Hypoplastic Left Heart Syndrome with Mitral Stenosis and Aortic Atresia-Echocardiographic Findings and Early Outcomes.

BACKGROUND: Mitral stenosis/aortic atresia (MS/AA) has been reported as a high-risk variant of hypoplastic left heart syndrome (HLHS), potentially related to ventriculocoronary connections (VCCs) or endocardial fibroelastosis (EFE) and myocardial hypoperfusion. We aimed to identify echocardiographic and clinical factors associated with early death or transplant in this group. METHODS: Patients with HLHS MS/AA treated at our center between 2000 and 2020 were included. Pre-stage I palliation echocardiograms were reviewed. Certain imaging factors, such as determination of VCC, EFE, and measurement of tricuspid annular plane systolic excursion were measured from retrospective review of preoperative images; others were derived from clinical reports. Groups were compared according to primary outcome of death or transplant prior to stage II palliation. RESULTS: Of 141 patients included, 39 (27.7%) experienced a primary outcome. Ventriculocoronary connections were identified in 103 (73.0%) patients and EFE in 95 (67.4%) patients. Among imaging variables, smaller ascending aorta size (median, 2.2 [interquartile range (IQR) 1.7-2.8] vs 2.6 [2.2-3.4] mm, P = .01) was associated with primary outcome. There was similar frequency of VCC (74.4% vs 72.5%, P = .83), EFE (59.0% vs 72.5%, P = .19), moderate or greater tricuspid regurgitation (5.1% vs 5.9%, P = 1.00), and similar right ventricular systolic function (indexed tricuspid annular plane systolic excursion 32.5 ± 7.3 vs 31.4 ± 7.2 mm/m2, P = .47) in the primary outcome group compared to other patients. Clinical factors associated with primary outcome included lower birth weight (mean, 2.8 ± SD 0.8 vs 3.3 ± 0.5 kg, P = .0003), gestational age <37 weeks (31.6% vs 4.9%, P < .0001), longer cardiopulmonary bypass time (median, 112 [IQR, 93-162] vs 82 [71-119] minutes, P = .001), longer intensive care unit length of stay (median, 19 [IQR, 10-30] vs 10 [7-15] days, P = .001), and extracorporeal membrane oxygenation following stage I palliation (43.6% vs 8.8%, P < .0001). Presence of VCCs and EFE was not associated with death or transplant after controlling for birth weight and era of stage I palliation. CONCLUSIONS: In one of the largest reported single-center cohorts of HLHS MS/AA, there were few pre-stage I palliation imaging characteristics associated with primary outcome. Imaging findings evaluated in this study, including the presence of VCC and/or EFE as determined using highly sensitive echocardiogram criteria, should not preclude intervention, although impact on long-term outcomes requires further evaluation.

Hybrid Interventions in Congenital Heart Disease: A Review of Current Practice and Rationale for Use.

BACKGROUND: Hybrid interventions have become a common option in the management for a variety of patients with congenital heart disease. In this review, we discuss the data that have driven decision making about hybrid interventions to date. METHODS: The existing literature on various hybrid approaches was reviewed and summarized. In addition, the key tenants to creating a successful hybrid program within a congenital heart center are elucidated. RESULTS: Hybrid strategies for single-ventricle patients, pulmonary atresia with intact ventricular septum, branch pulmonary artery stenosis, and muscular ventricular septal defect closure have important benefits and limitations compared with traditional approaches. CONCLUSION: A growing body of evidence supports the use of hybrid interventions in congenital heart disease. But important questions remain regarding improved survival and other long-term outcomes, such as neurocognition, that might impact widespread adoption as a primary treatment strategy.

Association Between Past and Present Operative Mortality in Congenital Heart Surgery: An Analysis of The Society of Thoracic Surgeons Congenital Heart Surgery Database.

