Outcomes of Neonates Born with Symptomatic Tetralogy of Fallot and Absent Ductus Arteriosus.

OBJECTIVE: To test the hypothesis that neonates with symptomatic tetralogy of Fallot (TOF) and absent ductus arteriosus (ADA) have worse clinical outcomes compared with those with a ductus arteriosus (DA), and that this difference is driven by those born with ADA and with critically deficient pulmonary blood flow (CDPBF). STUDY DESIGN: We performed a retrospective, multicenter cohort study of neonates who underwent intervention for symptomatic TOF comparing death and reintervention between subjects with and without a DA identified on fetal echocardiogram or on echocardiogram performed in the first postnatal day. Exclusion criteria were as follows: inability to define DA status, collaterals supplying pulmonary blood flow, atrioventricular septal defect, and absent pulmonary valve. We defined CDPBF as undergoing a procedure to augment pulmonary blood flow on the date of birth or extracorporeal membrane oxygenation prior to such a procedure. RESULTS: The study cohort included 519 patients, among whom 11% had ADA. Patients with ADA were more likely to have a genetic syndrome and had smaller branch pulmonary artery size. In analyses adjusting for center, interventional treatment strategy, genetic syndrome, and minimum branch pulmonary artery size, ADA was associated with higher mortality risk (adjusted hazard ratio of 2.37 (95% CI: 1.07,5.27; P = .034). Seven patients had CDPBF (1.3% of the entire cohort and 12% of patients with ADA). CONCLUSIONS: A minority of symptomatic TOF neonates have ADA, which is associated with higher adjusted mortality risk compared with those with a DA. CDPBF appears to be a rare but important entity in this population.

Impact of Management Strategy on Feeding and Somatic Growth in Neonates with Symptomatic Tetralogy of Fallot: Results from the Congenital Cardiac Research Collaborative.

OBJECTIVE: To evaluate early growth following primary or staged repair of neonatal symptomatic tetralogy of Fallot (sTOF). STUDY DESIGN: We performed a retrospective, multicenter cohort study of consecutive infants with sTOF who underwent initial intervention at age ≤30 days, from 2005 to 2017. Management strategies were either primary repair or staged repair (ie, initial palliation followed by complete repair). The primary outcome was change in weight-for-age z-score (ΔWAZ) from the initial intervention to age 6 ± 2 months. Secondary outcomes included method and mode of feeding, feeding-related medications, and feeding-related readmissions. Propensity score adjustment was used to account for baseline differences between groups. A secondary analysis was performed comparing patients stratified by the presence of adequate growth (6-month ΔWAZ > -0.5) or inadequate growth (6-month ΔWAZ ≤ -0.5), independent of treatment strategy. RESULTS: The study cohort included 143 primary repair subjects and 240 staged repair subjects. Prematurity was more common in the staged repair group. After adjustment, median ΔWAZ did not differ between treatment groups over the first 6 months of life (primary: -0.43 [IQR, -1.17 to 0.50]; staged: -0.31 [IQR, -1.31 to 0.71]; P = .55). For the entire cohort, ΔWAZ was negative (-0.36; IQR, -1.21 to 0.63). There were no between-group differences in the secondary outcomes. Secondary analysis revealed that the subjects with adequate growth were more likely to be orally fed at initial hospital discharge (P = .04). CONCLUSIONS: In neonates with sTOF, growth trajectory over the first 6 months of life was substandard, irrespective of treatment strategy. Those patients with adequate growth were more likely to be discharged from the index procedure on oral feeds.

Tissue Plasminogen Activator Use in Children: Bleeding Complications and Thrombus Resolution.

OBJECTIVE: To review our institutional experience with tissue plasminogen activator (tPA) to determine outcomes related to bleeding complications and thrombus resolution. STUDY DESIGN: We performed a retrospective review of all patients who received systemic tPA for thrombolysis. Data points included location of thrombus, initial and maximum tPA dose, and duration of tPA. The primary endpoint was bleeding complication. RESULTS: Between 2005 and 2014, 46 patients received systemic tPA for thrombolysis: 17 (37%) were patients with a primary cardiac diagnosis, there were 17 (37%) hematology/oncology patients, and 12 (26%) patients with noncardiac, nonhematology/oncology diagnoses. The indication for tPA was central venous thrombus (n = 23), pulmonary artery thrombus (n = 9), and cardiac or aortic thrombus (n = 14). Bleeding complications occurred in 15 patients (33%). Median initial tPA dose in the bleeding complication group was 0.10 mg/kg/h vs 0.03 mg/kg/h in the group without bleeding complication group (P = .01). Cardiac patients experienced more bleeding complications (P = .01). Multivariate analysis indicated that dose of tPA (P = .01) and diagnostic category (P < .01) were associated with bleeding complication. Complete thrombus resolution occurred in 21 patients, partial in 10 patients, and no resolution in 15 patients. Complete resolution of thrombus was not associated with diagnosis, thrombus location, tPA dose, or duration. CONCLUSIONS: Cardiac patients appear to be at highest risk of bleeding complication; bleeding complications were associated with higher doses of tPA, and cardiac patients were the cohort who received the highest doses of tPA. Higher tPA doses are associated with increased risk of bleeding complication but are not associated with successful thrombus resolution.

Coronary artery dilation in sickle cell disease.

OBJECTIVE: To evaluate the prevalence of coronary artery dilation in children with sickle cell disease (SCD). STUDY DESIGN: This is a retrospective analysis performed in patients, between 10 and 19 years old, with SCD who underwent a routine transthoracic echocardiographic evaluation over a 20-month period. The left main, left anterior descending, and proximal right coronary artery diameters, as well as clinical and laboratory variables and other echocardiographic results were collected. Echocardiographic measurements were converted to z scores by using information from a large control population of normal children. Coronary artery ectasia (CAE) was defined as a coronary artery diameter z score ≥ 2. The patients with CAE were compared with those without CAE by using univariate and multivariate analyses. RESULTS: Seventeen of 96 patients with SCD (17.7%) had CAE. There were no differences in sex, age, height, weight, body surface area, or genotype between those with and those without CAE. Patients with CAE had larger left ventricular end-diastolic dimension, shortening fraction, septal thickness, posterior wall thickness, mass, mass-to-volume ratio, and white blood cell count. Multivariate analysis revealed that the mass-to-volume ratio and elevated white blood cell count were associated with CAE. CONCLUSION: CAE is common in SCD and is associated with left ventricular hypertrophy and inflammation.