Textbook outcome for the Norwood operation-an informative quality metric in congenital heart surgery.

Objectives: To develop a more holistic measure of center performance than operative mortality, we created a composite "textbook outcome" for the Norwood operation using several postoperative end points. We hypothesized that achieving the textbook outcome would have a positive prognostic and financial impact. Methods: This was a single-center retrospective study of primary Norwood operations from 2005 to 2021. Through interdisciplinary clinician consensus, textbook outcome was defined as freedom from operative mortality, open or catheter-based reintervention, 30-day readmission, extracorporeal membrane oxygenation, cardiac arrest, reintubation, length of stay >75%ile from Society of Thoracic Surgeons data report (66 days), and mechanical ventilation duration >75%ile (10 days). Multivariable logistic regression and Cox proportional hazards modeling were used to determine predictive factors for textbook outcome achievement and association of the outcome with long-term survival, respectively. Results: Overall, 30% (58/196) of patients met the textbook outcome. Common reasons for failure to attain textbook outcome were prolonged ventilation (68/138, 49%) and reintubation (63/138, 46%). In multivariable analysis, greater weight (odds ratio [OR], 2.11; 95% confidence interval [CI], 1.17-3.95; P = .02) was associated with achieving the textbook outcome whereas preoperative shock (OR, 0.36; 95% CI, 0.13-0.87; P = .03) and longer bypass time (OR, 0.99; 95% CI, 0.98-1.00; P = .002) were negatively associated. Patients who met the outcome incurred fewer hospital costs ($152,430 [141,798-177,983] vs $269,070 [212,451-372,693], P < .001), and after adjusting for patient factors, achieving textbook outcome was independently associated with decreased risk of all-cause mortality (hazard ratio, 0.45; 95% CI, 0.22-0.89; P = .02). Conclusions: Outcomes continue to improve within congenital heart surgery, making operative mortality a less-sensitive metric. The Norwood textbook outcome may represent a balanced measure of a successful episode of care.

The Congenital Heart Surgeons' Society Kirklin/Ashburn Fellowship: The Fellows' Perspective.

Since its establishment in 2001, the Congenital Heart Surgeons' Society John W. Kirklin/David Ashburn Fellowship has contributed substantially to the field of congenital heart surgery research while simultaneously training the next generation of surgeon- scientists. To date, ten fellows (and counting) have successfully completed this rigorous training, producing over 40 published articles focused on longitudinal outcomes from the various Congenital Heart Surgeons' Society cohorts. As the Kirklin/Ashburn Fellowship expands and additional fellows matriculate, its legacy, the network of support, and the contribution to congenital heart surgery research will undoubtedly hold strong.

A dynamic Norwood mortality estimation: Characterizing individual, updated, predicted mortality trajectories after the Norwood operation.

Objective: Post-Norwood mortality remains high and unpredictable. Current models for mortality do not incorporate interstage events. We sought to determine the association of time-related interstage events, along with (pre)operative characteristics, with death post-Norwood and subsequently predict individual mortality. Methods: From the Congenital Heart Surgeons' Society Critical Left Heart Obstruction cohort, 360 neonates underwent Norwood operations from 2005 to 2016. Risk of death post-Norwood was modeled using a novel application of parametric hazard analysis, in which baseline and operative characteristics and time-related adverse events, procedures, and repeated weight and arterial oxygen saturation measurements were considered. Individual predicted mortality trajectories that dynamically update (increase or decrease) over time were derived and plotted. Results: After the Norwood, 282 patients (78%) progressed to stage 2 palliation, 60 patients (17%) died, 5 patients (1%) underwent heart transplantation, and 13 patients (4%) were alive without transitioning to another end point. In total, 3052 postoperative events occurred and 963 measures of weight and oxygen saturation were obtained. Risk factors for death included resuscitated cardiac arrest, moderate or greater atrioventricular valve regurgitation, intracranial hemorrhage/stroke, sepsis, lower longitudinal oxygen saturation, readmission, smaller baseline aortic diameter, smaller baseline mitral valve z-score, and lower longitudinal weight. Each patient's predicted mortality trajectory varied as risk factors occurred over time. Groups with qualitatively similar mortality trajectories were noted. Conclusions: Risk of death post-Norwood is dynamic and most frequently associated with time-related postoperative events and measures, rather than baseline characteristics. Dynamic predicted mortality trajectories for individuals and their visualization represent a paradigm shift from population-derived insights to precision medicine at the patient level.

