Search

We aim to identify the incidence and timing of dysfunction and failure of stented bioprosthetic valves in the pulmonary position in congenital heart disease patients.A total of 482 congenital heart disease patients underwent 484 stented bioprosthetic pulmonary valve implantations between 2008 and 2018. There were 164 porcine valves (Porcine) and 320 bovine pericardial valves (Pericardial) implanted. Primary endpoints were survival, valve dysfunction, and valve failure.Pericardial valves were implanted in older patients (22.0, interquartile range [IQR] 14-33 vs 16.0, IQR 11-23 years, P0.001). Five-year survival (96.7% vs 97.9%) for the Pericardial and Porcine groups, respectively, were similar, P0.05. Forty-six (34%) Porcine and 75 (27%) Pericardial group patients met criteria for valve dysfunction at a median echocardiographic follow-up time of 7.43 years (IQR 4.1-9.5 years) and 3.26 years (IQR 1.7-4.7 years), respectively. More Pericardial group patients suffered from at least mild late PR while late median peak gradient was higher in the Porcine group, P.001 for both. Risk factors for valve dysfunction included decreasing patient age for the entire cohort (hazard ratio [HR] 1.02, 95% confidence interval [CI] 1.00-1.04, P = .015) and lack of anticoagulation at discharge for the Porcine group (HR 3.06, 95% CI 1.03-9.10, P = .044) but not the Pericardial group. Five-year cumulative incidence of dysfunction was 39% for the Pericardial group and 17% for the Porcine group.Porcine stented and bovine pericardial stented valves can be implanted in the pulmonary position in all age groups safely. However, despite similar rates of valve failure, bovine pericardial stented valves have a higher incidence of valve dysfunction at mid-term follow-up.

Surgery for heart defects in children with trisomy 13 or 18 is controversial. We analyzed our 20-year experience.Since 2002, we performed 21 operations in 19 children with trisomy 13 (n = 8) or trisomy 18 (n = 11). Age at operation was 4 days to 12 years (median, 154 days). Principal diagnosis was ventricular septal defect in 10 patients, tetralogy of Fallot in 7 patients, arch hypoplasia in 1 patient, and patent ductus arteriosus in 1 patient.The initial operation was ventricular septal defect closure in 9 patients, tetralogy of Fallot repair in 7 patients, pulmonary artery banding in 1 patient, patent ductus arteriosus ligation in 1 patient, and aortic arch/coarctation repair in 1 patient. There were no operative or hospital deaths. Median postoperative intensive care and hospital stays were 189 hours (interquartile range, 70-548) and 14 days (interquartile range, 8.0-37.0), respectively, compared with median hospital stays in our center for ventricular septal defect repair of 4.0 days and tetralogy of Fallot repair of 5.0 days. On median follow-up of 17.4 months (interquartile range, 6.0-68), 1 patient was lost to follow-up after 5 months. Two patients had reoperation without mortality. There have been 5 late deaths (4 with trisomy 18, 1 with trisomy 13) predominately due to respiratory failure from 4 months to 9.4 years postoperatively. Five-year survival was 66.6% compared with 24% in a group of unoperated patients with trisomy 13 or 18.Cardiac operation with an emphasis on complete repair can be performed safely in carefully selected children with trisomy 13 or trisomy 18. Hospital resource use measured by postoperative intensive care and hospital stays is considerably greater compared with nontrisomy 13 and 18.

Background: There is a paucity of information reported regarding the use of milrinone in patients with hypoplastic left heart syndrome prior to the Norwood procedure. At our institution, milrinone is initiated in the pre-operative setting when over-circulation and elevated serum lactate levels develop. We aimed to review the responses associated with the administration of milrinone in the pre-operative hypoplastic left heart syndrome patient. Second, we compared patients who received high- versus low-dose milrinone prior to Norwood procedure. Methods: Single-centre retrospective study of patients diagnosed with hypoplastic left heart syndrome between January 2000 and December 2019 who underwent Norwood procedure. Patient characteristics and outcomes were compared. Results: During the study period, 375 patients were identified; 79 (21%) received milrinone prior to the Norwood procedure with median lactate 2.55 mmol/l, and SpO2 93%. Patients who received milrinone were older at the time of Norwood procedure (6 vs. 5 days) and were more likely to be intubated and sedated. In a subset analysis stratifying patients to low- versus high-dose milrinone, median lactate decreased from time of initiation (2.39 vs 2.75 to 1.6 vs 1.8 mmol/l) at 12 hours post-initiation, respectively. Repeated measures analysis showed a significant decrease in lactate levels by 4 hours following initiation of milrinone, that persisted over time, with no significant difference in mean arterial pressure. Conclusions: The use of milrinone in the pre-operative over-circulated hypoplastic left heart syndrome patient is well tolerated, is associated with decreased lactate levels, and was not associated with significant hypotension or worsening of excess pulmonary blood flow.

