The American Association for Thoracic Surgery (AATS) 2024 expert consensus document: Management of neonates and infants with Ebstein anomaly.

OBJECTIVES: Symptomatic neonates and infants with Ebstein anomaly (EA) require complex management. A group of experts was commissioned by the American Association for Thoracic Surgery to provide a framework on this topic focusing on risk stratification and management. METHODS: The EA Clinical Congenital Practice Standards Committee is a multinational and multidisciplinary group of surgeons and cardiologists with expertise in EA. A citation search in PubMed, Embase, Scopus, and Web of Science was performed using key words related to EA. The search was restricted to the English language and the year 2000 or later and yielded 455 results, of which 71 were related to neonates and infants. Expert consensus statements with class of recommendation and level of evidence were developed using a modified Delphi method, requiring 80% of members votes with at least 75% agreement on each statement. RESULTS: When evaluating fetuses with EA, those with severe cardiomegaly, retrograde or bidirectional shunt at the ductal level, pulmonary valve atresia, circular shunt, left ventricular dysfunction, or fetal hydrops should be considered high risk for intrauterine demise and postnatal morbidity and mortality. Neonates with EA and severe cardiomegaly, prematurity (<32 weeks), intrauterine growth restriction, pulmonary valve atresia, circular shunt, left ventricular dysfunction, or cardiogenic shock should be considered high risk for morbidity and mortality. Hemodynamically unstable neonates with a circular shunt should have emergent interruption of the circular shunt. Neonates in refractory cardiogenic shock may be palliated with the Starnes procedure. Children may be assessed for later biventricular repair after the Starnes procedure. Neonates without high-risk features of EA may be monitored for spontaneous closure of the patent ductus arteriosus (PDA). Hemodynamically stable neonates with significant pulmonary regurgitation at risk for circular shunt with normal right ventricular systolic pressure should have an attempt at medical closure of the PDA. A medical trial of PDA closure in neonates with functional pulmonary atresia and normal right ventricular systolic pressure (>20-25 mm Hg) should be performed. Neonates who are hemodynamically stable without pulmonary regurgitation but inadequate antegrade pulmonary blood flow may be considered for a PDA stent or systemic to pulmonary artery shunt. CONCLUSIONS: Risk stratification is essential in neonates and infants with EA. Palliative comfort care may be reasonable in neonates with associated risk factors that may include prematurity, genetic syndromes, other major medical comorbidities, ventricular dysfunction, or sepsis. Neonates who are unstable with a circular shunt should have emergent interruption of the circular shunt. Neonates who are unstable are most commonly palliated with the Starnes procedure. Neonates who are stable should undergo ductal closure. Neonates who are stable with inadequate pulmonary flow may have ductal stenting or a systemic-to-pulmonary artery shunt. Subsequent procedures after Starnes palliation include either single-ventricle palliation or biventricular repair strategies.

Staged Ventricular Septation of the Double-Inlet Ventricle: How-I-Do-It.

Ventricular septation of the double-inlet ventricle is a largely abandoned operation due to poor historical outcomes. However, there has been renewed interest in septation as an alternative to Fontan palliation given its long-term sequelae. As one of the few centers to revisit septation in the early 1990s, our institution has long-term data on a series of patients with a double-inlet ventricle who underwent biventricular repair. This manuscript is a summary of our approach to staged septation of the double-inlet ventricle, with a focus on patient selection criteria, surgical techniques, perioperative considerations on timing of interventions, and long-term results. We believe that septation of the double-inlet ventricle should be reconsidered in patients with suitable anatomy in light of the known complications of Fontan palliation.

Clinical Outcomes and Echocardiographic Predictors of Reintervention After Interrupted Aortic Arch Repair.