Sustained Total All-Region Perfusion During the Norwood Operation and Postoperative Recovery.

We developed a technique for the Norwood operation utilizing continuous perfusion of the head, heart, and lower body at mild hypothermia named Sustained Total All-Region (STAR) perfusion. We hypothesized that STAR perfusion would be associated with shorter operative times, decreased coagulopathy, and expedited post-operative recovery compared to standard perfusion techniques. Between 2012 and 2020, 80 infants underwent primary Norwood reconstruction at our institution. Outcomes for patients who received successful STAR perfusion (STAR, n = 37) were compared to those who received standard Norwood reconstruction utilizing regional cerebral perfusion only (SNR, n = 33), as well as to Norwood patients reported in the PC4 national database during the same timeframe (n = 1238). STAR perfusion was performed with cannulation of the innominate artery, descending aorta, and aortic root at 32-34°C. STAR patients had shorter median CPB time compared to SNR (171 vs 245 minutes, P < 0.0001), shorter operative time (331 vs 502 minutes, P < 0.0001), and decreased intraoperative pRBC transfusion (100 vs 270 mL, P < 0.0001). STAR patients had decreased vasoactive-inotropic score on ICU admission (6 vs 10.8, P = 0.0007) and decreased time to chest closure (2 vs 4.5 days, P = 0.0004). STAR patients had lower peak lactate (8.1 vs 9.9 mmol/L, P = 0.03) and more rapid lactate normalization (18.3 vs 27.0 hours, P = 0.003). In-hospital mortality in STAR patients was 2.7% vs 15.1% with SNR (P = 0.06) and 10.3% in the PC4 aggregate (P = 0.14). STAR perfusion is a novel approach to Norwood reconstruction associated with excellent survival, decreased transfusions, shorter operative time, and improved convalescence in the early post-operative period.

Impact of Ventricular Dominance on Long-Term Fontan Outcomes: A 25-year Single-institution Study.

BACKGROUND: The long-term impact of ventricular dominance on Fontan outcomes is controversial. This study examined this issue in a 25-year cohort. METHODS: Patients undergoing the Fontan operation at a single institution (Duke University Medical Center, Durham, NC) from October 1998 to February 2022 were reviewed. Primary outcomes were transplant-free survival and Fontan failure (death, heart transplantation, takedown, protein-losing enteropathy, or plastic bronchitis). Secondary outcomes included hospital and intensive care lengths of stay. Kaplan-Meier methodology compared outcomes by ventricular dominance. Multiphase parametric risk hazard analysis identified risk factors for primary outcomes. RESULTS: There were 195 patients (104 right ventricular dominant) included in the study. Baseline characteristics were comparable. Perioperative survival was similar (right ventricular dominant, 98%; non-right ventricular dominant, 100%; P = .51). The proportion of patients experiencing death or heart transplantation was 8.7%, and the rate of Fontan failure was 11.8% during a median follow-up of 4.5 years (interquartile range, 0.3-9.8 years). Right ventricular-dominant patients had reduced transplant-free survival (10-year estimates: 80% [95% CI, 70%-91%] vs 92% [95% CI, 83%-100%]; P = .04) and freedom from Fontan failure (73% [95% CI, 62%-86%] vs 92% [95% CI, 83%-100%]; P = .04). Multiphase hazard modeling resolved 2 risk phases. The early phase spanned from surgery to approximately 6 months afterward. The late phase spanned from approximately 6 months after surgery onward. In multivariable analysis, right ventricular dominance was an independent risk factor for death or heart transplantation (parameter estimate, 1.3 ± 0.6; P = .04) and Fontan failure (1.1 ± 0.5; P = .04) during the second phase, with no significant first-phase risk factors. CONCLUSIONS: Right ventricular dominance was associated with long-term complications after Fontan procedures, including mortality, heart transplantation, and Fontan failure. This cohort may benefit from heightened surveillance in a multidisciplinary Fontan clinic after the perioperative period.