The Society of Thoracic Surgeons Congenital Heart Surgery Database is a comprehensive clinical outcomes registry that captures almost all pediatric cardiac surgical operations in the United States. It is the platform for all activities of The Society of Thoracic Surgeons related to the analysis of outcomes and improvement of quality in this subspecialty. This report summarizes current aggregate national outcomes in congenital and pediatric cardiac surgery performed between July 1, 2016, and June 30, 2020. The reported data on aggregate national outcomes are exemplified by an analysis of 10 prespecified benchmark operation groups performed. This report further reviews related activities in the areas of data collection and analysis, quality measurement, performance improvement, and research.

Patent ductus arteriosus (PDA) stenting is an accepted method for securing pulmonary blood flow in cyanotic neonates. In neonates with pulmonary atresia and single source ductal-dependent pulmonary blood flow (SSPBF), PDA stenting remains controversial. We sought to evaluate outcomes in neonates with SSPBF, comparing PDA stenting and surgical Blalock–Taussig shunt (BTS). Neonates with SSPBF who underwent PDA stenting or BTS at the four centers of the Congenital Catheterization Research Collaborative from January 2008 to December 2015 were retrospectively reviewed. Reintervention on the BTS or PDA stent prior to planned surgical repair served as the primary endpoint. Additional analyses of peri-procedural complications, interventions, and pulmonary artery growth were performed. A propensity score was utilized to adjust for differences in factors. Thirty-five patients with PDA stents and 156 patients with BTS were included. The cohorts had similar baseline characteristics, procedural complications, and mortality. Interstage reintervention rates were higher in the PDA stent cohort (48.6% vs. 15.4%, p

Prematurity is a common risk factor in children, affecting approximately 10% of live births, globally. It is more common in children with critical congenital heart disease (CCHD) and carries important implications in this group of patients. While outcomes have been improving over the years, even late preterm birth is associated with worse outcomes in children born with critical congenital heart disease compared to those without. Infants with both prematurity and CCHD are at particularly high risk for important comorbidities, including: necrotizing enterocolitis, intraventricular hemorrhage, white matter injury, neurodevelopmental anomalies and retinopathy of prematurity. Lesion-specific intensive care management of these infants, interventional and peri-operative management specifically tailored to their needs, and multidisciplinary care all have the potential to improve outcomes in this challenging group.

Background:Feeding difficulty is a known complication of congenital heart surgery. Despite this, there is a relative sparsity in the available data regarding risk factors, incidence, associated symptoms, and outcomes.Methods:In this retrospective chart review, patients aged 0–18 years who underwent congenital heart surgery at a single institution between January and December, 2017 were reviewed. Patients with feeding difficulties before surgery, multiple surgeries, and potentially abnormal recurrent laryngeal nerve anatomy were excluded. Data collected included patient demographics, feeding outcomes, post-operative symptoms, flexible nasolaryngoscopy findings, and rates of readmission within a 1-year follow-up period. Multivariable regression analyses were performed to evaluate the risk of an alternative feeding plan at discharge and length of stay.Results:Three-hundred and twenty-six patients met the inclusion criteria for this study. Seventy-two (22.09%) were discharged with a feeding tube and 70 (97.22%) of this subgroup were younger than 12 months at the time of surgery. Variables that increased the risk of being discharged with a feeding tube included patient age, The Society of Thoracic Surgeons–European Association for Cardio-Thoracic Surgery score, procedure group, aspiration, and reflux. Speech-language pathology was the most frequently utilised consulting service for patients discharged with feeding tubes (90.28%) while other services were not frequently consulted. The median length of stay was increased from 4 to 10 days for patients who required an enteral feeding tube at discharge.Discussion:Multidisciplinary management protocol and interventions should be developed and standardised to improve feeding outcomes following congenital heart surgery.

