Minimally invasive cytoreductive radical prostatectomy, exploring the safety and feasibility of a single-port or multi-port robotic platform.

BACKGROUND: Consolidative resection or cytoreductive radical prostatectomy (CRP) may benefit men with non-organ confined prostate cancer. We report the safety, feasibility, and outcomes of robot-assisted laparoscopic CRP using a single-port (SP) or multi-port (MP) platform. METHODS: We reviewed consecutive men with clinical node positive or metastatic castrate-sensitive prostate cancer who underwent IRB-approved CRP and extended pelvic lymph node dissection using the da Vinci SP or MP Surgical Systems (Intuitive Surgical, Sunnyvale, CA) from 2015-2022. Perioperative data and Clavien-Dindo 90-day complications were recorded. RESULTS: Twenty-four men with a median age of 61 (IQR 56-69) years and prostate-specific antigen of 32.1 (IQR 21.9-62.3) ng/mL were included. Clinical N1, M1, or N1 + M1 disease were detected in 8 (33%), 9 (38%), 7 (29%) patients, respectively. There was no difference in positive margins, 41% vs. 29% (P = 0.67), lymph node yield, 21 (IQR 14-28) vs. 20 (IQR 13.5-21) nodes (P = 0.31), or estimated blood loss, 150 mL (IQR 100-200) vs. 50 mL (IQR 50-125) (P = 0.06), between the MP and SP cohorts, respectively. Hospital length of stay was significantly shorter for the SP group, same-day discharge (IQR 0-0), compared to MP, 1-day (IQR 1-1), P < 0.001. One grade III bowel obstruction and lymphocele occurred in the MP cohort. No major complications occurred in the SP cohort. CONCLUSION: Robot-assisted laparoscopic CRP is safe and feasible for select men with advanced castrate-sensitive prostate cancer.

Chronic kidney disease and outcomes in hospitalized sickle cell disease patients: A National Inpatient Sample analysis.

OBJECTIVES: To examine the association between chronic kidney disease (CKD) and outcomes in sickle cell disease (SCD) patients. METHODS: Patients ≥18 years with SCD in the National Inpatient Sample database between 2016 and 2018 were identified using ICD-10-CM diagnosis codes. A 1:1 propensity-score matched analysis was performed to compare in-hospital outcomes of patients with and without CKD. RESULTS: Of the 366 240 SCD admissions, 19 365 (5.3%) had CKD. The CKD group had higher odds of in-hospital mortality (odds ratio [OR] 2.59, 95% confidence interval [CI]: 1.63-4.12, p = <.01), blood transfusion (OR 1.67, 95% CI: 1.47-1.90, p < .01), mechanical ventilation (OR 2.20, 95% CI: 1.56-3.12, p = <.01), sepsis (OR 1.75, 95% CI: 1.46-2.10, p < .01), incurred higher costs ($53 255 vs. $47 294, p < .001), but had lower odds of acute chest syndrome (OR 0.71, 95% CI: 0.54-0.95, p = 0.02) and pulmonary embolism (OR 0.45, 95% CI: 0.31-0.67, p < .01). CONCLUSIONS: CKD was associated with higher mortality, higher costs, blood transfusion, sepsis, and mechanical ventilation in SCD patients. Further studies are needed to explore the reasons for the reduced odds of pulmonary embolism and acute chest syndrome.

Effect of patient factors, center, and era on Fontan timing: An observational study using the Pediatric Health Information Systems Database.

