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Kevin J Chua, Mark Mikhail, Hiren V Patel, Alexandra L Tabakin, Sai Krishnaraya Doppalapudi, Joshua Sterling, Hari SGR Tunuguntla Division of Urology, Department of Surgery, Rutgers, The State University of New Jersey, Robert Wood Johnson Medical School, New Brunswick, NJ, 08901, USACorrespondence: Hari SGR TunuguntlaRutgers, The State University of New Jersey, Robert Wood Johnson Medical School, Division of Urology, Department of Surgery, Medical Education Building, Suite 584-B, 1, RWJ Place, New Brunswick, NJ, 08901, USAEmail tunuguha@rwjms.rutgers.eduAbstract: Female urethral strictures are rare, but underdiagnosed pathologies that can cause voiding dysfunction. These strictures are best managed with open reconstruction, as endoscopic treatments have high rates of failure. A flap urethroplasty can be performed with vaginal, labial or bladder tissue. Meanwhile, graft urethroplasties can utilize vaginal, labial, buccal or lingual tissue. It is important to consider the etiology and type of stricture, local vascularity, and prior attempts at repair when selecting the type of repair. Multiple different techniques have been described with theoretical advantages to each one. While some studies have reviewed a few of the reconstructive techniques to treat female urethral strictures, no single study has accounted for each individual technique. In this review, we discuss techniques captured by a number of systematic reviews and other articles. We will herein focus on reviewing and describing each unique technique of reconstruction in the setting of female urethral stricture.Keywords: urethral stricture, urethral obstruction

Heart failure is the leading cause of morbidity and mortality in adults with congenital heart disease. However, for many in this population, heart transplantation is not possible or requires longer wait times, necessitating prolonged circulatory support. The medium to long-term durable ventricular assist device therapy provides a possible solution. We analyzed outcomes of 9 patients with congenital heart disease and at least 3 years of durable mechanical support, all age 18 or older at the time of ventricular assist device implantation at our affiliated pediatric and adult hospitals. Palliated congenital anatomies varied and included biventricular physiologies as well as single ventricles with Fontan circulation. The median duration of support was 4.2 years, including 3 patients successfully bridged to transplant, averaging 2.1 years on the waitlist. Device-related complications were infrequent with HeartMate 3, a feasible and sustainable option for either bridge to transplant or destination strategies in adults with congenital heart disease., Competing Interests: Disclosure statement C.R.B. reports a financial relationship with Abbott; H.T. reports a relationship with Abbott; I.A. serves as a consultant and proctor for Berlin Heart, Inc., Medtronic Inc., Jarvik Inc., BiVACOR Inc., and Sony-Olympus Medical Solutions Inc.; A.C. reports financial relationships with Medtronic Inc. and Abbott. The remaining authors have no conflicts of interest to report., (Copyright © 2024 International Society for the Heart and Lung Transplantation. Published by Elsevier Inc. All rights reserved.)

Background: The Advanced Cardiac Therapies Improving Outcomes Network (ACTION) began in 2018 as a collaborative learning health system committed to improving outcomes in pediatric heart failure, including children and adults with congenital heart disease, supported with ventricular assist devices (VADs). This report describes patient and device characteristics, and outcomes through 1-year post-implant., Methods: The ACTION VAD registry report was created from data submitted to the ACTION learning network from April 2018 to June 2023. It includes 1,430 devices implanted in 1,220 pediatric patients (≤18) from 57 sites across North America., Results: Males comprised 55% of the registry patients. The median age was 3.7 years with a median implant weight of 13.6 kg; 36% of the cohort was <10 kg. Nearly 40% of patients had a primary diagnosis of congenital heart disease (CHD). Patients with CHD represented 26% of VAD implants in 2018 which increased to 42% in 2023 (p=0.03). At implant, 25% of patients were supported with extracorporeal membrane oxygenation (ECMO), 4.9% with dialysis, and 54% were mechanically ventilated. Paracorporeal pulsatile pumps comprised 40.2% of implants, followed in incidence by paracorporeal continuous flow (28.5%), and implantable continuous flow (24.1%). The number of patients in the VAD Registry patients increased from 102 in 2018 to 256 in 2022, partly reflecting increased center participation in ACTION. Overall survival on support at 1 year was 79.2%, and the incidence of stroke was 13.7%. Infants demonstrated the poorest outcomes, with a 1-year survival of 72.9% and a higher incidence of stroke (20.8%)., Conclusion: The 5-year ACTION VAD experience highlights the growing collaboration in the pediatric VAD community and changes in clinical practice. More work is needed to improve survival and limit adverse outcomes, especially in younger patients., (Copyright © 2025 International Society for the Heart and Lung Transplantation. Published by Elsevier Inc. All rights reserved.)

