Multicenter Analysis of Truncal Valve Management and Outcomes in Children with Truncus Arteriosus.

Truncal valve management in patients with truncus arteriosus is a clinical challenge, and indications for truncal valve intervention have not been defined. We sought to evaluate truncal valve dysfunction and primary valve intervention in patients with truncus arteriosus and determine risk factors for later truncal valve intervention. We conducted a retrospective cohort study of children who underwent truncus arteriosus repair at 15 centers between 2009 and 2016. Multivariable competing risk analysis was performed to determine risk factors for later truncal valve intervention. We reviewed 252 patients. Forty-two patients (17%) underwent truncal valve intervention during their initial surgery. Postoperative extracorporeal support, CPR, and operative mortality for patients who underwent truncal valve interventions were statistically similar to the rest of the cohort. Truncal valve interventions were performed in 5 of 64 patients with mild insufficiency; 5 of 16 patients with mild-to-moderate insufficiency; 17 of 35 patients with moderate insufficiency; 5 of 9 patients with moderate-to-severe insufficiency; and all 10 patients with severe insufficiency. Twenty patients (8%) underwent later truncal valve intervention, five of whom had no truncal valve intervention during initial surgical repair. Multivariable analysis revealed truncal valve intervention during initial repair (HR 11.5; 95% CI 2.5, 53.2) and moderate or greater truncal insufficiency prior to initial repair (HR 4.0; 95% CI 1.1, 14.5) to be independently associated with later truncal valve intervention. In conclusion, in a multicenter cohort of children with truncus arteriosus, 17% had truncal valve intervention during initial surgical repair. For patients in whom variable truncal valve insufficiency is present and primary intervention was not performed, late interventions were uncommon. Conservative surgical approach to truncal valve management may be justifiable.

Outcomes Following Norwood Procedures: Analysis of a "Small Volume" Program.

BACKGROUND: Institutional survival following Norwood procedures is traditionally correlated with a center's surgical volume. Multiple single and multi-institutional studies conducted at large-volume centers have recently demonstrated improved survival following Norwood procedures. We report both short- and long-term outcomes at a single, small-volume institution and comment on factors potentially influencing outcomes at this institution. METHODS: All patients undergoing Norwood procedures from January 1, 2005, to January 1, 2020, at our institution were included in this study. Kaplan-Meier survival and Cox regression risk factor analyses were performed in addition to first interstage risk factor scoring to compare observed versus expected survival. RESULTS: The cohort included 113 patients. Kaplan-Meier freedom from death or transplant was 88%, 80%, and 76% at 1, 5, and 10 years, respectively. Freedom from death following hospital discharge after Norwood procedures was 94%, 87%, and 83% at 1, 5, and 10 years, respectively. The presence of genetic syndromes was a significant risk factor for mortality. First interstage observed-to-expected mortality following discharge was 0.57 (P = .04). Postoperative length of stay was comparable to that reported for the period 2015 to 2018 in the Society of Thoracic Surgeons Database. CONCLUSIONS: Survival outcomes at this single, small-volume institution were similar to those reported by large-volume centers and multi-institutional collaborative studies. These results may be related to structural and functional features that have been demonstrated to influence outcomes in other studies. These factors are achievable by small-volume programs with sufficient resource allocation.

Utilization of inhaled nitric oxide after surgical repair of truncus arteriosus: A multicenter analysis.

