Heart transplantation in neonatal Marfan syndrome: Saving life in a rare and fatal condition.

Background: Neonatal Marfan syndrome is a rare disease with mortality in the first year of life reported as high as 95% predominantly due to progressive heart failure from valvar regurgitation and cardiomyopathy. Multisystem involvement and uncertain prognosis have historically precluded transplant candidacy, and current management options are of limited success.

Case Report: We present a baby girl with a postnatal diagnosis of neonatal Marfan syndrome who at 1 year of age underwent mitral valve and tricuspid valve repair with postoperative profound left ventricular and moderate right ventricular dysfunction necessitating biventricular assist device (BiVAD) support and subsequent heart transplant.

Optimal Donor Allograft Function: The Search for the Lowest Acceptable Donor Left Ventricular Ejection Fraction in Pediatric Heart Transplantation.

Background: The availability of heart donors is limited by organ shortage. Due to concerns of reduced survival, donors with depressed left ventricular ejection fraction (LVEF <50%) have been cautiously used in pediatric heart transplantation. One strategy to expand the donor pool is to re-evaluate whether lower donor LVEF may be acceptable for transplantation.

Waitlist and post-transplant outcomes for children with myocarditis listed for heart transplantation over 3 decades.

Background: There is limited and conflicting information on waitlist and transplant outcomes for children with myocarditis.

Methods: Retrospective review included children with myocarditis and dilated cardiomyopathy (DCM) listed for HT from January 01, 1993 to December 31, 2019 in the Pediatric Heart Transplant Society database. Clinical characteristics, waitlist and post-HT outcomes (graft loss, rejection, cardiac allograft vasculopathy, infection and malignancy) for children listed from early (1993-2008) and current era (2009-2019) with myocarditis were evaluated and compared to those with DCM.

George Daicoff: A Pioneering Surgeon and Humanitarian of The Southern Thoracic Surgical Association.

George Daicoff was a true pioneer who excelled in patient care, research, teaching, and advocacy; he performed cardiac surgery on thousands. He trained at Mayo Clinic under the tutelage of John Kirklin in 1966. He served on the faculty at University of Florida from 1967 to 1977 and became Chief of Thoracic and Cardiovascular Surgery at University of Florida.

Venous Thromboembolism in Premature Neonates.

While the incidence of venous thromboembolism (VTE) is lower among children than adults, the newborn period is one of two bimodal peaks (along with adolescence) in VTE incidence in the pediatric population. Most VTE cases in neonates occur among critically ill neonates being managed in the neonatal intensive care unit, and most of these children are born premature. For this reason, the presentation, diagnosis, management, and outcomes of VTE among children born premature deserve special emphasis by pediatric hematologists, neonatologists, pharmacists, and other pediatric health care providers, as well as by the scientific community, and are described in this review.

ENNOBLE-ATE trial: an open-label, randomised, multi-centre, observational study of edoxaban for children with cardiac diseases at risk of thromboembolism.

Children with cardiac diseases suffer from significant morbidity and mortality secondary to thromboembolic complications. Anticoagulant agents currently used for thromboprophylaxis have many limitations, including subcutaneous administration (low molecular weight heparins) and requirement for frequent monitoring via venipuncture (vitamin K antagonists). Edoxaban is an oral direct factor Xa inhibitor without need of monitoring.

Heart Transplantation for Pediatric and Congenital Cardiac Disease: A Comparison of Two Eras over 23 Years and 188 Transplants at a Single Institution.

Background: To assess changes in patterns of practice and outcomes over time, we reviewed all patients who underwent heart transplantation (HTx) at our institution and compared two consecutive eras with significantly different immunosuppressive protocols (cohort 1 [80 HTx, June 1995-June 2006]; cohort 2 [108 HTx, July 2006-September 2018]).

Methods: Retrospective study of 180 patients undergoing 188 HTx (June 1995-September 2018; 176 first time HTx, 10 second HTx, and 2 third HTx). In 2006, we commenced pre-HTx desensitization for highly sensitized patients and started using tacrolimus as our primary postoperative immunosuppressive agent.

Cardiac Biomarkers Associated With Hospital Length of Stay After Pediatric Congenital Heart Surgery.

Background: Prolonged hospital length of stay after congenital heart surgery is a significant cost burden and is associated with postoperative morbidity. Our goal was to evaluate the association between pre- and postoperative biomarker levels and in-hospital length of stay for children after congenital heart surgery.

Methods: We enrolled patients <18 years of age who underwent at least 1 congenital heart operation at Johns Hopkins Hospital from 2010 to 2014.

Registry-based trials: a potential model for cost savings?

Background/aims: Registry-based trials have emerged as a potentially cost-saving study methodology. Early estimates of cost savings, however, conflated the benefits associated with registry utilisation and those associated with other aspects of pragmatic trial designs, which might not all be as broadly applicable. In this study, we sought to build a practical tool that investigators could use across disciplines to estimate the ranges of potential cost differences associated with implementing registry-based trials versus standard clinical trials.