Left ventricular outflow tract obstruction (LVOTO) remains a significant complication after primary repair of interrupted aortic arch with ventricular septal defect (IAA-VSD). Clinical and echocardiographic predictors for LVOTO reoperation are controversial and procedures to prophylactically prevent future LVOTO are not reliable. However, it is important to identify the patients at risk for future LVOTO intervention after repair of IAA-VSD. Patients who underwent single-stage IAA-VSD repair at our center 2006-2021 were retrospectively reviewed, excluding patients with associated cardiac lesions. Two-dimensional measurements, LVOT gradients, and 4-chamber (4C) and short-axis (SAXM) strain were obtained from preoperative and predischarge echocardiograms. Univariate risk analysis for LVOTO reoperation was performed using unpaired t-test. Thirty patients were included with 21 (70%) IAA subtype B and mean weight at surgery 3.0 kg. Repair included aortic arch patch augmentation in 20 patients and subaortic obstruction intervention in three patients. Seven (23%) required reoperations for LVOTO. Patient characteristics were similar between patients who required LVOT reoperation and those who did not. Patch augmentation was not associated with LVOTO reintervention. Patients requiring reintervention had significantly smaller LVOT AP diameter preoperatively and at discharge, and higher LVOT velocity, smaller AV annular diameter, and ascending aortic diameter at discharge. There was an association between LVOT-indexed cross-sectional area (CSAcm2/BSAm2) ≤ 0.7 and reintervention. There was no significant difference in 4C or SAXM strain in patients requiring reintervention. LVOTO reoperation was not associated with preoperative clinical or surgical variables but was associated with smaller LVOT on preoperative echo and smaller LVOT, smaller AV annular diameter, and increased LVOT velocity at discharge.

Aortic insufficiency following balloon aortic valvuloplasty does not impact long-term autograft durability after the Ross procedure.

OBJECTIVE: Preoperative aortic insufficiency (AI) is associated with inferior autograft durability after the Ross procedure. However, many patients with aortic stenosis (AS) undergo balloon aortic valvuloplasty (BAV) early and present with longstanding AI before Ross. We studied how BAV and subsequent valvular pathology impacts autograft durability. METHODS: Patients undergoing the Ross operation from 1993 to 2020 were identified. Those who underwent BAV before Ross were compared with patients who did not undergo BAV and underwent Ross for predominant AI (AI group) or AS (AS group). Those who underwent previous open surgical aortic valve intervention were excluded. Primary outcome of interest was autograft failure, defined as a composite of autograft reintervention or severe insufficiency. RESULTS: A total of 198 patients were included. Seventy-nine (39.9%) underwent BAV and subsequently underwent the Ross for predominant AI (45.6%) or AS (54.4%). Of patients who did not undergo BAV, 66 (33.3%) presented with predominant AI and 53 (26.8%) with AS. Freedom from autograft failure at 15 years was 90%, 92%, and 62% in BAV, AS, and AI groups, respectively. The AI group was at significantly increased risk of long-term autograft failure (hazard ratio, 5.6; P = .01), whereas the AS and BAV groups had similar, low risk (hazard ratio, 1.1; P = .91). Autograft durability was similar among patients who received BAV and presented with AS or AI before the Ross (P = .84). CONCLUSIONS: BAV before the Ross procedure is common in patients with AS. These patients have excellent long-term autograft durability regardless of preoperative valvular pathology and should strongly be considered for the Ross operation.

Long-term autograft dilation and durability after the Ross procedure are similar in infants, children, and adolescents with primary aortic stenosis.

BACKGROUND: Autograft durability and remodeling are thought to be superior in younger pediatric patients after the Ross operation. We sought to delineate the fate of autografts across the pediatric age spectrum in patients with primary aortic stenosis (AS). METHODS: We retrospectively reviewed patients age ≤18 years with primary AS who underwent the Ross operation between 1993 and 2020. Patients were categorized by age. The primary endpoint was autograft dimensional change, and secondary endpoints were severe neo-aortic insufficiency (AI) and autograft reintervention. RESULTS: A total of 119 patients underwent the Ross operation, including 37 (31.1%) in group I (age <18 months), 24 (20.2%) in group II (age 18 months-8 years), and 58 (48.7%) in group III (age 8-18 years). All groups exhibited similar annular growth rates within the first 5 postoperative years, followed by a collective decrease in annulus growth rates from year 5 to year 10. Group III experienced rapid sinus dilation in the first 5 years, followed by stabilization of the sinus z-score from year 5 to year 10, whereas groups I and II demonstrated stable sinus z-scores over 10 years. There were 4 early deaths (3.4%) and 2 late deaths (1.7%) at a median follow-up of 8.1 years (range, 0.01-26.3 years). At 15 years, the incidences of severe neo-AI (0.0 ± 0.0% vs 0.0 ± 0.0% vs 3.9 ± 3.9%; P = .52) and autograft reintervention (8.4 ± 6.0% vs 0.0 ± 0.0% vs 2.4 ± 2.4%; P = .47) were similar in the 3 groups. CONCLUSIONS: Age at the time of Ross operation for primary AS does not influence long-term autograft remodeling or durability. Other physiologic or technical factors are likely greater determinants of autograft fate.