BACKGROUND: Elevated pulmonary vascular resistance (PVR) is common following repair of truncus arteriosus. Inhaled nitric oxide (iNO) is an effective yet costly therapy that is frequently implemented postoperatively to manage elevated PVR. OBJECTIVES: We aimed to describe practice patterns of iNO use in a multicenter cohort of patients who underwent repair of truncus arteriosus, a lesion in which recovery is often complicated by elevated PVR. We also sought to identify patient and center factors that were more commonly associated with the use of iNO in the postoperative period. DESIGN: Retrospective cohort study. SETTING: 15 tertiary care pediatric referral centers. PATIENTS: All infants who underwent definitive repair of truncus arteriosus without aortic arch obstruction between 2009 and 2016. INTERVENTIONS: Descriptive statistics were used to demonstrate practice patterns of iNO use. Bivariate comparisons of characteristics of patients who did and did not receive iNO were performed, followed by multivariable mixed logistic regression analysis using backward elimination to identify independent predictors of iNO use. MAIN RESULTS: We reviewed 216 patients who met inclusion criteria, of which 102 (46%) received iNO in the postoperative period: 69 (68%) had iNO started in the operating room and 33 (32%) had iNO initiated in the ICU. Median duration of iNO use was 4 days (range: 1-21 days). In multivariable mixed logistic regression analysis, use of deep hypothermic circulatory arrest (odds ratio: 3.2; 95% confidence interval: 1.2, 8.4) and center (analyzed as a random effect, p = .02) were independently associated with iNO use. CONCLUSIONS: In this contemporary multicenter study, nearly half of patients who underwent repair of truncus arteriosus received iNO postoperatively. Use of iNO was more dependent on individual center practice rather than patient characteristics. The study suggests a need for collaborative quality initiatives to determine optimal criteria for utilization of this important but expensive therapy.

Multicenter Analysis of Early Childhood Outcomes After Repair of Truncus Arteriosus.

BACKGROUND: Literature describing morbidity and mortality after truncus arteriosus repair is predominated by single-center reports. We created and analyzed a multicenter dataset to identify risk factors for late mortality and right ventricle-to-pulmonary artery (RV-PA) conduit reintervention for this patient population. METHODS: We retrospectively collected data on children who underwent repair of truncus arteriosus without concomitant arch obstruction at 15 centers between 2009 and 2016. Cox regression survival analysis was conducted to determine risk factors for late mortality, defined as death occurring after hospital discharge and greater than 30 days after operation. Probability of any RV-PA conduit reintervention was analyzed over time using Fine-Gray modeling. RESULTS: We reviewed 216 patients with median follow-up of 2.9 years (range, 0.1 to 8.8). Operative mortality occurred in 15 patients (7%). Of the 201 survivors there were 14 (7%) late deaths. DiGeorge syndrome (hazard ratio [HR], 5.4; 95% confidence interval [CI], 1.6 to 17.8) and need for postoperative tracheostomy (HR, 5.9; 95% CI, 1.8 to 19.4) were identified as independent risk factors for late mortality. At least one RV-PA conduit catheterization or surgical reintervention was performed in 109 patients (median time to reintervention, 23 months; range, 0.3 to 93). Risk factors for reintervention included use of pulmonary or aortic homografts versus Contegra (Medtronic, Inc, Minneapolis, MN) bovine jugular vein conduits (HR, 1.9; 95% CI, 1.2 to 3.1) and smaller conduit size (HR per mm/m2, 1.05; 95% CI, 1.03 to 1.08). CONCLUSIONS: In a multicenter dataset DiGeorge syndrome and need for tracheostomy postoperatively were found to be independent risk factors for late mortality after repair of truncus arteriosus, whereas risk of conduit reintervention was independently influenced by both initial conduit type and size.

Septal Leaflet Versus Chordal Detachment in Closure of Hard-to-Expose Ventricular Septal Defects.

BACKGROUND: Different techniques have been used for exposure of ventricular septal defect (VSD) margins when there is crowding of the VSD anatomy by tricuspid valve subvalvar apparatus. This study compared surgical outcomes for the two techniques of tricuspid valve leaflet detachment and the rarely described tricuspid valve chordal detachment for hard-to-expose VSDs. METHODS: Patients undergoing transatrial VSD repair were identified from our institutional database. Follow-up echocardiography and patient data were obtained from medical records. Between January 2005 and August 2016, 130 isolated conoventricular VSDs were repaired. Among these, 26 patients underwent leaflet detachment, 15 underwent chordal detachment, and 89 underwent regular VSD repair (reference group). RESULTS: The groups did not differ significantly in age, weight, postoperative length of stay, genetic/syndromic abnormalities, time to extubation, and left and right ventricular systolic function. The cardiopulmonary bypass and cross-clamp time were significantly longer in the leaflet detachment group than in the reference group (118 ± 28 vs 102 ± 32 minutes [p = 0.02] and 73 ± 20 vs 61 ± 23 minutes [p = 0.01], respectively). Echocardiographic follow-up was available for 87 patients at a mean of 2.6 years (range, 1 month to 11 years). Tricuspid regurgitation was rated as none or trivial in 66 (76%), mild in 20 (23%), and moderate in 1 reference group patient. There was no difference in presence of residual VSD or degree of tricuspid regurgitation among the three groups. There was no reoperation for tricuspid regurgitation. CONCLUSIONS: Tricuspid valve leaflet and chordal detachment techniques provide an equally viable and safe alternative to closure of hard-to-expose VSDs while maintaining appropriate tricuspid valve function. Their use in our series did not lead to increased tricuspid valve dysfunction at early-to-midterm echocardiographic assessment.