BACKGROUND: There are no consensus guidelines defining optimal timing for the Fontan operation, the last planned surgery in staged palliation for single-ventricle heart disease. OBJECTIVES: Identify patient-level characteristics, center-level variation, and secular trends driving Fontan timing. METHODS: A retrospective observational study of subjects who underwent Fontan from 2007 to 2021 at centers in the Pediatric Health Information Systems database was performed using linear mixed-effects modeling in which age at Fontan was regressed on patient characteristics and date of operation with center as random effect. RESULTS: We included 10,305 subjects (40.4% female, 44% non-white) at 47 centers. Median age at Fontan was 3.4 years (IQR 2.6-4.4). Hypoplastic left heart syndrome (-4.4 months, 95%CI -5.5 to -3.3) and concomitant conditions (-2.6 months, 95%CI -4.1 to -1.1) were associated with younger age at Fontan. Subjects with technology-dependence (+4.6 months, 95%CI 3.1-6.1) were older at Fontan. Black (+4.1 months, 95%CI 2.5-5.7) and Asian (+8.3 months, 95%CI 5.4-11.2) race were associated with older age at Fontan. There was significant variation in Fontan timing between centers. Center accounted for 10% of variation (ICC 0.10, 95%CI 0.07-0.14). Center surgical volume was not associated with Fontan timing (P = .21). Operation year was associated with age at Fontan, with a 3.1 month increase in age for every 5 years (+0.61 months, 95%CI 0.48-0.75). CONCLUSIONS: After adjusting for patient-level characteristics there remains significant inter-center variation in Fontan timing. Age at Fontan has increased. Future studies addressing optimal Fontan timing are warranted.

Long-term Outcomes and Patient Satisfaction Following Salvage Robot-assisted Radical Prostatectomy: A Modern Perspective.

Background and objective: Approximately two-thirds of men who undergo primary treatment for prostate cancer (PC) will experience biochemical recurrence (BCR). Salvage robot-assisted radical prostatectomy (sRARP) offers curative treatment in this disease setting and men who choose this option may avoid palliative androgen deprivation therapy (ADT). The purpose of this study was to describe long-term outcomes and patient feedback following sRARP. Methods: We reviewed data for consecutive men with biopsy-proven localized BCR who underwent sRARP and pelvic lymph node dissection at a single tertiary referral center between 2004 and 2021. Perioperative data, Clavien-Dindo complications, and functional outcomes were recorded. The Kaplan-Meier method was used to estimate prostate-specific antigen-free (≥0.2 ng/ml) survival (PSAFS) and metastasis-free survival (MFS). Three Likert-type items (score 1-5) from the validated Surgical Satisfaction Questionnaire-8 were distributed to patients postoperatively. Key findings and limitations: We included 78 men, of whom 72 (92%) had undergone primary radiotherapy and six (8%) had received primary prostate ablation. Median follow-up was 10.1 yr (interquartile range 5.8-12.4). Final pathology identified ≥pT3N0M0 in 35 patients (45%) and positive margins in 23 (29%). The overall complication rate was 50%. Of the 26 (33%) major (grade ≥III) complications, anastomotic stricture (32%) was most common. The estimated 3-, 5-, and 10-yr survival rates were 85.6% and 80.2%, 83.5% for PSAFS (n = 11), and 74.1%, 83.5%, and 70.5% for MFS (n = 23), respectively. At last follow-up, postoperative ADT had been administered to 17 patients (22%), and 39 men (50%) remained alive a decade after sRARP. Continence and potency were maintained in 33/62 (53%) and 1/16 (6%) patients, respectively. Thirty-five respondents (45%) reported median questionnaire scores (≥4) in favor of sRARP. Limitations include the small single-center series and a single query point for patient feedback. Conclusions and clinical implications: Long-term outcomes of sRARP suggest that the technical challenges and morbidity of the procedure are qualified by patient feedback and the opportunity to evade the morbidity and mortality of biochemically recurrent PC. Patient summary: We reviewed the cancer outcomes and side effects of robot-assisted surgical removal of the prostate after treatment failure with radiation or ablation for prostate cancer. We found that this type of treatment has substantial risks and long-term side effects, but the surgery provides an opportunity to cure prostate cancer and/or avoid the consequences of indefinite hormonal treatment. Overall, most men who underwent this surgery were not disappointed with their decision despite the higher risks and consequences.

Longitudinal echocardiographic parameters before and after pacemaker placement in congenital complete heart block.

