± 5.9%, p = 0.32), both decreased compared to controls. However, CS in D-TGA was higher than controls ( - 14.1 ± 4.1% vs. - 11.4 ± 4.4%, p = 0.01), with a higher CS:LS ratio (1.2 ± 0.7 vs. 0.6 ± 0.3, p < 0.0001), while CS in TOF and controls did not differ. RVEF in D-TGA correlated closely with CS (r = - 0.85, p < 0.0001) but not LS (r = 0.10, p = 0.58). I n conclusion, CMR can differentiate strain patterns in pressure- and volume-loaded RVs, with decreased LS in both conditions, while systemic RVs compensate with supra-normal CS. CS may be a more clinically relevant measure of RV function in this population.

Analysis of Inflammatory Cytokines in Postoperative Fontan Pleural Drainage.

Prolonged pleural drainage is a common complication in patients after Fontan palliation and is associated with short- and long- term morbidities. Among many potential etiologies, prolonged drainage has an inflammatory component, but there are no descriptions of cytokines in Fontan pleural drainage to date. This study aimed to examine the inflammatory make-up of Fontan pleural drainage. This prospective age-range-matched cohort study recruited 25 patients undergoing Fontan procedure and 15 bi-ventricular patients undergoing cardiopulmonary bypass (CPB). Chest tube samples were taken on postoperative day (POD) 1-4, 7, and 10. Cytokines were measured using Bio-Plex Assays. Univariate comparisons were made in patient characteristics and cytokine levels. Median age was 3.7 y (IQR 2.8-3.9) for controls and 2.5 y (IQR 2.1-2.9) in Fontan patients (p = 0.02). Median drainage duration and daily volume was higher in Fontan patients (both p < 0.001). Inflammatory cytokines (IL-17A, IFN-y, MIP-1ß, and TNF-α) were higher in Fontan patients than controls (all p < 0.02). There was an increase in pro-inflammatory cytokines (IL-8, MIP-1ß, and TNF-α) from POD1 to the last chest tube day (LCD) in Fontan patients (all p < 0.0001) and a decrease in the anti-inflammatory cytokine IL-10 (p = 0.001). There was no difference in cytokine concentration from POD1 to LCD among controls. There was a significant association with the cytokine concentration of TNF-α on POD1 and duration of chest tube drainage (p < 0.05). Inflammatory cytokine levels in the pleural fluid of Fontan patients are higher compared to bi-ventricular controls and rise over time where controls do not. This suggests ongoing localized inflammation that is not a result of CPB alone and may be an important contributor to pleural drainage in patients after the Fontan procedure.

Routine Septal Myectomy During Subaortic Stenosis Membrane Resection: Effect on Recurrence Rates.

Recurrence of subaortic stenosis (SubAS) is up to ~ 19% following resection. Historically, treatment has consisted of membrane resection alone. This study investigated the effect of routine septal myectomy in addition to membrane resection. A single-center retrospective review was performed in all patients < 18 years of age undergoing membrane resection with septal myectomy for SubAS from 2003 to 2013. Demographic, perioperative, and follow-up data were collected. Freedom from reoperation and risk factors for reoperation were determined. 107 patients (median age 4.8 years) were included. There was one in-hospital death, five patients (5%) requiring pacemaker, and no iatrogenic ventricular septal defects. Follow-up was 80% complete and median follow-up was 4.9 years (range 0.5-12 years). Fourteen (16%) subjects required reoperation. Freedom from reoperation was 98% at 1 year, 86% at 5 years, and 69% at 10 years (Fig. 1). There was no difference in decrease of peak gradient between subjects who did and did not require reoperation (- 47 vs. - 40 mmHg; p = 0.59). In univariate analysis, chromosomal anomaly (hazard ratio [HR] 5.0, p = 0.02), smaller body surface area (HR 0.1, p = 0.03), and younger age at surgery (HR 0.7, p = 0.01) were significantly associated with reoperation. The routine use of myectomy with membrane excision did not result in a lower rate of reoperation or higher rates of complications compared to historical controls. Younger age, smaller size, and chromosomal anomaly were associated with increased risk for reoperation. Patients with these risk factors may benefit from more intensive long-term follow-up.

Similar Interstage Outcomes for Single Ventricle Infants Palliated With an Aortopulmonary Shunt Compared to the Norwood Procedure.

BACKGROUND: Interstage outcomes for single ventricle infants following Norwood operation have been well studied, showing significant mortality. Other single ventricle infants require only an aortopulmonary shunt. The aim of the study was to describe the interstage outcomes of this group compared to Norwood patients and identify risk factors for mortality. METHODS: A single-center retrospective cohort review was performed in patients who underwent a Norwood operation (Norwood) or aortopulmonary shunt (Shunt) during 2000 to 2011 and survived to discharge. Hybrid or pulmonary artery banding patients were excluded. Univariate comparison was made between Norwood and Shunt patients as well as a Shunt subgroup analysis. RESULTS: A total of 486 patients (368 Norwood and 118 Shunt) were included. Norwood and Shunt patients were similar in terms of preterm birth, surgery weight, and stage 1 complications. Shunt patients were more likely to be female, have an extracardiac or genetic anomaly, and have a shorter hospital length of stay compared to the Norwood patients (all P < .0001). No significant difference in interstage mortality was seen between the Shunt and Norwood patients (6.8% vs 11.1%, respectively; P = .17). Stage 2 mortality was also similar (Shunt 4.6% vs Norwood 7.8%; P = .25). In the Shunt patients, those who died during interstage weighed less at surgery (2.7 [0.7] kg vs 3.3 [0.7] kg, P = .03) and were more likely to have arrhythmias (50% vs 12%, P = .01), compared to survivors. CONCLUSIONS: Shunt patients have an interstage mortality that is not significantly less than Norwood patients. Lower weight at surgery and arrhythmias are risk factors for interstage death in Shunt patients.