Transcatheter Patent Ductus Arteriosus Occlusion in Small Infants.

BACKGROUND: Transcatheter patent ductus arteriosus (PDA) occlusion is feasible in small infants and may improve lung function in symptomatic patients. We aimed to describe transcatheter PDA closure in small infants including predictors of technical success and rate of complication and to identify factors associated with improved respiratory status after closure. METHODS: All patients in the NICU at our center who were referred for transcatheter PDA occlusion between 1/2010 and 11/2014 were retrospectively identified. Relevant details were extracted. Additionally, a modification of the respiratory severity score (RSS) (FiO2 × mean airway pressure) was used to characterize degree of pulmonary support before and at intervals after catheterization. RESULTS: Twenty patients were identified with median age of 96 days (13-247) and weight of 3.1 kg (1.7-4.7). The PDA was type F morphology in 14 (70%) patients. The PDA was successfully occluded in 16 (80%) patients. Ratio of minimum PDA diameter/length was >0.5 in all unsuccessful attempts and <0.4 in all successful cases (P = .01). Of the 16 cases of occlusion, Amplatzer Vascular Plug II was used in 15 (94%). No deaths or pulse loss occurred. Five (25%) patients required blood transfusion and transfusion was associated with lower hemoglobin (P = .049), lower weight (P = .008), and lower aortic pressure (P = .04). Excluding 1 patient with significant congenital heart disease, the RSS improved at 3 days in 9 (60%) patients and at 7 days in 12 (80%) compared with preintervention value. Patient factors were not associated with improved RSS at 3 or 7 days. CONCLUSIONS: In our cohort of symptomatic infants, transcatheter PDA occlusion was successful in most and a ratio of minimum PDA diameter/length of <0.4 was predictive of technical success. Using a surrogate for pulmonary support, the majority of patients were on less support 7 days after closure.

Culture Negative Stent Infection in an Infant with Hypoplastic Left Heart and Persistent Fever.

We present an infant with hypoplastic left heart with persistent fever despite two courses of antibiotics and repeatedly negative blood cultures. He eventually underwent surgical extraction of two stents. The stent cultures became positive; he was treated with 4 weeks of antibiotics and the fever resolved.

Usefulness of urinary immune biomarkers in the evaluation of neonatal sepsis: a pilot project.

OBJECTIVE: Our hypothesis is that specific proinflammatory and anti-inflammatory urinary cytokines are useful in the diagnostic evaluation of risk for sepsis in term neonates. We conducted a pilot, prospective hospital-based longitudinal observational study to test the urine of term neonates with a 13 biomarker panel of cytokines. METHODS: Infants were divided into 2 groups: The control group (n = 15) consisted of infants admitted to newborn nursery, and the test group (n = 15) consisted of infants admitted to the neonatal intensive care unit for presumed sepsis. Bagged urine samples were collected from 30 term neonates for testing our hypothesis. RESULTS: Urinary interleukin (IL)-8 (P = .004*), inducible protein (IP)-10 (P = .007*), and monocyte chemoattractant protein (MCP)-1 (P = .02) were significantly increased in the test group compared with the control group. CONCLUSIONS: Urinary IL-8, IP-10, and MCP-1 are proinflammatory cytokines that are increased in the neonate during an infectious inflammatory process. These may be useful predictors as an adjunct to the current protocols to recognize neonatal sepsis.