Days alive and out of hospital for children born with single-ventricle heart disease.

BACKGROUND: This study describes the illness burden in the first year of life for children with single-ventricle heart disease, using the metric of days alive and out of hospital to characterize morbidity and mortality. METHODS: This is a retrospective single-centre study of single-ventricle patients born between 2005 and 2021 who had their initial operation performed at our institution. Patient demographics, anatomical details, and hospitalizations were extracted from our institutional single-ventricle database. Days alive and out of hospital were calculated by subtracting the number of days hospitalized from number of days alive during the first year of life. A multivariable linear regression with stepwise variable selection was used to determine independent risk factors associated with fewer days alive and out of hospital. RESULTS: In total, 437 patients were included. Overall median number of days alive and out of hospital in the first year of life for single-ventricle patients was 278 days (interquartile range 157-319 days). In a multivariable analysis, low birth weight (<2.5kg) (b = -37.55, p = 0.01), presence of a dominant right ventricle (b = -31.05, p = 0.01), moderate-severe dominant atrioventricular valve regurgitation at birth (b = -37.65, p < 0.05), index hybrid Norwood operation (b = -138.73, p < 0.01), or index heart transplant (b = -158.41, p < 0.01) were all independently associated with fewer days alive and out of hospital. CONCLUSIONS: Children with single-ventricle heart defects have significant illness burden in the first year of life. Identifying risk factors associated with fewer days alive and out of hospital may aid in counselling families regarding expectations and patient prognosis.

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.

Use of microaxial flow pumps in adolescents.

Objectives: The Impella 5.5 has been successfully used in the adult population; however, safety and efficacy data in patients aged less than 18 years are limited. Methods: Six pediatric patients, aged 13 to 16 years and weighing 45 to 113 kg, underwent axillary artery graft placement and attempted placement of the Impella 5.5 device at our institution between August 2020 and March 2023. Results: Indications for implantation were heart failure secondary to myocarditis (2), rejection of prior orthotopic heart transplant, idiopathic dilated cardiomyopathy (2), and heart failure after transposition of the great arteries repair. Placement was unsuccessful in a 13.8-year-old female patient due to prohibitively acute angulation of the right subclavian artery, and venoarterial extracorporeal membrane oxygenation cannulation was performed via the axillary graft. In 5 patients with successful Impella 5.5 placement, median duration of support was 13.5 days (range, 7-42 days). One experienced cardiac arrest secondary to coagulation-associated device failure, requiring temporary HeartMate3 implantation. Four patients were bridged to transplant; 3 patients received a transplant directly from Impella 5.5, and 1 patient received a transplant after HeartMate3. The final patient received the HeartMate3 on Impella day 42 and is awaiting transplant. Conclusions: Although exact size cutoffs and anatomy are still being determined, our experience provides a framework for use of the Impella 5.5 in adolescents.

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.

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.

Understanding and Managing Direct Operating Room Supply Costs in Cardiac Surgery.

BACKGROUND: Value-based bundles require surgeons to understand their costs. Current approaches to cost reporting are confusing and difficult to reproduce. Using the Epic surgical receipt function, we describe an intuitive and systematic approach for evaluating financial data within the operating room. METHODS: We conducted a retrospective review of all congenital cardiac procedures performed at a single academic medical center between January 1, 2020, and January 1, 2021. Direct operating room supply costs were obtained using the Epic surgical receipt function. Costs were analyzed on the basis of contribution to total annual cost and variability in case cost. Implications for strategies identified within congenital cardiac surgery were then evaluated in adult cardiac surgery. RESULTS: Five procedures representing 71 patients accounted for more than 50% of the total direct operating room supply costs (left ventricular assist device, Norwood procedure, pulmonary valve replacement, right ventricle-to-pulmonary artery shunt, and aortic arch augmentation). Disposable vascular clips, suture brand preference, and surgical patch materials accounted for 3.7%, 6.6%, and 26.5% of annual direct operating room supply costs, respectively. Improvements to these categories would represent 12% to 14% ($250 000) in annual savings without an anticipated effect on outcomes. Across adult and congenital cardiac surgery, 95% of all name-brand suture use was tied to preference cards. An opt-in vs default approach to name-brand polypropylene suture could save more than $250 000 annually. CONCLUSIONS: The surgical receipt represents a reliable and intuitive way for reporting surgical costs. Systematically analyzing costs and their impact on outcomes will help surgeons improve the value of care they provide.

Single stage biventricular repair of hypoplastic right ventricle with straddling tricuspid valve.

We present the case of a five-year-old child with an inlet ventricular septal defect, subpulmonic stenosis, hypoplastic right ventricle, and straddling tricuspid valve who received a successful one-stage biventricular repair with right ventricular rehabilitation, right ventricular outflow tract augmentation, papillary muscle transposition, ventricular septal defect closure, and fenestrated atrial septation. This report outlines the surgical decision making and operative technique.

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.

Minimally Invasive Approach for Cardiac Hemangioma Resection in a Teenager.

Cardiac hemangiomas are a rare tumor traditionally resected by median sternotomy. We performed a minimally invasive right ventricular cardiac hemangioma resection via a left anterior mini-incision (LAMI). The procedure was without complication, and the patient was discharged on postoperative day 2. The LAMI has been used broadly by our team for operations involving the right ventricular outflow tract, as an alternative to median sternotomy. Here we show that it can also be used for the resection of a cardiac tumor.

BRG1 is a biomarker of hypertrophic cardiomyopathy in human heart specimens.

Hypertrophic cardiomyopathy (HCM) is a genetic disease of the sarcomere that causes otherwise unexplained cardiac hypertrophy and is associated with sudden death. While previous studies showed the role of the epigenetic modifier Brg1 in mouse models of HCM, additional work is needed to identify its role in humans. We tested the hypothesis that BRG1 expression is increased in periods of cardiac remodeling during fetal growth and in development of HCM. We employed immunohistochemical staining to evaluate protein expression of BRG1 in 796 human cardiac specimens (81 from patients with HCM) and describe elevated BRG1 expression in human fetal hearts in early development. In addition, we not only demonstrate increased expression of BRG1 in HCM, but we also show that other diseases that lead to heart failure have similar BRG1 expression to healthy controls. Inhibition of BRG1 in human induced pluripotent stem cell-derived cardiomyocytes significantly decreases MYH7 and increases MYH6, suggesting a regulatory role for BRG1 in the pathological imbalance of the two myosin heavy chain isoforms in human HCM. These data are the first demonstration of BRG1 as a specific biomarker for human HCM and provide foundation for future studies of epigenetics in human cardiac disease.