Predictors of increased postoperative length of stay after complete atrioventricular canal repair.

BACKGROUND: The optimal timing of surgical repair for infants with complete atrioventricular canal defect remains controversial, as there are risks to both early and late repair. We address this debate by investigating the association of various risk factors, including age and weight at surgery, markers of failure to thrive, and pulmonary vascular disease, with postoperative length of stay following complete atrioventricular canal repair. METHODS: Infants who underwent repair of complete atrioventricular canal were identified from our institutional Society of Thoracic Surgeons Congenital Heart Surgery Database. Additional clinical data were collected from the electronic medical record. Descriptive statistics were computed. Associations between postoperative length of stay and covariates of interest were evaluated using linear regression with bootstrap aggregation. RESULTS: From 2001 to 2020, 150 infants underwent isolated complete atrioventricular canal repair at our institution. Pre-operative failure to thrive and evidence of pulmonary disease were common. Surgical mortality was 2%. In univariable analysis, neither weight nor age at surgery were associated with mortality, postoperative length of stay, duration of mechanical ventilation, or post-operative severe valvular regurgitation. In multivariable analysis of demographic and preoperative clinical factors using bootstrap aggregation, increased postoperative length of stay was only significantly associated with previous pulmonary artery banding (33.9 day increase, p = 0.03) and preoperative use of supplemental oxygen (19.9 day increase, p = 0.03). CONCLUSIONS: Our analysis shows that previous pulmonary artery banding and preoperative use of supplemental oxygen were associated with increased postoperative length of stay after complete atrioventricular canal repair, whereas age and weight were not. These findings suggest operation prior to the onset of pulmonary involvement may be more important than reaching age or weight thresholds.

Survival After Intervention for Single-Ventricle Heart Disease Over 15 Years at a Single Institution.

BACKGROUND: Children with single-ventricle (SV) heart disease possess a spectrum of heart malformations, yet progress through similar hemodynamic states, suggesting differences in outcomes are related to fundamental morphologic differences, patient characteristics, or procedural pathways. We sought to provide a holistic overview of survival after intervention for SV heart disease at our institution. METHODS: SV heart disease was defined as patients born with a hypoplastic or dysfunctional ventricle with uncertain or unacceptable candidacy for a 2-ventricle circulation. Patients were stratified into 8 diagnostic groups and 11 procedural categories based on the initial interventional procedure. RESULTS: Between 2005 and 2020, 381 patients born with SV heart disease underwent intervention at our institution. Ten-year survival was highest for patients with double inlet left ventricle (89% ± 7%) and lowest for patients with hypoplastic left heart syndrome (55% ± 5%). Initial palliation with less invasive procedures, such as ductal stent (4-year: 100%) or pulmonary artery banding (10-year: 95% ± 5%), demonstrated superior survival compared with more invasive procedures such as the Norwood procedure (10-year: 59% ± 4%). Survival of patients who achieved a biventricular circulation was superior to patients who remained with SV physiology (10-year: 87% ± 5% vs 63% ± 3%, P = .04). In a multivariable analysis, chromosomal/syndromic abnormality, lower weight, hybrid Norwood procedure, nonleft ventricular dominance, and earlier year of operation were risk factors for death. CONCLUSIONS: Survival differences in patients with SV heart disease were related primarily to underlying cardiac anatomy, patient characteristics, and procedural complexity. Left ventricular dominance, more recent intervention, and attainment of a 2-ventricle circulation were associated with improved survival.

Anomalous Aortic Origin of a Coronary Artery Repair Through an Anterior Minithoracotomy.