BACKGROUND: Congenital complete heart block (CCHB) is seen in 1:15,000-1:20,000 live births, with risk of left ventricular (LV) dysfunction or dilated cardiomyopathy in 7%-23% of subjects. OBJECTIVE: The purpose of this study was to investigate serial changes in LV size and systolic function in paced CCHB subjects to examine the effect of time from pacemaker on echocardiographic parameters. METHODS: Single-center retrospective cohort analysis of paced CCHB subjects was performed. Echocardiographic data were collected before and after pacemaker placement. Linear mixed effect regression of left ventricular end-diastolic dimension (LVEDD) z-score, left ventricular shortening fraction (LVSF), and left ventricular ejection fraction (LVEF) was performed, with slopes compared before and after pacemaker placement. RESULTS: Of 114 CCHB subjects, 52 had echocardiographic data before and after pacemaker placement. Median age at CCHB diagnosis was 0.6 [interquartile range 0.0-3.5] years; age at pacemaker placement 3.4 [0.5-9.0] years; and pacing duration 10.8 [5.2-13.7] years. Estimated LVEDD z-score was 1.4 at pacemaker placement and decreased -0.08 per year (95% confidence interval [CI] -0.12 to -0.04; P = .002) to 0.2 (95% CI -0.3 to +0.3) 15 years postplacement. Estimated LVSF decreased -1.1% per year (95% CI -1.7% to -0.6%; P <.001) from 6 months prepacemaker placement to 34% (95% CI 32%-37%) 4 years postplacement. There was no significant change in LVSF between 4 and 15 years postplacement. Estimated LVEF did not change significantly after pacemaker placement, with estimated LVEF 59% (95% CI 55%-62%) 15 years postplacement. CONCLUSION: In 52 paced CCHB subjects, estimated LVEDD z-score decreased significantly after pacemaker placement, and estimated LVSF and LVEF remained within normal limits at 15 years postpacemaker placement.

Social determinants of health and lung cancer surgery: a qualitative study.

Introduction: Social determinants of health (SDOH) are non-clinical factors that may affect the outcomes of cancer patients. The purpose of this study was to describe the influence of SDOH factors on quality of life (QOL)-related outcomes for lung cancer surgery patients. Methods: Thirteen patients enrolled in a randomized trial of a dyadic self-management intervention were invited and agreed to participate in semi-structured key informant interviews at study completion (3 months post-discharge). A conventional content analysis approach was used to identify codes and themes that were derived from the interviews. Independent investigators coded the qualitative data, which were subsequently confirmed by a second group of independent investigators. Themes were finalized, and discrepancies were reviewed and resolved. Results: Six themes, each with several subthemes, emerged. Overall, most participants were knowledgeable about the concept of SDOH and perceived that provider awareness of SDOH information was important for the delivery of comprehensive care in surgery. Some participants described financial challenges during treatment that were exacerbated by their cancer diagnosis and resulted in stress and poor QOL. The perceived impact of education varied and included its importance in navigating the healthcare system, decision-making on health behaviors, and more economic mobility opportunities. Some participants experienced barriers to accessing healthcare due to insurance coverage, travel burden, and the fear of losing quality insurance coverage due to retirement. Neighborhood and built environment factors such as safety, air quality, access to green space, and other environmental factors were perceived as important to QOL. Social support through families/friends and spiritual/religious communities was perceived as important to postoperative recovery. Discussion: Among lung cancer surgery patients, SDOH factors can impact QOL and the patient's survivorship journey. Importantly, SDOH should be assessed routinely to identify patients with unmet needs across the five domains. SDOH-driven interventions are needed to address these unmet needs and to improve the QOL and quality of care for lung cancer surgery patients.

Association of Interstage Monitoring Era and Likelihood of Hemodynamic Compromise at Intervention for Recoarctation Following the Norwood Operation.

Background Intensive monitoring has been associated with a lower death rate between the Norwood operation and superior cavopulmonary connection, possibly due to early identification and effective treatment of residual anatomic lesions like recoarctation before lasting harm occurs. Methods and Results Neonates undergoing a Norwood operation and receiving interstage care at a single center between January 1, 2005, and September 18, 2020, were studied. In those with recoarctation, we evaluated association of era ([1] preinterstage monitoring, [2] a transitional phase, [3] current era) and likelihood of hemodynamic compromise (progression to moderate or greater ventricular dysfunction/atrioventricular valve regurgitation, initiation/escalation of vasoactive/respiratory support, cardiac arrest preceding catheterization, or interstage death with recoarctation on autopsy). We also analyzed whether era was associated with technical success of transcatheter recoarctation interventions, major adverse events, and transplant-free survival. A total of 483 subjects were studied, with 22% (n=106) treated for recoarctation during the interstage period. Number of catheterizations per Norwood increased (P=0.005) over the interstage eras, with no significant change in the proportion of subjects with recoarctation (P=0.36). In parallel, there was a lower likelihood of hemodynamic compromise in subjects with recoarctation that was not statistically significant (P=0.06), with a significant difference in the proportion with ventricular dysfunction at intervention (P=0.002). Rates of technical success, procedural major adverse events, and transplant-free survival did not differ (P>0.05). Conclusions Periods with interstage monitoring were associated with increased referral for catheterization but also reduced likelihood of ventricular dysfunction (and a suggestion of lower likelihood of hemodynamic compromise) in subjects with recoarctation. Further study is needed to guide optimal interstage care of this vulnerable population.