Influence of preoperative aortic regurgitation on long-term autograft durability and dilatation in children and adolescents undergoing the Ross procedure.

OBJECTIVE: Primary aortic insufficiency (AI) is a risk factor for autograft reintervention in adults undergoing the Ross procedure. We sought to examine the influence of preoperative AI on autograft durability in children and adolescents. METHODS: From 1993 to 2020, 125 consecutive patients between ages 1 and 18 underwent a Ross procedure. The autograft was implanted using a full-root technique in 123 (98.4%) and included in a polyethelene terephthalate graft in 2 (1.6%). Patients with aortic stenosis (aortic stenosis group) (n = 85) were retrospectively compared with those with AI or mixed disease (AI group) (n = 40). Median length of follow-up was 8.2 years (interquartile range, 3.3-15.4 years). The primary end point was the incidence of severe AI or autograft reintervention. Secondary end points included changes in autograft dimensions analyzed using mixed-effect models. RESULTS: The incidence of severe AI or autograft reintervention was 39.0% ± 13.0% in the AI group and 8.8% ± 4.4% in the aortic stenosis group at 15 years (P = .02). Annulus z scores increased in both aortic stenosis and AI groups over time (P < .001). However, the annulus dilated at a faster rate in the AI group (absolute difference, 3.8 ± 2.0 vs 2.5 ± 1.7; P = .03). Sinus of Valsalva z scores increased in both groups as well (P < .001), but at similar rates over time (P = .11). CONCLUSIONS: Children and adolescents with AI undergoing the Ross procedure have higher rates of autograft failure. Patients with preoperative AI have more pronounced dilatation at the annulus. Akin to adults, a surgical aortic annulus stabilization technique that modulates growth is needed in children.

Valved Sano conduit improves immediate outcomes following Norwood operation compared with nonvalved Sano conduit.

OBJECTIVE: Use of a valved Sano during the Norwood procedure has been reported previously, but its impact on clinical outcomes needs to be further elucidated. We assessed the impact of the valved Sano compared with the nonvalved Sano after the Norwood procedure in patients with hypoplastic left heart syndrome. METHODS: We retrospectively reviewed 25 consecutive neonates with hypoplastic left heart syndrome who underwent a Norwood procedure with a valved Sano conduit using a femoral venous homograft and 25 consecutive neonates with hypoplastic left heart syndrome who underwent a Norwood procedure with a nonvalved Sano conduit between 2013 and 2022. Primary outcomes were end-organ function postoperatively and ventricular function over time. Secondary outcomes were cardiac events, all-cause mortality, and Sano and pulmonary artery reinterventions at discharge, interstage, and pre-Glenn time points. RESULTS: Postoperatively, the valved Sano group had significantly lower peak and postoperative day 1 lactate levels (P = .033 and P = .025, respectively), shorter time to diuresis (P = .043), and shorter time to enteral feeds (P = .038). The valved Sano group had significantly fewer pulmonary artery reinterventions until the Glenn operation (n = 1 vs 8; P = .044). The valved Sano group showed significant improvement in ventricular function from the immediate postoperative period to discharge (P < .001). From preoperative to pre-Glenn time points, analysis of ventricular function showed sustained ventricular function within the valved Sano group, but a significant reduction of ventricular function in the nonvalved Sano group (P = .003). Pre-Glenn echocardiograms showed competent conduit valves in two-thirds of the valved Sano group (n = 16; 67%). CONCLUSIONS: The valved Sano is associated with improved multi-organ recovery postoperatively, better ventricular function recovery, and fewer pulmonary artery reinterventions until the Glenn procedure.

Comparing apples to apples: Exploring public reporting of congenital cardiac surgery outcomes based on common congenital heart operations.