BACKGROUND: Mortality after congenital heart surgery is an important metric across benchmarking, quality, and reporting initiatives. All rely on estimates from prior years, and how well these reflect current outcomes is unclear. METHODS: Index operations from The Society of Thoracic Surgeons Congenital Database (2014-2019) were included. Adjusted operative mortality in "past" 4-year and 1-year intervals vs the most recent year ("present") was evaluated using Bayesian hierarchical logistic regression with results presented as odds ratios and 95% credible intervals (CrI). RESULTS: Among 115,699 operations (106 hospitals), overall present observed mortality was 2.5%. Regression to the mean was evident, and individual hospital's present vs past adjusted mortality were only weakly correlated (-0.11 vs past 1-year data, 0.22 vs past 4-year data). A significant relationship was found between past and present mortality only for the group of hospitals in the highest mortality quartile, most prominent for past 4-year data (adjusted odds ratio vs lowest mortality quartile, 2.04; 95% CrI, 1.44-2.80). The proportion of present hospital mortality variation explained by past mortality quartile was 52% (95% CrI, 20%-90%) using past 4-year data and only 27% (95% CrI, 92%-83%) using past 1-year data. Overall 66% of hospitals changed mortality quartiles from past to present (30% by ≥2 quartiles). CONCLUSIONS: Past mortality relates to present primarily for groups of hospitals at the extremes, with past 4-year data more informative than past 1-year data. For individual hospitals, past may differ from present, regression to the mean is common, and many change quartiles. Past mortality should be used thoughtfully and not as the sole factor informing present decision making.

Survival After Surgical Aortic Valve Replacement in Low-Risk Patients: A Contemporary Trial Benchmark.

BACKGROUND: The use of transcatheter aortic valve replacement for severe aortic stenosis in low-risk patients necessitates an evaluation of contemporary long-term, real-world outcomes of similar patients undergoing surgical aortic valve replacement (SAVR) in a national cohort. METHODS: All patients undergoing primary, isolated SAVR in The Society of Thoracic Surgeons (STS) database between 2011 and 2019 were examined. The study population of 42,586 adhered to the inclusion/exclusion criteria of the Placement of Aortic Transcatheter Valves (PARTNER) 3 and Evolut Low Risk randomized trials. Patients were further stratified by STS predicted risk of mortality (PROM), age, and left ventricular ejection fraction. The primary end-point was all-cause National Death Index mortality. Unadjusted survival to 8 years was estimated using the Kaplan-Meier method. RESULTS: Mean age was 74.3 ± 5.7 years and mean STS PROM was 1.9% ± 0.8%. The overall Kaplan-Meier time to event analysis for all-cause mortality at 1, 3, 5, and 8 years was 2.6%, 4.5%, 7.1%, and 12.4%, respectively. In subset analyses, survival was significantly better for (1) lower STS PROM (P < .001), (2) younger vs older age (P < .001), and (3) higher vs lower left ventricular ejection fraction (P < .001). When STS PROM was below 1% or the patient age was below age 75 years, the 8-year survival after SAVR was 95%. CONCLUSIONS: The results of this national study confirm that long-term survival after SAVR remains excellent, at 92.9% at 5 years. These contemporary longitudinal data serve to aid in the balanced interpretation of current and future trials comparing SAVR and transcatheter aortic valve replacement and may assist in the clinical decision-making process for patients of lower surgical risk.

Long-term impact of anatomic subtype in hypoplastic left heart syndrome after Fontan completion.

BACKGROUND: Aortic atresia (AA)/mitral stenosis (MS) is a well-known risk factor for survival after Norwood; however, the effect of anatomical subtypes in those who survive surgical palliation is unknown. METHODS: We performed a retrospective single-center study of patients with classic hypoplastic left heart syndrome (HLHS) who underwent Fontan at our center between August 1989 and July 2017. Clinical outcomes, as well as ventricular and atrioventricular-valve (AVV) function, were determined for each patient, and the effects of HLHS subtype were estimated using multivariable statistical analyses. RESULTS: We included 418 patients with HLHS (AA/mitral atresia [MA] 153, AA/MS 100, aortic stenosis [AS]/MS 154, and AS/MA 11). The median follow-up period was 8.6 (interquartile range, 2.9-15.8) years. Overall transplant-free survival, cumulative incidence of AVV failure, and ventricular failure, which were defined by moderate dysfunction or greater or the necessity of surgical interventions, were 70.1%, 35.9%, and 17.9% at 20 years, respectively. Of the 3 major subtypes, AA/MS was associated with lower survival rate (AA/MA 74.6% vs AS/MS 79.1% vs AA/MS 56.1% at 17 years, P = .04). The subanalysis between AA/MA and AA/MS revealed AA/MS tended to have a greater rate of ventricular failure without a significant difference of AVV failure (AA/MA 11.2% vs AA/MS 26.2% at 17 years, P = .053). CONCLUSIONS: The survival risk of the anatomic subtype AA/MS persisted long term after Fontan completion and was likely due to a greater rate of single ventricle rather than AVV failure. These findings suggest that the abnormal pressure overload condition of the hypoplastic left ventricle created by AA/MS has a detrimental effect on single right ventricle function.

Recommendations for Centers Performing Pediatric Heart Surgery in the United States.