OBJECTIVE: The benefits of minimally invasive adult cardiac surgery are well established. Nevertheless, minimally invasive congenital cardiac procedures, even for adult patients, are uncommon. In 2018, we started repairing anomalous aortic origin of a coronary artery (AAOCA) through a 5 cm anterior minithoracotomy when possible to improve cosmesis and avoid sternal precautions. We hypothesized this approach was safe and reliable. METHODS: A 5 cm incision was made in the right second intercostal space. The incision was carried down to the pericardium while preserving the internal mammary artery. With the pericardium in view, the second and third ribs were disarticulated. Central cardiopulmonary bypass was established, and the repair was carried out based on the patient's anatomy. The technique was modified to a left anterior minithoracotomy for 1 patient who required pulmonary artery translocation. At any point, if the dissection or repair was not progressing appropriately, the minimally invasive exposure was converted to a partial or traditional median sternotomy. RESULTS: Between June 2018 and June 2019, 11 patients underwent minimally invasive anomalous coronary repair. Four patients (3 with body mass index >30) were converted to traditional sternotomy due to poor visualization. Postoperatively, 1 patient required coronary artery bypass after 335 days, due to extensive collaterals and stable angina. Otherwise, at a median follow-up of 437 days (IQR 340 to 480), patients had resumed baseline activity without recurrent symptoms. CONCLUSIONS: Minimally invasive AAOCA repair may be appealing, although surgeons should be cautious given the high conversion rate.

Complex Repair of a Completely Fused Nullicuspid Aortic Valve Late in Adolescence.

We report a case of an 18-year-old female who presented with severe aortic stenosis and insufficiency, eight years following resection of a subaortic membrane. On echocardiography, she was found to have a completely fused or nullicuspid valve, with three equal sinuses and three commissural fusions. Aortic valve repair included leaflet tricuspidization, three commissurotomies, trileaflet ring annuloplasty, and pericardial leaflet reconstruction. At one year follow-up, the patient is asymptomatic, with stable gradients.

Transition to Ductal Stenting for Single Ventricle Patients Led to Improved Survival: An Institutional Case Series.

BACKGROUND: The use of systemic-to-pulmonary shunts (SPS) in neonates with single ventricle heart defects and ductal-dependent pulmonary blood flow (ddPBF) was historically associated with high morbidity and mortality at our center. As a result, we transitioned to the preferential use of ductus arteriosus stents (DS) when feasible. This report describes our initial results with this strategy. METHODS: A single-center study of single ventricle patients that received DS or SPS from 2015 to 2019 was performed to assess whether DS was associated with decreased in-hospital morbidity and increased survival to stage II palliation. RESULTS: A total of 34 patients were included (DS = 11; SPS = 23). Underlying cardiac anomalies were similar between groups and included pulmonary atresia, unbalanced atrioventricular septal defect, and tricuspid atresia. Procedure success was similar between groups (82% vs 83%). Two DS patients were converted to SPS, due to ductal vasospasm or pulmonary artery obstruction, and four SPS patients required surgical shunt revision. In DS patients, postprocedure mechanical ventilation duration was shorter (one vs three days, P = .009) and fewer required postprocedure extracorporeal membrane oxygenation (9% vs 39%, P = .11). A higher proportion of DS patients survived to stage II palliation (100% vs 64%, P = .035), and the probability of one-year survival was higher in DS patients (100% vs 61%, P = .02). CONCLUSIONS: At our center, patients with single ventricle heart defects and ddPBF that received DS experienced reduced in-hospital morbidity and increased survival to stage II palliation compared to SPS.

Implantation of a HeartMate 3 ventricular assist device in a 21-kg pediatric patient with Fontan failure.

AIMS: The HeartMate 3 (HM3) ventricular assist device (VAD) is gaining popularity in adults due to a favorable risk profile. However, reports of HM3 use in children are limited, potentially due to concerns with device size. MATERIALS AND METHODS: Here we report the successful use of an HM3-VAD as a bridge to transplantation in a 21 kg (BSA 0.84), an 8-year-old male child with Fontan failure on home inotropes. RESULTS: Urgent VAD implantation was performed. The standard adult sewing ring was used. The tricuspid valve and papillary muscles were completely excised from the ventricular cavity to prevent inflow obstruction. The pump was placed in the left pleural space. Outflow graft and driveline implantation were routine. VAD function appeared excellent with a reduction in Fontan pressures and improved kidney and liver function. Reoperation was required once to rule out tamponade and twice to evacuate a recurrent right hemothorax. The patient was discharged 3 months later in good condition and underwent successful heart transplantation 10 months after VAD placement. DISCUSSION: This report demonstrates the feasibility of HM3-VAD implantation in a 21-kg Fontan patient, suggesting HM3 size is not a prohibitive limitation at this weight.

Left Anterior Mini-Incision for Pulmonary Valve Replacement in a 12-Year-Old.