Single Ventricle and Total Anomalous Pulmonary Venous Connection: Implications of Prenatal Diagnosis.

BACKGROUND: Single ventricle (SV) patients with total anomalous pulmonary venous connection (TAPVC) are at high risk. Given the limited published data available, we examined outcomes and the implications of a prenatal diagnosis of SV/TAPVC. METHODS: A single-center, retrospective review was performed in neonates with SV/TAPVC from 1998 to 2014, identified through institutional databases. Patient demographic, perioperative, and follow-up data were collected. RESULTS: Thirty-four eligible infants with SV/TAPVC were identified (mean birth weight: 3.0 kg). The TAPVC types were supracardiac (59%), infracardiac (21%), mixed (12%), and cardiac (9%). Heterotaxy syndrome was present in 25 (74%) infants. A prenatal diagnosis of SV was made in 26 (76%) infants, with TAPVC identified in 12 (35%). Seventeen (50%) had obstructed TAPVC within the first 48 hours of life; 7 of these patients had obstructed TAPVC identified prenatally. There were two preoperative deaths. Overall survival for the cohort was 65% at 1 year and 50% at 3 years. Survival in the obstructed group was significantly worse compared to the unobstructed group (47% vs 81% at 1 year; 27% vs 73% at 3 years, P = .01). Obstructed TAPVC and a prenatal prediction of obstructed TAPVC were significantly associated with postoperative mortality ( P = .01 and .03, respectively). CONCLUSIONS: Patients with SV/TAPVC remain a high-risk group, with obstructed TAPVC a significant risk factor for mortality. Prenatal diagnosis of TAPVC in SV patients is challenging, but given those with obstructed TAPVC are especially at high risk, improved prenatal diagnostic techniques in this group may enhance counseling/delivery planning.

Prevalence and risk factors associated with non-attendance in neurodevelopmental follow-up clinic among infants with CHD.

BACKGROUND: Neurodevelopmental impairment is increasingly recognised as a potentially disabling outcome of CHD and formal evaluation is recommended for high-risk patients. However, data are lacking regarding the proportion of eligible children who actually receive neurodevelopmental evaluation, and barriers to follow-up are unclear. We examined the prevalence and risk factors associated with failure to attend neurodevelopmental follow-up clinic after infant cardiac surgery. METHODS: Survivors of infant (<1 year) cardiac surgery at our institution (4/2011-3/2014) were included. Socio-demographic and clinical characteristics were evaluated in neurodevelopmental clinic attendees and non-attendees in univariate and multivariable analyses. RESULTS: A total of 552 patients were included; median age at surgery was 2.4 months, 15% were premature, and 80% had moderate-severe CHD. Only 17% returned for neurodevelopmental evaluation, with a median age of 12.4 months. In univariate analysis, non-attendees were older at surgery, had lower surgical complexity, fewer non-cardiac anomalies, shorter hospital stay, and lived farther from the surgical center. Non-attendee families had lower income, and fewer were college graduates or had private insurance. In multivariable analysis, lack of private insurance remained independently associated with non-attendance (adjusted odds ratio 1.85, p=0.01), with a trend towards significance for distance from surgical center (adjusted odds ratio 2.86, p=0.054 for ⩾200 miles). CONCLUSIONS: The majority of infants with CHD at high risk for neurodevelopmental dysfunction evaluated in this study are not receiving important neurodevelopmental evaluation. Efforts to remove financial/insurance barriers, increase access to neurodevelopmental clinics, and better delineate other barriers to receipt of neurodevelopmental evaluation are needed.

A randomised trial of early palliative care for maternal stress in infants prenatally diagnosed with single-ventricle heart disease.

Children with single-ventricle disease experience high mortality and complex care. In other life-limiting childhood illnesses, paediatric palliative care may mitigate maternal stress. We hypothesised that early palliative care in the single-ventricle population may have the same benefit for mothers. In this pilot randomised trial of early palliative care, mothers of infants with prenatal single-ventricle diagnoses completed surveys measuring depression, anxiety, coping, and quality of life at a prenatal visit and neonatal discharge. Infants were randomised to receive early palliative care - structured evaluation, psychosocial/spiritual, and communication support before surgery - or standard care. Among 56 eligible mothers, 40 enrolled and completed baseline surveys; 38 neonates were randomised, 18 early palliative care and 20 standard care; and 34 postnatal surveys were completed. Baseline Beck Depression Inventory-II and State-Trait Anxiety Index scores exceeded normal pregnant sample scores (mean 13.76±8.46 versus 7.0±5.0 and 46.34±12.59 versus 29.8±6.35, respectively; p=0.0001); there were no significant differences between study groups. The early palliative care group had a decrease in prenatal to postnatal State-Trait Anxiety Index scores (-7.6 versus 0.3 in standard care, p=0.02), higher postnatal Brief Cope Inventory positive reframing scores (p=0.03), and a positive change in PedsQL Family Impact Module communication and family relationships scores (effect size 0.46 and 0.41, respectively). In conclusion, these data show that mothers of infants with single-ventricle disease experience significant depression and anxiety prenatally. Early palliative care resulted in decreased maternal anxiety, improved maternal positive reframing, and improved communication and family relationships.