Care and outcomes for the more than 40,000 patients undergoing pediatric and congenital heart surgery in the United States annually are known to vary widely. While consensus recommendations have been published across numerous fields as one mechanism to promote a high level of care delivery across centers, it has been more than two decades since the last pediatric heart surgery recommendations were published in the United States. More recent guidance is lacking, and collaborative efforts involving the many disciplines engaged in caring for these children have not been undertaken to date. The present initiative brings together professional societies spanning numerous care domains and congenital cardiac surgeons, pediatric cardiologists, nursing, and other healthcare professionals from diverse programs around the country to develop consensus recommendations for United States centers. The focus of this initial work is on pediatric heart surgery, and it is recommended that future efforts focus in detail on the adult congenital population. We describe the background, rationale, and methodology related to this collaborative effort, and recommendations put forth for Essential Care Centers (essential services necessary for any program), and Comprehensive Care Centers (services to optimize comprehensive and high-complexity care), encompassing structure, process, and outcome metrics across 14 domains.

Recommendations for centers performing pediatric heart surgery in the United States.

Care and outcomes for the more than 40,000 patients undergoing pediatric and congenital heart surgery in the United States annually are known to vary widely. While consensus recommendations have been published across numerous fields as one mechanism to promote a high level of care delivery across centers, it has been more than two decades since the last pediatric heart surgery recommendations were published in the United States. More recent guidance is lacking, and collaborative efforts involving the many disciplines engaged in caring for these children have not been undertaken to date. The present initiative brings together professional societies spanning numerous care domains and congenital cardiac surgeons, pediatric cardiologists, nursing, and other healthcare professionals from diverse programs around the country to develop consensus recommendations for United States centers. The focus of this initial work is on pediatric heart surgery, and it is recommended that future efforts focus in detail on the adult congenital population. We describe the background, rationale, and methodology related to this collaborative effort, and recommendations put forth for Essential Care Centers (essential services necessary for any program), and Comprehensive Care Centers (services to optimize comprehensive and high-complexity care), encompassing structure, process, and outcome metrics across 14 domains.

Outcomes After Hybrid Palliation for Infants With Critical Left Heart Obstruction.

BACKGROUND: Hybrid palliation (bilateral pulmonary artery banding with or without ductal stenting) is an initial management strategy for infants with critical left heart obstruction and serves as palliation until subsequent operations are pursued. OBJECTIVES: This study sought to determine patient characteristics and factors associated with subsequent outcomes for infants who underwent hybrid palliation. METHODS: From 2005 to 2019, 214 of 1,236 prospectively enrolled infants within the Congenital Heart Surgeons' Society's critical left heart obstruction cohort underwent hybrid palliation across 24 institutions. Multivariable hazard modeling with competing risk methodology was performed to determine risk and factors associated with outcomes of biventricular repair, Fontan procedure, transplantation, or death. RESULTS: Preoperative comorbidities (eg, prematurity, low birth weight, genetic syndrome) were identified in 70% of infants (150 of 214). Median follow-up was 7 years, ranging up to 17 years. Overall 12-year survival was 55%. At 5 years after hybrid palliation, 9% had biventricular repair, 36% had Fontan procedure, 12% had transplantation, 35% died without surgical endpoints, and 8% were alive without an endpoint. Factors associated with transplantation were absence of ductal stent, older age, absent interatrial communication, smaller aortic root size, larger tricuspid valve area z-score, and larger left ventricular volume. Factors associated with death were low birth weight, concomitant genetic syndrome, cardiopulmonary bypass use during hybrid palliation, moderate to severe tricuspid valve regurgitation, and smaller ascending aortic size. CONCLUSIONS: Mortality remains high after hybrid palliation for infants with critical left heart obstruction. Nonetheless, hybrid palliation may facilitate biventricular repair for some infants and for others may serve as stabilization for intended functional univentricular palliation or primary transplantation.

Recommendations for Centers Performing Pediatric Heart Surgery in the United States.

Care and outcomes for the more than 40,000 patients undergoing pediatric and congenital heart surgery in the United States annually are known to vary widely. While consensus recommendations have been published across numerous fields as one mechanism to promote a high level of care delivery across centers, it has been more than two decades since the last pediatric heart surgery recommendations were published in the United States. More recent guidance is lacking, and collaborative efforts involving the many disciplines engaged in caring for these children have not been undertaken to date. The present initiative brings together professional societies spanning numerous care domains and congenital cardiac surgeons, pediatric cardiologists, nursing, and other healthcare professionals from diverse programs around the country to develop consensus recommendations for United States centers. The focus of this initial work is on pediatric heart surgery, and it is recommended that future efforts focus in detail on the adult congenital population. We describe the background, rationale, and methodology related to this collaborative effort, and recommendations put forth for Essential Care Centers (essential services necessary for any program), and Comprehensive Care Centers (services to optimize comprehensive and high-complexity care), encompassing structure, process, and outcome metrics across 14 domains.