Background:Following cardiac surgery, infants often remain endotracheally intubated upon arrival to the cardiac ICU. High-flow nasal cannula and non-invasive positive pressure ventilation are used to support patients following extubation. There are limited data on the superiority of either mode to prevent extubation failure.Methods:We conducted a single-centre retrospective study for infants (Results:There were 424 patients who met inclusion criteria, 320 (75%) were extubated to high-flow nasal cannula, 104 (25%) to non-invasive positive pressure ventilation, and 64 patients (15%) failed extubation. The high-flow nasal cannula group had lower rates of extubation failure (11%, versus 29%, p = 0.001). Infants failing extubation were younger and had higher STAT score (p < 0.05). Compared to high-flow nasal cannula, non-invasive positive pressure ventilation patients were at 3.30 times higher odds of failing extubation after adjusting for patient factors (p < 0.0001).Conclusions:Extubation failure after cardiac surgery occurs in smaller, younger infants, and those with higher risk surgical procedures. Patients extubated to non-invasive positive pressure ventilation had 3.30 higher odds to fail extubation than patients extubated to high-flow nasal cannula. The optimal mode of respiratory support in this patient population is unknown.

The optimal timing for neonatal cardiac surgery is a potentially modifiable factor that may affect outcomes. We studied the relationship between age at surgery (AAS) and outcomes across multiple hospitals, focusing on neonatal operations where timing appears is not emergency.We studied neonates ≥37 weeks' gestation and ≥2.5 kg admitted to a treating hospital on or before day of life 2 undergoing selected index cardiac operations. The impact of AAS on outcomes was evaluated across the entire cohort and a standard risk subgroup (ie, free of preoperative mechanical ventilation, mechanical circulatory support, or other organ failure). Outcomes included mortality, major morbidity (ie, cardiac arrest, mechanical circulatory support, unplanned cardiac reintervention, or neurologic complication), and postoperative cardiac intensive care unit and hospital length of stay. Post hoc analyses focused on operations undertaken between day of life 2 and 7.We studied 2536 neonates from 47 hospitals. AAS from day of life 2 through 7 was not associated with risk adjusted mortality or major morbidity among the entire cohort and the standard risk subgroup. Older AAS, although associated with modest increases in postoperative cardiac intensive care unit and hospital length of stay in the entire cohort, was not associated with hospital length of stay in the standard risk subgroup.Among select nonemergency neonatal cardiac operations, AAS between day of life 2 and 7 was not found to be associated with risk adjusted mortality or major morbidity. Although delays in surgical timing may modestly increase preoperative resource use, studies of AAS and outcomes not evident at the time of discharge are needed.

Background: Fetal diagnosis of coarctation of aorta is challenging, with limited data examining prenatal detection rates in patients requiring simple vs. extensive arch reconstruction. This study aims to evaluate prenatal detection rate s and fetal cardiac morphometric differences in neonates requiring simple vs. complex coarctation repair. Methods: Pre- and postnatal records of neonates with isolated coarctation repair from the Society of Thoracic Surgeons database from Jan 2010 - Dec 2020 at our center were reviewed. Patients were classified as requiring discrete (Group 1) or extended arch repair (Group 2). Fetal echocardiograms were measured by a single observer blinded to repair type. Results: Of 228 neonates requiring repair, 67 (29%) had a prenatal diagnosis. The proportion with prenatal diagnosis was not different in group 1 (28%) vs. group 2 (32%) (χ2 = 0.45, p = 0.5). At the first fetal echo (median gestational age (GA) of 21.9 wks in group 1 vs 23.6 wks in group 2, p = 0.2), there were no differences between groups when comparing right ventricular (RV) and left ventricular (LV) dimensions, valvular dimensions, or aortic arch dimensions. At the final fetal echo (median GA of 33.7 wks in both groups, p = 0.6), there were statistical differences between group 1 and 2 in RV geometry with ratio of RV minor to major axis (0.72 vs 0.83, p = 0.03) and ratio of LV to RV minor axis (0.68 vs 0.59, p = 0.02), ascending aorta Z score (-2.6 vs -3.4, p = 0.03), distal arch Z score (-3.9 vs -4.6, p = 0.048), and ratio of isthmus to distal arch dimension (0.88 vs 1.02, p = 0.045) (Figure). Conclusion: The rate of prenatal diagnosis in fetuses requiring neonatal coarctation repair remains low, regardless of ultimate surgical repair type. Fetuses ultimately requiring extensive arch repair have smaller aortic sizes, and greater RV/LV size disproportion in the third trimester. This data may be useful in counseling families with regard to postnatal surgical management in fetuses with suspected coarctation.

We present an adolescent male with a single intracardiac mass and pulmonary emboli, complicated by peripheral venous thrombosis and subsequent development of pulmonary pseudoaneurysms, leading to diagnosis of Hughes-Stovin syndrome. Remission was achieved with cyclophosphamide, corticosteroids, and pseudoaneurysm resection and maintained with infliximab and methotrexate.