Objective: To investigate if predictors of wound complications differed between patients undergoing excision and primary anastomosis urethroplasty (EPA) and augmented urethroplasty., Methods: The National Surgical Quality Improvement Program database from 2006 to 2018 was queried for male patients undergoing urethroplasty. Thirty-day wound complications were identified and categorized (superficial/deep/organ-space surgical site infections and dehiscence). Multivariable logistic regression was performed to determine risk factors associated with wound complications. Smoking history was defined as current smoker within the past year., Results: Urethroplasty was performed in 2251 males, with 25.46% (n = 573) using a flap or graft. There was no significant difference in wound complications for patients undergoing augmented urethroplasty (n = 17, 2.97%) or EPA (n = 45, 2.68%) (p = 0.9). The augmented group had a higher BMI, longer operative time, and longer length of stay. On multivariable logistic regression, risk factors associated with wound complications for patients undergoing EPA were diabetes (OR 2.56, p = 0.03) and smoking (OR 2.32, p = 0.02). However, these factors were not associated with wound complications in patients undergoing augmented urethroplasty., Conclusions: Smoking and diabetes were associated with increased wound complications for men undergoing EPA, but not in patients undergoing augmented urethroplasty. Patients with comorbidities associated with worse wound healing may be more likely to have a wound complication when undergoing EPA., (© 2024. The Author(s), under exclusive licence to Springer Nature B.V.)

Background: There is no FDA-approved left ventricular assist device (LVAD) for smaller children permitting routine hospital discharge. Smaller children supported with LVADs typically remain hospitalized for months awaiting heart transplant-a major burden for families and a challenge for hospitals. We describe the initial outcomes of the Jarvik 2015, a miniaturized implantable continuous flow LVAD, in the NHLBI-funded Pumps for Kids, Infants, and Neonates (PumpKIN) study, for bridge-to-heart transplant., Methods: Children weighing 8 to 30 kg with severe systolic heart failure and failing optimal medical therapy were recruited at 7 centers in the United States. Patients with severe right heart failure and single-ventricle congenital heart disease were excluded. The primary feasibility endpoint was survival to 30 days without severe stroke or non-operational device failure., Results: Of 7 children implanted, the median age was 2.2 (range 0.7, 7.1) years, median weight 10 (8.2 to 20.7) kilograms; 86% had dilated cardiomyopathy; 29% were INTERMACS profile 1. The median duration of Jarvik 2015 support was 149 (range 5 to 188) days where all 7 children survived including 5 to heart transplant, 1 to recovery, and 1 to conversion to a paracorporeal device. One patient experienced an ischemic stroke on day 53 of device support in the setting of myocardial recovery. One patient required ECMO support for intractable ventricular arrhythmias and was eventually transplanted from paracorporeal biventricular VAD support. The median pump speed was 1600 RPM with power ranging from 1-4 Watts. The median plasma free hemoglobin was 19, 30, 19 and 30 mg/dL at 7, 30, 90 and 180 days or time of explant, respectively. All patients reached the primary feasibility endpoint. Patient-reported outcomes with the device were favorable with respect to participation in a full range of activities. Due to financial issues with the manufacturer, the study was suspended after consent of the eighth patient., Conclusion: The Jarvik 2015 LVAD appears to hold important promise as an implantable continuous flow device for smaller children that may support hospital discharge. The FDA has approved the device to proceed to a 22-subject pivotal trial. Whether this device will survive to commercialization remains unclear because of the financial challenges faced by industry seeking to develop pediatric medical devices. (Supported by NIH/NHLBI HHS Contract N268201200001I, clinicaltrials.gov 02954497)., (Copyright © 2024 International Society for the Heart and Lung Transplantation. All rights reserved.)

Background: Traditionally, low cardiac output has been considered the primary hemodynamic driver of renal function and injury. Adult data suggest that central venous pressure (CVP) is a more important factor., Objectives: The authors hypothesized that in children with cardiovascular disease, higher CVP predicts lower estimated glomerular filtration rate (eGFR) and worsening renal function (WRF)., Methods: We performed a single-center cohort study of patients aged 3 months to 21 years with biventricular circulation undergoing cardiac catheterization. Pearson's correlation and linear and Cox regression analyses were performed to determine associations with eGFR at the time of catheterization and WFR within 180 days after catheterization., Results: 312 patients had median age 7.9 years (IQR: 2.3 to 14.5 years), median eGFR 97 mL/min/1.73 m 2 (IQR: 81-118 mL/min/1.73 m 2 ), median CVP 7 mm Hg (IQR: 5-9 mm Hg), and median cardiac index 3.7 mL/min/m 2 (IQR: 2.9-4.6 mL/min/m 2 ). Nearly half (48%) were transplant recipients. In multivariable analysis, CVP was independently associated with eGFR (β = -2.65; 95% CI: -4.02, -1.28; P  < 0.001), as was being a transplant recipient (β = -10.20; 95% CI: -17.74, -2.65; P  = 0.008), while cardiac index was not. Fifty-one patients (16%) developed WRF. In a proportional hazards model adjusting for cardiac index, only higher CVP (HR: 1.10; 95% CI: 1.04-1.17; P  = 0.002) and greater contrast volume by weight (HR: 1.05; 95% CI: 1.01-1.10; P  = 0.021) predicted WRF. CVP ≥7 mm Hg likewise predicted WRF (HR: 2.57; 95% CI: 1.29-5.12; P  = 0.007)., Conclusions: Among children with a spectrum of cardiovascular disease, higher CVP is associated with lower eGFR and development of WRF, independent of cardiac index., Competing Interests: The authors have reported that they have no relationships relevant to the contents of this paper to disclose., (© 2024 The Authors.)