Pulmonary Artery Hypoplasia in Neonates With Tetralogy of Fallot.

BACKGROUND: Neonates with symptomatic tetralogy of Fallot (sTOF) with hypoplastic pulmonary arteries (hPA) are considered high risk. Data are needed to inform the impact of hPA on outcomes, and the ideal management strategy. OBJECTIVES: The objectives of this study were to quantify the impact of hPA on outcomes in neonates with sTOF and measure the impact of strategy on pulmonary artery (PA) growth in this population. METHODS: Neonates with sTOF from 2005 to 2017 were reviewed from the Congenital Cardiac Research Collaborative. Criteria for hPA included a unilateral PA z score <-2.0 and contralateral PA z score <0. Primary outcome was mortality. Secondary outcomes included reintervention and PA growth. RESULTS: We included 542 neonates with sTOF, including 188 (35%) with hPA and 354 (65%) with normal PA, with median follow-up of 4.1 years. Median right and left hPA z scores were -2.19 (25th-75th percentile: -2.55 to -1.94) and -2.23 (25th-75th percentile: -2.64 to -1.91), respectively. Staged repair (vs primary TOF repair) was less common in the hPA cohort (36 vs 44%; P = 0.07). Survival was similar between groups (unadjusted P = 0.16; adjusted P = 0.25). Reintervention was more common in the hPA group (HR: 1.28; 95% CI: 1.01-1.63; P = 0.044); there was no difference after definitive repair (HR: 1.21; 95% CI: 0.93-1.58; P = 0.16). PA growth at 1 year was greater in the hPA cohort, particularly for the right PA (P < 0.001). CONCLUSIONS: Despite perception, the presence of hPA in neonates with sTOF conferred no increase in overall hazard of mortality or reintervention after definitive repair. PA growth was superior in the hPA cohort. These findings suggest that the presence of hPA does not adversely impact outcomes in sTOF.

Contemporary Relationship Between Hospital Volume and Outcomes in Congenital Heart Surgery.

BACKGROUND: Studies examining the volume-outcome relationship in congenital heart surgery (CHS) are more than a decade old. Since then, mortality has declined, and case-mix adjustment has evolved. We determined the current relationship between hospital CHS volume and outcomes. METHODS: Patients aged ≤18 years undergoing index operations in The Society of Thoracic Surgeons-Congenital Heart Surgery Database (2017-2020) were included. Associations between annual hospital volume and case-mix-adjusted operative mortality, major complications, failure to rescue (FTR), and postoperative length of stay (PLOS) were assessed using Bayesian hierarchical models, overall, by The Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery (STAT) category, and for the Norwood procedure. RESULTS: Across 101 centers (76,714 index operations), median annual volume was 144 operations/y. Operative mortality was 2.7%. Lower-volume hospitals had higher mortality, with an apparent transition zone at ∼190 operations/y (95% credible interval [CrI], 115-450 operations/y), below which a sustained uptick in the estimated odds of death occurred. Odds of death compared with a 450 operations/y reference were 50 operations/y (odds ratio [OR], 1.84; 95% CrI, 1.41-2.37), 100 operations/y (OR, 1.37; 95% CrI, 1.08-1.71), 200 operations/y (OR, 0.92; 95% CrI, 0.1-1.18), 300 operations/y (OR, 0.89; 95% CrI, 0.76-1.04). The volume-outcome effect was more apparent for STAT 4 to 5 than STAT 1 to 3 operations. In the overall cohort, PLOS and complications were similar across hospital volumes, whereas FTR rates were higher at lower-volume hospitals. Lower-volume hospitals had worse outcomes after the Norwood procedure, most notably mortality and FTR. CONCLUSIONS: Hospital volume is associated with mortality and FTR after CHS. The relationship is strongest for high-risk operations. These data can inform ongoing initiatives to improve CHS care.

Representation of Women and Minority Faculty and Fellows in Academic Pediatric Cardiology Training Programs.