Assessment of fluid removal using ultrasound, bioimpedance and anthropometry in pediatric dialysis: a pilot study.

BACKGROUND: Fluid overload is associated with morbidity and mortality in children receiving dialysis. Accurate clinical assessment is difficult, and using deuterium oxide (D2O) to measure total body water (TBW) is impractical. We investigated the use of ultrasound (US), bioimpedance spectroscopy (BIS), and anthropometry to assess fluid removal in children receiving maintenance hemodialysis (HD). METHODS: Participants completed US, BIS, and anthropometry immediately before and 1-2 h after HD for up to five sessions. US measured inferior vena cava (IVC) diameter, lung B-lines, muscle elastography, and dermal thickness. BIS measured the volume of extracellular (ECF) and intracellular (ICF) fluid. Anthropometry included mid-upper arm, calf and ankle circumferences, and triceps skinfold thickness. D2O was performed once pre-HD. We assessed the change in study measures pre- versus post-HD, and the correlation of change in study measures with percent change in body weight (%∆BW). We also assessed the agreement between TBW measured by BIS and D2O. RESULTS: Eight participants aged 3.4-18.5 years were enrolled. Comparison of pre- and post-HD measures showed significant decrease in IVC diameters, lung B-lines, dermal thickness, BIS %ECF, mid-upper arm circumference, ankle, and calf circumference. Repeated measures correlation showed significant relationships between %∆BW and changes in BIS ECF (rrm =0.51, 95% CI 0.04, 0.80) and calf circumference (rrm=0.80, 95% CI 0.51, 0.92). BIS TBW correlated with D2O TBW but overestimated TBW by 2.2 L (95% LOA, -4.75 to 0.42). CONCLUSION: BIS and calf circumference may be helpful to assess changes in fluid status in children receiving maintenance HD. IVC diameter, lung B-lines and dermal thickness are potential candidates for future studies.

Child Abuse Pediatrics Research Network: The CAPNET Core Data Project.

OBJECTIVE: Examine the epidemiology of subspecialty physical abuse evaluations within CAPNET, a multicenter child abuse pediatrics research network. METHODS: We conducted a cross-sectional study of children <10 years old who underwent an evaluation (in-person or remote) by a child abuse pediatrician (CAP) due to concerns for physical abuse at ten CAPNET hospital systems from February 2021 through December 2021. RESULTS: Among 3667 patients with 3721 encounters, 69.4% were <3 years old; 44.3% <1 year old, 59.1% male; 27.1% Black; 57.8% White, 17.0% Hispanic; and 71.0 % had public insurance. The highest level of care was outpatient/emergency department in 60.7%, inpatient unit in 28.0% and intensive care in 11.4%. CAPs performed 79.1% in-person consultations and 20.9% remote consultations. Overall, the most frequent injuries were bruises (35.2%), fractures (29.0%), and traumatic brain injuries (TBI) (16.2%). Abdominal (1.2%) and spine injuries (1.6%) were uncommon. TBI was diagnosed in 30.6% of infants but only 8.4% of 1-year old children. In 68.2% of cases a report to child protective services (CPS) was made prior to CAP consultation; in 12.4% a report was made after CAP consultation. CAPs reported no concern for abuse in 43.0% of cases and mild/intermediate concern in 22.3%. Only 14.2% were categorized as definite abuse. CONCLUSION: Most children in CAPNET were <3 years old with bruises, fractures, or intracranial injuries. CPS reports were frequently made prior to CAP consultation. CAPs had a low level of concern for abuse in majority of cases.

Higher Hospital Frailty Risk Score Is an Independent Predictor of In-Hospital Mortality in Hospitalized Older Adults with Obstructive Sleep Apnea.