Background: Published palliation outcomes of infants with functional single ventricle (SV) and common atrioventricular septal defect (AVSD) are poor due to associated cardiac and extracardiac anomalies and development of atrioventricular valve (AVV) regurgitation. We report current palliation results. Methods: From 2002 to 2012, 80 infants with functional SV with AVSD underwent multistage palliation. Competing-risks analyses modeled events after first-stage surgery and Glenn (death/transplantation vs next palliation surgery) and examined factors associated with survival and AVV intervention. Results: Sixty-eight (80%) patients received neonatal palliation: modified Blalock-Taussig shunt (n = 33, 41%), Norwood (n = 20, 25%), and pulmonary artery band (n = 15, 19%), whereas 12 (15%) received primary Glenn. On competing-risks analysis, one-year following first-stage surgery, 29% of patients had died or received transplantation and 62% had undergone Glenn. Five years following Glenn, 9% of patients had died or received transplantation and 68% had undergone Fontan. Overall eight-year survival was 64% and was lower in patients with genetic syndromes (53% vs 82%), patients requiring concomitant total anomalous pulmonary venous connection repair (53% vs 69%), and those requiring neonatal palliation (48% vs 100%). Factors associated with mortality were unplanned reoperation (hazard ratio [HR]: 3.7 [1.7-8.0], P = .001) and extracorporeal membrane oxygenation use (HR: 7.1 [3.0-16.6], P < .001). Initial AVV regurgitation ≥ moderate was associated with AVV intervention (HR: 6.2 [2.4-16.1], P = .002) with eight-year freedom from death or AVV intervention of 25% in those patients. Conclusions: Patients with SV with AVSD are a distinct group and commonly have associated cardiac and extracardiac malformations that complicate care and affect survival. The development of AVV regurgitation requiring intervention is common but does not affect survival.

Patent ductus arteriosus (PDA) stenting is an accepted method for securing pulmonary blood flow in cyanotic neonates. In neonates with pulmonary atresia and single source ductal-dependent pulmonary blood flow (SSPBF), PDA stenting remains controversial. We sought to evaluate outcomes in neonates with SSPBF, comparing PDA stenting and surgical Blalock-Taussig shunt (BTS). Neonates with SSPBF who underwent PDA stenting or BTS at the four centers of the Congenital Catheterization Research Collaborative from January 2008 to December 2015 were retrospectively reviewed. Reintervention on the BTS or PDA stent prior to planned surgical repair served as the primary endpoint. Additional analyses of peri-procedural complications, interventions, and pulmonary artery growth were performed. A propensity score was utilized to adjust for differences in factors. Thirty-five patients with PDA stents and 156 patients with BTS were included. The cohorts had similar baseline characteristics, procedural complications, and mortality. Interstage reintervention rates were higher in the PDA stent cohort (48.6% vs. 15.4%, p 0.001).

Background/Aims:Registry-based trials have emerged as a potentially cost-saving study methodology. Early estimates of cost savings, however, conflated the benefits associated with registry utilisation and those associated with other aspects of pragmatic trial designs, which might not all be as broadly applicable. In this study, we sought to build a practical tool that investigators could use across disciplines to estimate the ranges of potential cost differences associated with implementing registry-based trials versus standard clinical trials.Methods:We built simulation Markov models to compare unique costs associated with data acquisition, cleaning, and linkage under a registry-based trial design versus a standard clinical trial. We conducted one-way, two-way, and probabilistic sensitivity analyses, varying study characteristics over broad ranges, to determine thresholds at which investigators might optimally select each trial design.Results:Registry-based trials were more cost effective than standard clinical trials 98.6% of the time. Data-related cost savings ranged from $4300 to $600,000 with variation in study characteristics. Cost differences were most reactive to the number of patients in a study, the number of data elements per patient available in a registry, and the speed with which research coordinators could manually abstract data. Registry incorporation resulted in cost savings when as few as 3768 independent data elements were available and when manual data abstraction took as little as 3.4 seconds per data field.Conclusions:Registries offer important resources for investigators. When available, their broad incorporation may help the scientific community reduce the costs of clinical investigation. We offer here a practical tool for investigators to assess potential costs savings.