A 12-year-old, 32 kg male with history of Tetralogy of Fallot status post repair at 8 days of life presented with progressive pulmonary insufficiency and left pulmonary artery stenosis. Surgical options were discussed, and the patient and his family elected to pursue minimally invasive pulmonary valve replacement with left pulmonary artery augmentation through a 5-cm left anterior mini-incision. The procedure was performed without complication, and he was discharged on postoperative day 3. At the time of his last follow-up, the patient was recovering well without evidence of pulmonary stenosis or insufficiency.

Left Anterior Mini-Incision for Pulmonary Valve Replacement Following Tetralogy of Fallot Repair.

Pulmonary insufficiency is a known complication following Tetralogy of Fallot repair. With over 90% of patients now surviving to adulthood, surgeons are once again faced with the question of when, and more importantly, how to reintervene. We developed a novel approach to pulmonary valve replacement in this population through a 5-cm left anterior mini-incision. The incision is optimized for exposing and operating on the right ventricular outflow tract and the main pulmonary artery in patients with a history of median sternotomy. Early outcomes are reassuring, and we believe our approach is a safe and reliable alternative to median sternotomy within this patient population, with the ability to quickly convert intraoperatively when needed.

Initial Application of a Bicuspid Aortic Annuloplasty Ring in Pediatric Cardiac Surgery.

Aortic stenosis and aortic insufficiency (AI) are common valvular conditions that may necessitate repair or replacement of the aortic valve. Aortic valve replacement is associated with higher long-term complications and thus, a consistent, reliable method of repair is needed. This is especially true in the pediatric population where lifelong anticoagulation and development of recurrent aortic stenosisor aortic insufficiency are especially problematic. The Hemispherical Aortic Annuloplasty Reconstruction Technology ring has been developed and used for annular stabilization in adults with success, though its efficacy in the pediatric population has yet to be demonstrated. Herein, we discuss the use of a geometric ring in aortic valve repair for the pediatric patient.

Right Anterior Mini-Incision Approach to Anomalous Right Coronary Artery Repair.

The right anterior mini-incision has emerged as an effective minimally invasive approach for adult aortic root and valve operations. However, adoption of minimally invasive techniques has been limited in congenital heart surgery. We report a case of anomalous aortic origin of the right coronary artery repair performed through this approach. Following successful right coronary artery unroofing, the patient had an uncomplicated postoperative hospitalization.

Intervention for arch obstruction after the Norwood procedure: Prevalence, associated factors, and practice variability.

OBJECTIVE: Arch obstruction after the Norwood procedure is common and contributes to mortality. We determined the prevalence, associated factors, and practice variability of arch reintervention and assessed whether arch reintervention is associated with mortality. METHODS: From 2005 to 2017, 593 neonates in the Congenital Heart Surgeons' Society Critical Left Heart Obstruction cohort underwent a Norwood procedure. Median follow-up was 3.7 years. Multivariable parametric models, including a modulated renewal analysis, were performed. RESULTS: Of the 593 neonates, 146 (25%) underwent 218 reinterventions for arch obstruction after the Norwood procedure: catheter-based (n = 168) or surgical (n = 50) at a median age of 4.3 months (quartile 1-quartile 3, 2.6-5.7). Interdigitation of the distal aortic anastomosis was protective against arch reintervention. Development of ≥ moderate tricuspid valve regurgitation and right ventricular dysfunction at any point was associated with arch reintervention. Nonsignificant variables for arch reintervention included shunt type and preoperative aortic measurements. Surgical arch reintervention was protective against arch reintervention, but transcatheter reintervention was associated with increased reintervention. Arch reintervention was not associated with increased mortality. There was wide institutional variation in incidence of arch reintervention (range, 0-40 reinterventions per 100 years patient follow-up) and in preintervention gradient (range, 0-64 mm Hg). CONCLUSIONS: Interdigitation of the distal aortic anastomosis during the Norwood procedure decreased the risk of arch reintervention. Surgical arch reintervention is more definitive than transcatheter. Arch reintervention after the Norwood procedure is not associated with increased mortality. Serial surveillance for arch obstruction, integrated with changes in right ventricular function and tricuspid valve regurgitation, is recommended after the Norwood procedure to improve outcomes.