Background: Frailty predisposes individuals to stressors, increasing morbidity and mortality risk. Therefore, this study examined the impact of frailty defined by the Hospital Frailty Risk Score (HFRS) and other characteristics in older hospitalized patients with Obstructive Sleep Apnea (OSA). Methods: We conducted a retrospective study using the National Inpatient Sample 2016 in patients ≥65 years old with OSA. Logistic regression was used to evaluate the impact of frailty on inpatient mortality. A Kaplan-Meier curve with a log-rank test was used to estimate survival time between frailty groups. Results: 182,174 discharge records of elderly OSA were included in the study. 54% of the cohort were determined to be a medium/high frailty risk, according to HFRS. In multivariable analysis, frailty was associated with a fourfold (medium frailty, adjusted odd ratio (aOR): 4.12, 95% Confidence Interval (CI): 3.76−4.53, p-value < 0.001) and sixfold (high frailty, OR: 6.38, 95% CI: 5.60−7.27, p-value < 0.001) increased odds of mortality. Hospital survival time was significantly different between the three frailty groups (Log-rank test, p < 0.0001). Comorbidity burden defined by Charlson comorbidity Index (CCI) was associated with increased mortality (p < 0.001). Conclusion: More than half of the whole cohort was determined to be at medium and high frailty risk. Frailty was a significant predictor of in-hospital deaths in hospitalized OSA patients. Frailty assessment may be applicable for risk stratification of older hospitalized OSA patients.

Impact of Comorbidity Burden on Cardiac Implantable Electronic Devices Outcomes.

Background: There is limited data on the impact of comorbidity burden on clinical outcomes of patients undergoing cardiac implantable electronic devices (CIED) implantation. Objectives: Our aim was to assess trends in CIED implantations and explore the relationship between comorbidity burden and outcomes in patients undergoing de novo implantations. Methods: Using the National Inpatient Sample database from 2000 to 2014, we identified adults ⩾18 years undergoing de novo CIED procedures. Comorbidity burden was assessed by Charlson comorbidity Index (CCI), and patients were classified into 4 categories based on their CCI scores (CCI = 0, CCI = 1, CCI = 2, CCI ⩾3). Annual implantation trends were evaluated. Logistic regression was conducted to measure the association between categorized comorbidity burden and outcomes. Results: A total of 3 103 796 de-novo CIED discharge records were identified from the NIS database. About 22.4% had a CCI score of 0, 28.2% had a CCI score of 1, 22% had a CCI score of 2, and 27.4 % had a CCI score ⩾3. Annual de-novo CIED implantations peaked in 2006 and declined steadily from 2010 to 2014. Compared to CCI 0, CCI ⩾3 was independently associated with increased odds of in-hospital mortality, bleeding, pericardial, and cardiac complications (all P < .05). Length of stay and hospital charges increased with increasing comorbidity burden. Conclusions: CCI is a significant predictor of adverse outcomes after CIED implantation. Therefore, comorbidity burden needs to be considered in the decision-making process for CIED implant candidates.

Personal Health Practices and Perceptions of Lifestyle Counseling and Preventive Services Among Residents.

Background. Primary care residents are expected to provide lifestyle counseling and preventive services for patients with chronic diseases; also, physicians' personal lifestyle practice impacts patient care. The purpose of this article is to assess healthy lifestyle behaviors and attitudes to engage in lifestyle counseling and preventive services among residents and fellows in different training levels and specialty. Methods. A cross-sectional pilot study was conducted on medical residents and fellows (n = 57). Surveys collected information on lifestyle behaviors and perceptions of lifestyle counseling and preventive services. Comparisons of study measures were made across residents' specialty and training levels. Fisher's exact and analysis of variance tests were used for statistical analysis. Results. There were several significant differences in perceptions of counseling and screening by specialty and training level. There were no significant differences in personal lifestyle behaviors between all resident specialties and training levels. Conclusion. Our findings suggest that there are opportunities to improve healthy lifestyle behaviors and perceptions of lifestyle counseling and preventive services among residents in different specialties and training levels. This knowledge can inform development of training programs in lifestyle and preventive medicine practice during residency and fellowship.

Health Care Use of Cardiac Specialty Care in Children With Muscular Dystrophy in the United States.