Objectives This study compares 30-day, 1-year, and 3-year echocardiographic findings and clinical outcomes of transcatheter pulmonary valve-in-valve replacement (TPVR) and repeat surgical pulmonary valve replacement (SPVR). Background In patients with adult congenital heart disease and previous pulmonary valve replacement (PVR) who require redo PVR, it is unclear whether TPVR or repeat SPVR is the preferred strategy. Methods We retrospectively identified 66 patients (TPVR, n = 36; SPVR, n = 30) with bioprosthetic pulmonary valves (PVs) who underwent either TPVR or repeat SPVR at Emory Healthcare from January 2007 to August 2017. Results The TPVR cohort had fewer men and more patients with baseline New York Heart Association (NYHA) functional class III or IV. There was no difference in mortality, cardiovascular readmission, or post-procedural PV reintervention at 30 days, 1 year, or 3 years. Post-procedural echocardiographic findings showed no difference in mean PV gradients between the TPVR and SPVR groups at 30 days, 1 year, or 3 years. In the TPVR cohort, there was less right ventricular dysfunction at 30 days (2.9% vs. 46.7%; p Conclusions In patients with bioprosthetic PV dysfunction who underwent either TPVR or SPVR, there was no difference in mortality, cardiovascular readmission, or repeat PV intervention at 30 days, 1 year, or 3 years. Additionally, TPVR and SPVR had similar intermediate-term PV longevity, with no difference in PV gradients or PVR. The TPVR cohort also had less right ventricular dysfunction at 30 days despite a higher baseline NYHA functional classification. These intermediate-term results suggest that TPVR may be an attractive alternative to SPVR in patients with previous bioprosthetic surgical PVs.

Introduction Vocal fold motion impairment (VFMI) is a known potential complication of congenital heart surgery (CHS). Flexible nasolaryngoscopy (FNL) is the gold standard for evaluation of vocal fold movement but has risks, including epistaxis, desaturation, and changes in heart rate. Laryngeal ultrasound (LUS) has begun to emerge as a diagnostic tool and has been shown to have high accuracy in the evaluation of VFMI. We sought to assess the utility of hand-held LUS as a point-of-care screening tool to assess VFMI in pediatric patients following congenital heart surgery. Methods Using a prospective cohort design, children under 18 years who were undergoing congenital heart surgery at a tertiary care pediatric hospital were enrolled. All patients underwent postoperative LUS and FNL. All studies were reviewed by two otolaryngology reviewers blinded to the clinical diagnosis. Higher quality studies were reviewed by two cardiology reviewers also blinded to the clinical diagnosis. Accuracy and inter-rater reliability were calculated. Results Sixty-two children were screened. Fourteen children with VFMI were identified via FNL. When comparing LUS and FNL, both individual accuracy (90.3% and 75.8%) and interrater agreement (79% overall, 96% for high quality videos) were high for the otolaryngology reviewers. The cardiology reviewers were able to obtain 100% accuracy for high quality videos. Conclusion Handheld LUS has utility as a point-of-care screening tool to assess VFMI. This may have benefit in low-resource settings, for universal screening in cardiac intensive care units, or in settings where otolaryngology consultation may be difficult to obtain.

Background Double inlet left ventricle (DILV) is a heterogeneous single ventricle anomaly in which initial presentation, and consequently, timing and palliation mode vary based on morphology and degree of pulmonary or systemic outflow obstruction. Very few reports, mostly old, focused on palliation outcomes of DILV. We report current-era results and examine whether morphologic and subsequently surgical factors influence survival. Methods Fifty-eight infants with DILV underwent single ventricle palliation. Echocardiographic examination showed pulmonary (n = 29, 50%), systemic outflow tract (n = 11, 19%), and arch (n = 17, 29%) obstruction. Factors associated with death or transplantation were examined. Results Forty-four patients (76%) required neonatal first-stage palliation: modified Blalock-Taussig shunt (n = 15, 26%), Norwood (n = 15, 26%), or pulmonary artery band (n = 14, 24%), whereas 14 (24%) received primary Glenn. There was 1 hospital death (2%) and 2 interstage deaths before Glenn, in addition to 1 late death that was noncardiac. Overall 10-year survival was 94% and was comparable for different palliative surgeries ( p = 0.49). Three patients (6%) underwent heart transplantation after first-stage palliation (n = 1) or after Glenn (n = 2) for ventricular noncompaction (n = 1), ventricular and atrioventricular valve dysfunction (n = 1), and pacemaker-induced cardiomyopathy (n = 1). Overall 10-year freedom from death or transplantation was 87% and was comparable for different palliative surgeries ( p = 0.58). On regression risk analysis, none of the tested morphologic or surgical variables was associated with the risk of death or transplantation. Conclusions Current outcomes of multistage palliation of DILV are relatively good compared with published reports of other single ventricle anomalies. Survival is not greatly affected by cardiac morphology or initial palliative surgery type.

