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In neonatal, symptomatic tetralogy of Fallot (sTOF), data are lacking on whether high-risk groups would benefit from staged (SR) or complete repair (CR). We studied the association of gestational age (GA) at birth and z-score for birth weight (BWz), with management strategy and outcomes in sTOF. California population-based cohort study (2011–2017) of infants with sTOF (defined as catheter or surgical intervention prior to 44 weeks corrected GA) was performed, comparing management strategy and timing by GA and BWz categories. Multivariable models evaluated composite outcomes and days alive and out of hospital (DAOOH) in the first year of life. Among 345 patients (SR = 194; CR = 151), management strategy did not differ by GA or BWz with complete repair defined as prior to 44 weeks corrected gestational age; however, did differ by GA with regard to complete/timely repair (defined as complete repair within first 30 days of life). Full-term and early-term neonates underwent CR 20 (95%CI: − 27.1, − 14.1; p < 0.001) and 15 days (95%CI: − 22.1, − 8.2; p < 0.001) sooner than preterm neonates. Prematurity and major anomaly were associated with mortality or non-cardiac morbidity, while only major anomaly was associated with mortality or cardiac morbidity (OR = 3.5, 95%CI: 1.8,6.7, p <.0001). Full-term infants had greater DAOOH compared to preterm infants (35.2 days, 95%CI: 4.0, 66.5, p = 0.03). LGA infants and those with major anomaly had significantly lower DAOOH. In sTOF, patient specific risk factors such as prematurity and major anomaly were more associated with outcomes than management strategy. [ABSTRACT FROM AUTHOR]

Background: There are conflicting reports regarding the importance of mitral stenosis and aortic atresia as a risk factor for Norwood mortality. This study reviews outcomes of this anatomic subgroup at our institution and examines the utility of preoperative cardiac catheterization and its correlation with clinical outcomes and pathology findings., Methods: This is a single-center, retrospective review of hypoplastic left heart syndrome patients who underwent modified Norwood operation between October 2005 and May 2013., Results: Fourteen of 74 hypoplastic left heart syndrome patients (19%) had mitral stenosis and aortic atresia. Operative mortality for MS/AA was 29% versus 7% for all other hypoplastic left heart syndrome anatomic subgroups (p = 0.04). Although only 19% of the entire cohort, the mitral stenosis and aortic atresia subgroup constituted 50% of the total operative mortality and the only interstage deaths. Autopsies support myocardial ischemia as the mechanism of death. Although preoperative angiography defined the presence of ventriculo-coronary connections, it did not clearly risk stratify patients in regard to operative mortality., Conclusions: Mitral stenosis and aortic atresia is a risk factor for perioperative myocardial ischemia and mortality. Further exploration of myocardial reserve is warranted., (Copyright © 2016 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Complete atrioventricular block (AVB) is a recognized complication of ventricular septal defect (VSD) repair. The objective of this study was to examine incidence and risk factors., Methods: This is a single-center, retrospective study. All pediatric patients (age <18 years) who underwent VSD repair between November 2001 and August 2009 with concordant atrioventricular and ventriculoarterial connections were included. Patients were classified as having no AVB or transient AVB, and outcomes of early pacemaker placement (before hospital discharge) or late pacemaker placement (after hospital discharge) were recorded., Results: Eight hundred twenty-eight patients (48.6% female) underwent VSD repair during the study period. Mean age at repair was 14 ± 29.4 months. A total of 64 patients (7.7%) developed surgical AVB. Among those patients who developed AVB, 48 (75%) had transient AVB. Sixteen patients (1.9%) required a pacemaker, 14 early (88%) and 2 late (12%). Patients <4 kg (4.2% vs. 1%, P ≤ .01) and those with inlet VSDs (11.6% vs. 1.4%, P ≤ .01) were more likely to develop surgical AVB. Surgical AVB was not influenced by the presence of chromosomal abnormalities or other congenital heart disease., Conclusion: The overall incidence of surgical AVB is consistent with previous reports. Weight <4 kg and presence of an inlet VSD were significant risk factors., (© 2013 Wiley Periodicals, Inc.)

Objective: The objectives of this study were (1) to determine the use of temporary epicardial pacing wires to diagnose and treat early postoperative arrhythmias in pediatric cardiac surgical patients and (2) to determine the predictive factors for the need of pacing wires for diagnostic or therapeutic purposes., Methods: We collected preoperative, intraoperative, and postoperative data in a prospective, observational format from patients undergoing pediatric cardiac surgery between August 2010 and January 2011 at a single academic children's hospital., Results: A total of 157 patients met the inclusion criteria during the study period. Of these 157 patients, pacing wires were placed in 127 (81%). Pacing wires were used in 25 patients (19.6%) for diagnostic purposes, 26 patients (20.4%) for therapeutic purposes, 15 patients (11.8%) for both diagnostic and therapeutic purposes, and 36 patients (28.3%) for diagnostic or therapeutic purposes. Need for cardioversion in the operating room, presence of 2 or more intracardiac catheters, severely reduced ventricular ejection fraction, and elevated serum lactate level at the time of operating room discharge were found to be independent predictors for the use of pacing wires. The only complication noted in the cohort was a skin infection at a pacing wire insertion site in 1 patient. A permanent pacemaker was required in 8 (6.2%) of all patients with temporary pacing wires., Conclusions: Our data support the use of temporary epicardial pacing wires in approximately 30% of children after congenital heart surgery. We found the need for cardioversion in the operating room, presence of 2 or more intracardiac catheters, severely reduced ventricular ejection fraction, and high serum lactate level at the time of discharge from the operating room to be independent predictors of the use of pacing wires in the early postoperative period., (Copyright © 2012 The American Association for Thoracic Surgery. Published by Mosby, Inc. All rights reserved.)

Objective: To determine presentation, clinical course, and outcome of a cohort of pregnant women with coccidioidomycosis and compare findings with common observations reported in the literature., Methods: Thirty-two women who delivered live infants or aborted fetuses in 1993 and had confirmed diagnoses of coccidioidomycosis were included in the study. Medical records were evaluated retrospectively for clinical characteristics, laboratory results, and disease course., Results: Dissemination occurred in three of 32 cases. The most common management was supportive and symptomatic care. At 1 year, 26 of 32 had recovered. There were no maternal deaths., Conclusion: The common depiction of coccidioidomycosis in pregnancy has overstated morbidity and mortality likely because of reporting bias. Many women will have favorable outcomes without drug treatment, and the practice of abortions or early delivery in subjects with active infection should be rare.

Large-restriction-fragment pattern comparison of Mycobacterium avium from 85 blood, stool, and respiratory specimens from 25 human immunodeficiency virus-infected San Francisco patients revealed 4 strains that infected multiple people (3 groups of 2 patients and 1 group of 3 patients). Most patients harbored a single M. avium strain, but 2 strains were recovered from 8 patients. The significance of recovering 2 strains is not clear, since the second strain was seldom recovered more than once. The strain recovered from blood was recovered from stool of 4 patients and respiratory secretions of 6 patients >4 weeks before detection of bacteremia, indicating that the intestinal and respiratory tracts are entry portals from which M. avium can disseminate. M. avium from 21 cities outside of California served as controls. Thus, a single M. avium strain can cause disseminated infection in multiple patients. This may represent infection from a common environmental source or person-to-person spread.