Background Duchenne and Becker muscular dystrophy are progressive disorders associated with cardiac mortality. Guidelines recommend routine surveillance; we assess cardiac resource use and identify gaps in care delivery. Methods and Results Male patients, aged 1 to 18 years, with Duchenne and Becker muscular dystrophy between January 2013 and December 2017 were identified in the IBM MarketScan Research Database. The cohort was divided into <10 and 10 to 18 years of age. The primary outcome was rate of annual health care resource per person year. Resource use was assessed for place of service, cardiac testing, and medications. Adjusted incidence rate ratios (IRRs) were estimated using a Poisson regression model. Medication use was measured by proportion of days covered. There were 1386 patients with a median follow-up time of 3.0 years (interquartile range, 1.9-4.7 years). Patients in the 10 to 18 years group had only 0.40 (95% CI, 0.35-0.45) cardiology visits per person year and 0.66 (95% CI, 0.62-0.70) echocardiography/magnetic resonance imaging per person year. Older patients had higher rates of inpatient admissions (IRR, 1.46; 95% CI, 1.03-2.09), outpatient cardiology visits (IRR, 2.0; 95% CI, 1.66-2.40), cardiac imaging (IRR, 1.59; 95% CI, 1.40-1.80), and Holter monitoring (IRR, 3.33; 95% CI, 2.35-4.73). A proportion of days covered >80% for angiotensin-converting enzyme inhibitors/angiotensin receptor blockers was observed in 13.6% (419/3083) of total person years among patients in the 10 to 18 years group. Conclusions Children 10 to 18 years of age have higher rates of cardiac resource use compared with those <10 years of age. However, rates in both age groups fall short of guidelines. Opportunities exist to identify barriers to resource use and optimize cardiac care for patients with Duchenne and Becker muscular dystrophy.

Incidence of morbidity and mortality in a cohort of congenital complete heart block patients followed over 40 years.

BACKGROUND: Congenital complete heart block (CCHB) is seen in 1:15,000-20,000 births and commonly requires pacemaker placement by young adulthood. There is limited understanding of cardiac morbidity and mortality. OBJECTIVE: The purpose of this study was to determine the long-term incidence of cardiac morbidity and mortality in subjects with CCHB and identify associated risk factors. METHODS: Retrospective cohort analysis of subjects with CCHB at Children's Hospital of Philadelphia between 1976 and 2018 was performed. The primary outcome was a composite of death, left ventricular systolic dysfunction, heart failure, cardiomyopathy, or cardiac resynchronization therapy (CRT). Cox proportional hazard models assessed independent risk factors for the primary outcome and its components (death, heart failure and/or cardiomyopathy, CRT). RESULTS: One-hundred fourteen subjects (58% female; median age at last visit 15.2 years) were included. Eighty-eight (77%) underwent pacemaker implantation (median age at placement 1.9 years; interquartile range [IQR] 0.1-8.0 years). Twenty-six subjects (23%) reached the primary outcome; 7 (6%) died and 14 (12%) were diagnosed with heart failure and/or cardiomyopathy. Median time from diagnosis to primary outcome was 3.1 years (IQR 0.0-10.8 years). There were no significant associations between age at diagnosis <1 year (hazard ratio [HR] 1.5; 95% confidence interval [CI] 0.6-3.9), fetal diagnosis (HR 2.3; 95% CI 0.96-5.6), or maternal antibody positivity (HR 2.4; 95% CI 0.9-6.6) and the primary outcome. Fetal diagnosis had a higher associated hazard of heart failure and/or cardiomyopathy (HR 4.5; 95% CI 1.3-15.0). CONCLUSION: In 114 subjects with CCHB, 23% reached the composite outcome of cardiac morbidity and mortality, with no significant association between age at diagnosis, fetal diagnosis, and maternal antibody status with composite cardiac morbidity and mortality.

Incidence of life-threatening events in children with Wolff-Parkinson-White syndrome: Analysis of a large claims database.