We aim to describe the impact of 22q11.2 deletion syndrome (22q11DS) on clinical characteristics, postoperative course, and early and late outcomes of neonates undergoing surgery for conotruncal anomalies.A retrospective review was performed (2002 to 2012) of 224 neonates who underwent surgery for interrupted aortic arch (n = 67), truncus arteriosus (n = 85), or ductal-dependent pulmonary atresia and ventricular septal defect (n = 72). Patients were divided into three groups: group 1, n = 119, no genetic syndrome; group 2, n = 64, 22q11DS; and group 3, n = 41, other genetic syndrome. Adjusted analysis to compare outcomes was performed.In comparison with group 1, group 2 had longer mechanical ventilation duration (148 versus 102 hours, p = 0.008), intensive care unit stay (268 versus 159 hours, p0.001), and hospital stay (19.3 versus 11.5 days, p 0.001). On adjusted analysis, there was an insignificant increase in unplanned reoperation (odds ratio [OR] 2.4, 95% confidence interval [CI]: 0.7 to 8.4, p = 0.167) but no increased extracorporeal membrane oxygenation use (OR 1.5, 95% CI: 0.3 to 6.1, p = 0.612), hospital mortality (OR 0.6, 95% CI: 0.1 to 3.3, p = 0.570), or decreased late survival (hazard ratio 0.9, 95% CI: 0.4 to 2.1, p = 0.822). In comparison with group 1, group 3 had longer mechanical ventilation duration (190 versus 102 hours, p0.001), intensive care unit stay (236 versus 159 hours, p = 0.007), and hospital stay (21.5 versus 11.5 days, p0.001); and increased unplanned reoperation (OR 3.7, 95% CI: 1.1 to 12.5, p = 0.032), extracorporeal membrane oxygenation use (OR 4.4, 95% CI: 1.1 to 17.6, p = 0.038), hospital mortality (OR 4.2, 95% CI: 1.2 to 14.5, p = 0.021), and diminished late survival (hazard ratio 4.0, 95% CI: 2.1 to 8.1, p0.001).In neonates with conotruncal anomalies, 22q11DS is associated with prolonged recovery and increased resource utilization. However, despite a small increase in unplanned reoperation, there is no significant impact on early or late survival. In comparison, other genetic syndromes are associated with increased unplanned reoperation, extracorporeal membrane oxygenation use, hospital mortality, and diminished late survival. These findings are important for family counseling and risk stratification.

Background The two most common surgical strategies for the treatment of neonates born with single-ventricle anomalies associated with aortic arch obstruction are the Norwood operation and pulmonary artery banding plus coarctation repair (PAB+COA). We reviewed characteristics and outcomes of neonates who underwent those two surgical strategies at our institution. Methods Between 2002 and 2012, 94 neonates with a single ventricle and aortic arch obstruction (excluding hypoplastic left heart syndrome) underwent Norwood (n = 65) or PAB+COA (n = 29). Outcomes were parametrically modeled, and risk factors associated with early and late death were analyzed. Results Competing-risks analysis showed that, at 2 years after the operation, 24% of patients had died or received transplantation and 75% had undergone a Glenn shunt. At 5 years after the Glenn shunt, 10% of patients had died or received transplantation, 62% had undergone Fontan, and 28% were alive awaiting Fontan. Overall 8-year survival was 70%. Outcomes after Norwood included extracorporeal membrane oxygenation use in 9 (14%), unplanned reoperation in 13 (20%), hospital death in 10 (15%), and interstage death in 8 (12%), with 8-year survival of 66%. Outcomes after PAB+COA included extracorporeal membrane oxygenation use in 1 (3%), unplanned reoperation in 9 (30%), hospital death in 1 (3%), and interstage death in 3 (10%), with 8-year survival of 76%. There was an association trend between underlying anatomy and survival (hazard ratio [HR], 2.1; 95% confidence interval [CI], 0.9 to 4.7; p = 0.087). On multivariable analysis, factors associated with death were extracorporeal membrane oxygenation use (HR, 5.5; 95% CI, 1.9 to 15.9; p = 0.002), genetic syndromes/extracardiac anomalies (HR, 3.5; 95% CI, 1.5 to 8.2; p = 0.003), and weight of 2.5 kg or less (HR, 3.0; 95% CI, 1.3 to 7.2; p = 0.012). Conclusions Anatomic and patient characteristics influence palliation outcomes in neonates born with single-ventricle anomalies associated with aortic arch obstruction. Although the Norwood operation is applicable in most of these patients, the PAB+COA strategy is a valid alternative in well-selected patients.