BACKGROUND: Studies have shown that diverse care teams optimize patient outcomes. Describing the current representation of women and minorities has been a critical step in improving diversity across several fields. OBJECTIVES: To address the lack of data specific to pediatric cardiology, the authors conducted a national survey. METHODS: U.S. academic pediatric cardiology programs with fellowship training programs were surveyed. Division directors were invited (July 2021 to September 2021) to complete an e-survey of program composition. Underrepresented minorities in medicine (URMM) were characterized using standard definitions. Descriptive analyses at the hospital, faculty, and fellow level were performed. RESULTS: Altogether, 52 of 61 programs (85%) completed the survey, representing 1,570 total faculty and 438 fellows, with a wide range in program size (7-109 faculty, 1-32 fellows). Although women comprise approximately 60% of faculty in pediatrics overall, they made up 55% of fellows and 45% of faculty in pediatric cardiology. Representation of women in leadership roles was notably less, including 39% of clinical subspecialty directors, 25% of endowed chairs, and 16% of division directors. URMM comprise approximately 35% of the U.S. population; however, they made up only 14% of pediatric cardiology fellows and 10% of faculty, with very few in leadership roles. CONCLUSIONS: These national data suggest a "leaky pipeline" for women in pediatric cardiology and very limited presence of URRM overall. Our findings can inform efforts to elucidate underlying mechanisms for persistent disparity and reduce barriers to improving diversity in the field.

Comparison of treatment strategies for neonates with tetralogy of Fallot and pulmonary atresia.

OBJECTIVE: Neonates with tetralogy of Fallot and pulmonary atresia (TOF/PA) but no major aorta-pulmonary collaterals are dependent on the arterial duct for pulmonary blood flow and require early intervention, either by primary (PR) or staged repair (SR) with initial palliation (IP) followed by complete repair (CR). The optimal approach has not been established. METHODS: Neonates with TOF/PA who underwent PR or SR were retrospectively reviewed from the Congenital Cardiac Research Collaborative. Outcomes were compared between PR and SR (IP + CR) strategies. Propensity scoring was used to adjust for baseline differences. The primary outcome was mortality. Secondary outcomes included complications, length of stay, cardiopulmonary bypass and anesthesia times, reintervention (RI), and pulmonary artery (PA) growth. RESULTS: Of 282 neonates, 106 underwent PR and 176 underwent SR (IP: 144 surgical, 32 transcatheter). Patients who underwent SR were more likely to have DiGeorge syndrome and greater rates of mechanical ventilation before the initial intervention. Mortality was not significantly different. Duration of mechanical ventilation, inotrope use, and complication rates were similar. Cumulative length of stay, cardiopulmonary bypass, and anesthesia times favored PR (P ≤ .001). Early RI was more common in patients who underwent SR (rate ratio, 1.42; P = .003) but was similar after CR (P = .837). Conduit size at the time of CR was larger with SR. Right PA growth was greater with PR. CONCLUSIONS: In neonates with TOF/PA, SR is more common in greater-risk patients. Accounting for this, SR and PR strategies have similar mortality. Perioperative morbidities, RI, and right PA growth generally favor PR, whereas SR allows for larger initial conduit implantation.

Aortic valve repair using geometric ring annuloplasty in pediatric and congenital heart disease patients.

OBJECTIVE: Existing replacement options for the aortic valve have significant drawbacks, especially in children. A geometric annuloplasty ring can help to achieve consistently successful aortic valve repair, but only limited experience with use of this device has been reported in pediatric and congenital heart disease patients. METHODS: All pediatric and adult congenital patients at our institution who underwent aortic valve repair with placement of a geometric annuloplasty ring were reviewed. The study period spanned from July 2018 to April 2022. Hemodynamic outcomes were evaluated using transthoracic echocardiography. RESULTS: The study included 36 subjects. The median age was 17.4 years (range, 8-30 years). Twenty-one subjects were younger than age 18 years. The most common primary diagnoses were neoaortic valve insufficiency or neoaortic root dilation, and congenital aortic stenosis with bicuspid or functionally unicuspid aortic valve. Of the 34 subjects with procedural success, 31 (91%) had use of additional valve repair techniques and 26 (76%) had an additional concomitant procedure performed. Operative mortality was 0% (0/33), and major complication rate was 6% (2/33). The median follow-up time was 1.9 years (maximum, 3.8 years). The mean grade of aortic insufficiency was signific antly reduced after repair, with no change in mean gradients. Freedom from reoperation over the follow-up period was 97% (33/34), and freedom from ≥3+ recurrent aortic insufficiency was 94% (32/34). CONCLUSIONS: A geometric annuloplasty ring can be used to help achieve consistently successful aortic valve repair with excellent perioperative and follow-up outcomes, even in pediatric and complex congenital heart disease patients.