BACKGROUND: Previous estimates of life-threatening event (LTE) risk in Wolff-Parkinson-White (WPW) syndrome are limited by selection bias inherent to tertiary care referral-based cohorts. OBJECTIVE: This analysis sought to measure LTE incidence in children with WPW syndrome in a large contemporary representative population. METHODS: A retrospective cohort study was conducted using claims data from the IBM MarketScan Research Databases, evaluating subjects with WPW syndrome (age 1-18 years) from any encounter between January 1, 2013, and December 31, 2018. Subjects with congenital heart disease and cardiomyopathy were excluded. The primary outcome was diagnosis of ventricular fibrillation (VF); a composite outcome, LTE, was defined as occurrence of VF and/or cardiac arrest. VF and LTE rates were compared to matched representative controls without WPW syndrome (3:1 ratio). RESULTS: The prevalence of WPW syndrome was 0.03% (8733/26,684,581) over a median follow-up of 1.6 years (interquartile range 0.7-2.9 years). Excluding congenital heart disease/cardiomyopathy, 6946 subjects were analyzed. An LTE occurred in 49 subjects (0.7%), including VF in 20 (0.3%). The incidence of VF was 0.8 events per 1000 person-years, and the incidence of LTE was 1.9 events per 1000 person-years. There were no occurrences of VF in controls; the rate of LTE was 70 times higher in subjects with WPW syndrome (0.7%; 95% confidence interval 0.5%-0.9%) than in controls (0.01%; 95% confidence interval 0%-0.02%). CONCLUSION: The use of a large claims data set allowed for an evaluation of VF and LTE risk in an unselected pediatric population with WPW syndrome. The observed range of 0.8-1.9 events per 1000 person-years is consistent with prior reports from selected populations. A comparison of event rates to matched controls confirms and quantifies the significant elevation in VF and LTE risk in pediatric WPW syndrome.

Single-Center Comparison of Outcomes Following Cardiac Surgery in Low Birth Weight and Standard Birth Weight Neonates.

OBJECTIVE: To compare outcomes between low birth weight (LBW; <2.5 kg) and standard birth weight neonates undergoing cardiac surgery. STUDY DESIGN: A single-center retrospective study of neonates undergoing cardiac surgery with cardiopulmonary bypass from 2012 to 2018. LBW neonates were 1:2 propensity score-matched to standard birth weight neonates (n = 93 to n = 186) using clinical characteristics. The primary and secondary outcomes were survival to hospital discharge and postoperative complications, respectively. After matching, regression analyses were conducted to compare outcomes. RESULTS: The LBW group had a higher proportion of premature neonates than the standard birth weight group (60% vs 8%; P < .01) and were less likely to survive to hospital discharge (88% vs 95%; OR, 0.39; 95% CI, 0.15-0.97). There was no difference in unplanned cardiac reoperations or catheter-based interventions, cardiac arrest, extracorporeal membrane oxygenation, infection, and end-organ complications between the groups. Among LBW infants, survival was improved at weight >2 kg. CONCLUSIONS: LBW is a risk factor for decreased survival. LBW neonates weighing >2 kg have survival comparable to those weighing >2.5 kg.

Spring-mediated cranioplasty versus endoscopic strip craniectomy for sagittal craniosynostosis.

OBJECTIVE: Endoscopic strip craniectomy (ESC) and spring-mediated cranioplasty (SMC) are two minimally invasive techniques for treating sagittal craniosynostosis in early infancy. Data comparing the perioperative outcomes of these two techniques are sparse. Here, the authors hypothesized that outcomes would be similar between patients undergoing SMC and those undergoing ESC and conducted a study using the multicenter Pediatric Craniofacial Surgery Perioperative Registry (PCSPR). METHODS: The PCSPR was queried for infants under the age of 6 months who had undergone SMC or ESC for sagittal synostosis. SMC patients were propensity score matched 1:2 with ESC patients on age and weight. Primary outcomes were transfusion-free hospital course, intensive care unit (ICU) admission, ICU length of stay (LOS), and hospital length of stay (HLOS). The authors also obtained data points regarding spring removal. Comparisons of outcomes between matched groups were performed with multivariable regression models. RESULTS: The query returned data from 676 infants who had undergone procedures from June 2012 through September 2019, comprising 580 ESC infants from 32 centers and 96 SMC infants from 5 centers. Ninety-six SMC patients were matched to 192 ESC patients. There was no difference in transfusion-free hospital course between the two groups (adjusted odds ratio [aOR] 0.78, 95% CI 0.45-1.35). SMC patients were more likely to be admitted to the ICU (aOR 7.50, 95% CI 3.75-14.99) and had longer ICU LOSs (incident rate ratio [IRR] 1.42, 95% CI 1.37-1.48) and HLOSs (IRR 1.28, 95% CI 1.17-1.39). CONCLUSIONS: In this multicenter study of ESC and SMC, the authors found similar transfusion-free hospital courses; however, SMC infants had longer ICU LOSs and HLOSs. A trial comparing longer-term outcomes in SMC versus ESC would further define the roles of these two approaches in the management of sagittal craniosynostosis.