Importance Vocal fold motion impairment (VFMI) is a known risk factor following congenital heart surgery (CHS). The impact of this diagnosis on utilization and outcomes is unknown. Objective To evaluate the cost, postprocedure length of stay (PPLOS), and outcomes for neonates with VFMI after CHS. Design, Setting, and Participants A cross-sectional analysis of the 2012 Kids’ Inpatient Database (KID) of neonates who underwent CHS was carried out. The KID is an administrative data set of patients, aged 20 years or younger, and contains data on more than 10 million hospitalizations from 44 states. The KID is limited to inpatient hospitalization and contains discharge summary level of data. Patients were limited to those who were born during the hospitalization and those who were aged 28 days or younger at the time of admission for CHS. A weighted total of 4139 neonates who underwent CHS were identified, of which 3725 survived. The proportion of neonates diagnosed with VFMI was 264 (6.92%) of 3725. Exposures Congenital heart surgery. Main Outcomes and Measures Cost of inpatient hospital stay, postprocedure length of stay, odds of pneumonia, gastrostomy tube placement, and tracheostomy tube placement. Risk-adjusted generalized linear models examined differences in cost and PPLOS between neonates who underwent CHS and were diagnosed with VFMI and those who were not. Risk-adjusted logistic regression compared the odds of selected outcomes (gastrostomy, tracheostomy, pneumonia). Models were weighted to provide national estimates. Results Of 3725 neonates (aged 0-28 days), 2203 (59.1%) were male and 1517 (40.7%) were female. Neonates diagnosed with VFMI had significantly higher total cost by $34 000 (95% CI, 2200-65 000) and PPLOS by 9.1 days (95% CI, 4.6-13.7) compared with those who did not. When PPLOS was included as a covariate in the model for cost, presence of VFMI was no longer significant. There were no differences in odds of pneumonia, gastrostomy, or tracheostomy. Conclusions and Relevance Vocal fold motion impairment after CHS was associated with significant increases in cost owing to increased PPLOS. These findings provide a foundation to further investigate standardized screening for VFMI following CHS; early identification and treatment may decrease cost and PPLOS.

We examined the effect of genetic syndromes and extracardiac (GS/EC) anomalies on single-ventricle (SV) palliation with focus on hospital and interstage death and progression toward subsequent palliation stages.First-stage palliation was performed in 530 neonates with SV: Norwood in 284 (53%), shunt in 173 (33%), and band in 73 (14%). Outcomes were compared between those with GS/EC anomalies (121 [23%]) and without GS/EC anomalies (409 [77%]). Regression analyses were adjusted for other risk factors (age, sex, prematurity, weight, SV anomaly, and first-stage palliation operation).GS/EC anomalies varied among SV defects (range, 3% for double-inlet left ventricle to 100% for atrial isomerism). Patients with GS/EC anomalies required significantly longer durations of mechanical ventilation and intensive care unit and hospital stay. Although patients had comparable rates of extracorporeal membrane oxygenation (13% vs 11%, p = 0.552) and unplanned reoperation (16% vs 11%, p = 0.189), hospital mortality was higher in patients with GS/EC anomalies (24% vs 12%, p = 0.0008). After discharge, patients with GS/EC anomalies had higher interstage death, with lower progression to Glenn (60% vs 77%, p = 0.002) and lower 10-year survival (56% vs 76%, p0.001). After adjustment for other risk factors, GS/EC anomalies significantly affected survival in almost all subgroups of patients.The presence of GS/EC anomalies varies among SV anomalies and is associated with additional risk factors such as prematurity and low weight. After adjusting for other risk factors, GS/EC anomalies are associated with prolonged recovery after first-stage palliation and increased hospital and interstage death, with subsequently fewer patients progressing toward Glenn shunt. The increased death risk in those patients is highest in the first 6 months and persists for 2 to 3 years after first-stage palliation, suggesting the need for more vigilant monitoring and outpatient care in these high-risk patients.