OBJECTIVE: We sought to simplify reporting of outcomes in congenital heart surgery that compares well-defined patient groups and accommodates multiple stakeholder needs while being easily understandable. METHODS: We selected 19 commonly performed congenital heart surgeries ranging in complexity from repair of atrial septal defects to the Norwood procedure. Strict inclusion/exclusion criteria ensured the creation of 19 well-defined diagnosis/procedure cohorts. Preoperative, procedural, and postoperative data were collected for consecutive eligible patients from 9 centers between January 1, 2016, and December 31, 2021. Unadjusted operative mortality rates and hospital length of stay for each of the 19 diagnosis/procedure cohorts were summarized in aggregate and stratified by each center. RESULTS: Of 8572 eligible cases included, numbers in the 19 diagnosis/procedure cohorts ranged from 73 for tetralogy of Fallot repair after previous palliation to 1224 for ventricular septal defect (VSD) repair for isolated VSD. In aggregate, the unadjusted mortality ranged from 0% for atrial septal defect repair to 28.4% for hybrid stage I. There was significant heterogeneity in case mix and mortality for different diagnosis/procedure cohorts across centers (eg, arterial switch operation/VSD, n = 7-42, mortality 0%-7.4%; Norwood procedure, n = 16-122, mortality 5.3%-25%). CONCLUSIONS: Reporting of institutional case volumes and outcomes within well-defined diagnosis/procedure cohorts can enable centers to benchmark outcomes, understand trends in mortality, and direct quality improvement. When made public, this type of report could provide parents with information on institutional volumes and outcomes and allow them to better understand the experience of each program with operations for specific congenital heart defects.

Strategies for the Surgical Management of Highly Aggressive Williams Syndrome Aortopathy: A Three Case Report.

Severe aortopathy in Williams syndrome can sometimes present with an initial ascending aortic pathology, followed in short order by more distal multilevel obstruction and recurrence requiring reintervention. In this series, an early, comprehensive surgical approach using a combination of various access and perfusion strategies yielded excellent long-term results.

Aortic root translocation (Nikaidoh) procedure for complex transposition of the great arteries with left ventricular outflow tract obstruction.

Background: Several surgical techniques have been developed for the management of complex transposition of the great arteries with ventricular septal defect and left ventricular outflow tract obstruction (TGA/VSD/LVOTO). Aortic root translocation, or the Nikaidoh operation, offers the most anatomic biventricular repair in these patients. However, the Nikaidoh operation commonly has been limited to patients with "typical" anatomy, including a conoventricular VSD and usual coronary anatomy. We sought to describe a single surgeon's experience with aortic root translocation for complex TGA/VSD/LVOTO. Methods: We present a series of 12 patients with complex anatomy who underwent the Nikaidoh operation over the last 13 years. Results: We report good mid- to long-term results, excellent performance of the reconstructed left ventricular outflow tract, aortic valve competence, and no coronary insufficiency. Conclusions: Our experience suggests that the Nikaidoh operation is a valid option even for patients with complex TGA/VSD/LVOTO.

Management of systemic to pulmonary shunts and elevated pulmonary vascular resistance.

Background: Repair of systemic to pulmonary shunts is timed to prevent the development of irreversible pulmonary vascular disease, including in patients with other factors contributing to pulmonary hypertension. This study assessed outcomes of an individualised strategy for managing patients with mild-moderately elevated pulmonary vascular resistance (PVR) deemed borderline eligible for repair. Methods: A retrospective chart review was conducted of patients with systemic to pulmonary shunts and baseline indexed PVR (PVRi) ≥3 WU·m2 treated at a single centre from 1 January 2005 to 30 September 2019. Data included demographics, World Health Organization functional class (WHO FC), medications and haemodynamic data at baseline and serial follow-up. Results: 30 patients (18 females) met criteria for inclusion. Median age at diagnosis of pulmonary arterial hypertension was 1.3 years (range 0.03-54 years) and at surgery was 4.1 years (range 0.73-56 years). Median follow-up time was 5.8 years (range 0.2-14.6 years) after repair. Most patients received at least one targeted pulmonary arterial therapy prior to repair and the majority (80%) underwent fenestrated shunt closure. There was a significant decrease in mean pulmonary arterial pressure (mPAP) (p<0.01), PVRi (p=0.0001) and PVR/systemic vascular resistance (p<0.01) between baseline and preoperative catheterisation and a decrease in PVRi (p<0.005), mPAP (p=0.0001) and pulmonary to systemic flow ratio (p<0.03) from baseline to most recent catheterisation. WHO FC improved from FC II-III at baseline to FC I post repair in most patients (p<0.003). Conclusions: In carefully selected patients with systemic to pulmonary shunts and elevated PVR considered borderline for operability, the use of preoperative targeted therapy in conjunction with fenestrated or partial closure of intracardiac shunts is associated with improvement in WHO FC and clinical outcomes.

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.