Ectopic Burden via Holter Monitors in Friedreich Ataxia.

BACKGROUND: Friedreich ataxia is the most commonly inherited ataxia; nearly 60% of deaths are cardiac in nature, with one in eight deaths due to arrhythmia. Additional or irregular heartbeats, measured as ectopy, can be quantified using portable heart rhythm monitoring. We sought to describe the ectopic burden in Friedreich ataxia. METHODS: Using a natural history study of patients with Friedreich ataxia at a single center, we analyzed portable heart rhythm monitors (Holters). Ectopic burden was defined as the proportion of atrial or ventricular ectopic beats over total beats. RESULTS: Of 456 patients, 131 had Holters. Sixty-eight (52.0%) were male, median age of symptom onset was 8.0 years (5.0 to 13.0, n = 111), median age at time of Holter was 17.3 years (interquartile range [IQR] 12.9 to 22.8, n = 129), and median duration of illness was 8.7 years (IQR 5.3 to 11.6, n = 110). Median GAA length on the shorter FXN allele was 706.0 (IQR 550.0 to 840.0, n = 112). Eight (7.8%, n = 103) had diminished cardiac function, and 74 (74.0%, n = 100) had ventricular hypertrophy. Ninety patients (83.0%) had atrial ectopy (supraventricular ectopy [SVE]): 85 (78.0%) with rare SVE (>0% to 5%) and five (5.0%) with frequent SVE (>10%). Twenty-five (19.0%) had supraventricular runs, and one (0.8%) had atrial fibrillation/flutter. Forty-five (41.0%) had ventricular ectopy (VE): 43 (39.0%) with rare VE (0% to 5%) and two (2.0%) with moderate VE (5% to 10%). Compared with patients with none and rare SVE, patients with frequent SVE had longer disease duration (18.3 versus 4.6 versus 9.0 years, P = 0.0005). CONCLUSION: Patients with longer disease duration had higher rates of SVE. Heart rhythm monitoring may be considered for risk stratification; however, longitudinal analysis is needed.

Age- and body weight-dependent association between sleep duration and hypertension in US adults: findings from the 2014-2017 National Health Interview Survey.

OBJECTIVES: Previous studies have confirmed the relationship between sleep duration and hypertension. However, there are unanswered questions on how this relationship is affected by age and body mass index (BMI). This study examined the association between sleep duration and hypertension in US adults and evaluated interaction by age and BMI. DESIGN: Nationwide, population-based, cross-sectional survey. SETTING: National Health Interview Survey (NHIS), 2014 to 2017. PARTICIPANTS: Adult participants aged 18 years or older (n = 130,139). MEASUREMENTS: Sleep duration, hypertension, age, and BMI status were assessed based on self-reported survey responses. Odds ratios (ORs) and 95% confidence intervals (CIs) for sleep duration-hypertension associations were estimated by logistic regression, adjusting for potential confounders. RESULTS: The proportion of participants who reported sleeping less than 7 hours (short sleepers) and more than 9 hours (long sleepers) per night was 32% and 4%, respectively. In adjusted analysis, short sleepers had higher odds of hypertension (OR: 1.54, 95% CI: 1.10-2.17). Although not statistically significant, long sleepers also had higher odds of hypertension (OR: 1.28, 95% CI: 0.80-2.05). In stratified analyses by age and BMI, the association between short sleep and hypertension was especially notable in adults aged 18-44 years (OR: 1.25, 95% CI: 1.16-1.35) and adults with normal weight (OR: 1.21, 95% CI: 1.11-1.33). CONCLUSIONS: Short sleep is associated with increased odds of hypertension among American adults and this relationship is dependent on age and BMI.