BackgroundPatients with total anomalous pulmonary venous connection can be problematic, particularly those with mixed-type pathology. We aimed to describe a cohort of patients with mixed-type anomalous drainage, highlighting the treatment challenges, and identifying risk factors for poor outcome.MethodsWe reviewed the clinical records of patients who underwent repair for mixed-type total anomalous pulmonary venous connection between 1986 and 2015.ResultsA total of 19 patients were identified. The median age and weight of patients at surgery were 18 days (with a range from 1 to 185) and 3.4 kg (with a range from 1.9 to 6.5), respectively. Venous anatomy included a combination of duplicate supracardiac (four), supracardiac and cardiac (11), and supracardiac and infracardiac (four) drainage. Out of 19 patients, six (32%) died within 30 days or the initial hospital stay; two additional patients died from progressive pulmonary vein stenosis at 72 and 201 days, respectively, resulting in 42% mortality within the 1st year. Follow-up data were available for 8/11 long-term survivors. The median follow-up period was 7.3 years (with a range from 1.8 to 15.7). Only one patient underwent re-intervention for recurrent pulmonary vein stenosis. For surgical mortality, no statistically significant risk factors were identified, although the risk trended to be higher (p⩽0.1) with lower age and weight, an infracardiac component, and prolonged cardiopulmonary bypass. For 1-year mortality, the risk became significant (p⩽0.05) with a lower weight (p=0.01), an infracardiac component (p=0.03), and prolonged cardiopulmonary bypass (p=0.04).ConclusionThe surgical and 1-year mortality in patients with mixed-type total anomalous pulmonary venous connection is high. On the other hand, among patients who survive past the 1st year, most have good outcomes without subsequent sequelae.

The Emory Adult Congenital Heart (Emory University, Atlanta, GA) program was founded in 2001. In 2004, the surgical component transitioned from a pediatric facility to an adult facility. The aim of this article is characterize the program as a whole, outline changes in the program, and discuss the challenges of the transition process. Between 2001 and 2015, changes in program structure and personnel were evaluated. There has been significant growth of the program between 2001 and 2015. There are currently 19 half-day clinics per week, with 2,700 clinic visits per year. There are six cardiologists, three congenital cardiac surgeons, two sonographers, one advanced practice provider, and one social worker dedicated to the program. There are Accreditation Council for Graduate Medical Education-accredited adult congenital cardiology and congenital cardiac surgery fellowships. One thousand forty-four operations were performed between 2001 and 2015. There were 828 open-heart operations, of which 581 (70%) were re-operations. Over the study period, the number of yearly operations increased from 30 to 119, and the mean age at surgery increased from 22 to 35 years. Over time, more of the operations were performed at the adult hospital: increasing from 3% in 2001 to 82% in 2015, and more of the operations were performed by congenital cardiac surgeons: 87% (114 of 131) before the 2004 transition to 97% (881 of 913) afterward. The Emory Adult Congenital Heart program has undergone significant growth and change, including transition of the surgical component from the pediatric to the adult facility. While numerous obstacles have been overcome and great progress has been made, additional challenges remain.

There is no previous report comparing clinical or echocardiographic outcomes in patients with a history of prior surgical pulmonary valve replacement (SPVR) who undergo repeat SPVR or transcatheter pulmonary valve replacement (TPVR). We retrospectively identified patients who had previous SPVR and

Background Differences in right ventricle and tricuspid valve structure compared with left ventricle and mitral valve structure make them less equipped to support the systemic circulation long term, with subsequent systemic right ventricle failure. We examined the effect of dominant ventricle morphology on single ventricle palliation outcomes. Methods We grouped 530 neonates who underwent first-stage palliation into two groups based on dominant ventricle morphology: right dominant ventricle (RV group; n = 302, 57%) and left dominant ventricle or functional single ventricle with two well-formed ventricles (LV group; n = 228, 43%). Comparisons of hospital outcomes, interstage mortality, progression to subsequent palliation stages, and late survival was performed, and factors affecting outcomes were examined. Results After first-stage palliation, the RV group and LV group, respectively, had comparable extracorporeal membrane oxygenation requirements (12% versus 11%, p = 0.648), unplanned reoperation (12% versus 13%, p = 0.586), and hospital death (16% versus 13%, p = 0.437). Among hospital survivors, interstage mortality (11% versus 9%, p = 0.509) and progression to Glenn operation (89% versus 84%, p = 0.182) were comparable; however, death after Glenn was higher in the RV group (10%, versus LV group 4%, p = 0.020) with a trend for lower 8-year survival (66% versus 73%, p = 0.081). On multivariable analysis, dominant RV was not associated with mortality (hazard ratio 0.75, 95% confidence interval: 0.6 to 1.0, p = 0.081), whereas factors such as genetic syndromes, weight 2.5 kg or less, underlying cardiac anomaly, and first-stage palliation type affected survival. Conclusions At midterm follow-up, underlying cardiac anomaly and patient characteristics affect single ventricle palliation outcomes more than dominant ventricular morphology. As right ventricle and associated tricuspid valve failure might occur at late stages, the impact of dominant ventricular morphology on long-term outcomes requires further assessment.