Objective: We report the largest pediatric single-center experience with an Impella (Abiomed Inc) catheter-based axial pump support., Methods: We conducted a retrospective cohort study of all patients with acute decompensated heart failure or cardiogenic shock requiring catheter-based axial pump support between October 2014 and February 2022. The primary outcome per individual encounter (hospital admission) was defined as bridge-to-recovery, bridge-to-durable ventricular assist device support, bridge-to-cardiac transplantation, or death at 6 months after catheter-based axial pump explantation. Adverse events were defined according to the Pediatric Interagency Registry for Mechanical Circulatory Support criteria., Results: Our final study cohort included 37 encounters with 43 catheter-based axial pump implantations. A single catheter-based axial pump device was used for support in 33 encounters (89%), with 2 catheter-based axial pump devices used in 3 (8%) separate encounters and 3 catheter-based axial pump devices used in 1 (3%) encounter. The median [range] age, weight, and body surface area at implantation were 16.8 [6.9-42.8] years, 61.1 [23.1-123.8] kg, and 1.7 [0.8-2.5] m 2 , respectively. The predominant causes of circulatory failure were graft failure/rejection in 16 patients (43%), followed by cardiomyopathy in 7 patients (19%), arrhythmia refractory to medical therapies in 6 patients (16%), myocarditis/endocarditis in 4 patients (11%), and heart failure due to congenital heart disease in 4 patients (11%). Competing outcomes analysis showed a positive outcome with bridge-to-recovery in 58%, bridge-to-durable VAD support in 14%, and bridge-to-cardiac transplantation in 14% at 6 months. Fourteen percent of encounters resulted in death at 6 months., Conclusions: We demonstrate that catheter-based axial pump support in children results in excellent 1- and 6-month survival with an acceptable adverse event profile., (Copyright © 2022. Published by Elsevier Inc.)

Background: PRDM16 plays a role in myocardial development through TGF-β (transforming growth factor-beta) signaling. Recent evidence suggests that loss of PRDM16 expression is associated with cardiomyopathy development in mice, although its role in human cardiomyopathy development is unclear. This study aims to determine the impact of PRDM16 loss-of-function variants on cardiomyopathy in humans., Methods: Individuals with PRDM16 variants were identified and consented. Induced pluripotent stem cell-derived cardiomyocytes were generated from a proband hosting a Q187X nonsense variant as an in vitro model and underwent proliferative and transcriptional analyses. CRISPR (clustered regularly interspaced short palindromic repeats)-mediated knock-in mouse model hosting the Prdm16 Q187X allele was generated and subjected to ECG, histological, and transcriptional analysis., Results: We report 2 probands with loss-of-function PRDM16 variants and pediatric left ventricular noncompaction cardiomyopathy. One proband hosts a PRDM16-Q187X variant with left ventricular noncompaction cardiomyopathy and demonstrated infant-onset heart failure, which was selected for further study. Induced pluripotent stem cell-derived cardiomyocytes prepared from the PRDM16-Q187X proband demonstrated a statistically significant impairment in myocyte proliferation and increased apoptosis associated with transcriptional dysregulation of genes implicated in cardiac maturation, including TGF-β-associated transcripts. Homozygous Prdm16 Q187X/Q187X mice demonstrated an underdeveloped compact myocardium and were embryonically lethal. Heterozygous Prdm16 Q187X/WT mice demonstrated significantly smaller ventricular dimensions, heightened fibrosis, and age-dependent loss of TGF-β expression. Mechanistic studies were undertaken in H9c2 cardiomyoblasts to show that PRDM16 binds TGFB3 promoter and represses its transcription., Conclusions: Novel loss-of-function PRDM16 variant impairs myocardial development resulting in noncompaction cardiomyopathy in humans and mice associated with altered TGF-β signaling., Competing Interests: Disclosures None.