Associations between anthropometric indices and outcomes of congenital heart operations in infants and young children: An analysis of data from the Society of Thoracic Surgeons Database.

Background: Children with congenital heart disease are at risk for growth failure due to inadequate nutrient intake and increased metabolic demands. We examined the relationship between anthropometric indices of nutrition (height-for-age z-score [HAZ], weight-for-age z-score [WAZ], weight-for-height z-score [WHZ]) and outcomes in a large sample of children undergoing surgery for congenital heart disease.

Methods: Patients in the Society of Thoracic Surgeons Congenital Heart Surgery Database having index cardiac surgery at age 1 month to 10 years were included.

Equivalent Survival Between Lobectomy and Segmentectomy for Clinical Stage IA Lung Cancer.

Background: The oncologic efficacy of segmentectomy is controversial. We compared long-term survival in clinical stage IA (T1N0) Medicare patients undergoing lobectomy and segmentectomy in The Society of Thoracic Surgeons database.

Methods: The Society of Thoracic Surgeons General Thoracic Surgery Database was linked to Medicare data in 14,286 lung cancer patients who underwent segmentectomy (n = 1654) or lobectomy (n = 12,632) for clinical stage IA disease from 2002 to 2015.

Post-operative blood loss is higher among African American neonates undergoing open-heart surgery with cardiopulmonary bypass for CHD.

Background: Neonates are at high risk of bleeding after open-heart surgery. We sought to determine pre-operative and intra-operative risk factors for increased bleeding after neonatal open-heart surgery with cardiopulmonary bypass.

Methods: We conducted a retrospective cohort study of neonates (0-30 days old) who underwent open-heart surgery with cardiopulmonary bypass from January, 2009, to March, 2013.

The world database for pediatric and congenital heart surgery: A collaboration with the Registro Nacional de Cirugía Cardiaca Pediátrica.

Objective: Following the notable work accomplished by the Mexican Association of Specialists in Congenital Heart Disease (Asociación Mexicana de Especialistas en Cardiopatías Congénitas) with the development of a national registry for congenital cardiac surgery, the World Society for Pediatric and Congenital Heart Surgery has implemented an international platform to collect data and analyze outcomes of children with congenital heart disease.

Methodology: This manuscript proposes a possible collaboration between Mexico's national congenital cardiac database (Registro Nacional de Cirugía Cardíaca Pediátrica) and the World Database for Pediatric and Congenital Heart Surgery.

Conclusion: Such a partnership would advance the countries' desire for the ongoing development of quality improvement processes and improve the overall treatment of children with congenital heart disease.

Longitudinal Outcomes After Surgical Repair of Postinfarction Ventricular Septal Defect in the Medicare Population.

Background: Patients undergoing post infarction ventricular septal defect repair are at high risk for early morbidity and mortality, but little is known about subsequent clinical events. This study uses short-term clinical data from The Society of Thoracic Surgeons National Database linked with Medicare data to examine longer term outcomes in these patients.

Methods: This was a retrospective review of The Society of Thoracic Surgeons National Database to link with Medicare data all adults (≥65 years) who underwent ventricular septal defect repair after a myocardial infarction between 2008 and 2012.

Early identification of impending cardiac arrest in neonates and infants in the cardiovascular ICU: a statistical modelling approach using physiologic monitoring data.

Objective: To develop a physiological data-driven model for early identification of impending cardiac arrest in neonates and infants with cardiac disease hospitalised in the cardiovascular ICU.

Methods: We performed a single-institution retrospective cohort study (11 January 2013-16 September 2015) of patients ≤1 year old with cardiac disease who were hospitalised in the cardiovascular ICU at a tertiary care children's hospital. Demographics and diagnostic codes of cardiac arrest were obtained via the electronic health record.

Enhancing efficiency and scientific impact of a clinical trials network: the Pediatric Heart Network Integrated CARdiac Data and Outcomes (iCARD) Collaborative.

Recent years have seen an exponential increase in the variety of healthcare data captured across numerous sources. However, mechanisms to leverage these data sources to support scientific investigation have remained limited. In 2013 the Pediatric Heart Network (PHN), funded by the National Heart, Lung, and Blood Institute, developed the Integrated CARdiac Data and Outcomes (iCARD) Collaborative with the goals of leveraging available data sources to aid in efficiently planning and conducting PHN studies; supporting integration of PHN data with other sources to foster novel research otherwise not possible; and mentoring young investigators in these areas.

Use of Near-Infrared Spectroscopy to Monitor Lower Extremity Perfusion in Pediatric Patients Undergoing Cardiac Catheterization.

Acute femoral artery occlusion is common in pediatric patients following cardiac catheterization. A variety of means are utilized to assess lower extremity (LE) perfusion and arterial patency following cardiac catheterization including palpation of pulses, pulse oximetry, subjective assessment of lower extremity color and temperature, and ultrasound. We sought to evaluate the utility of Near-Infrared Spectroscopy (NIRS) to monitor LE perfusion in pediatric patients undergoing cardiac catheterization.

The Society of Thoracic Surgeons Congenital Heart Surgery Database: 2019 Update on Research.

As the largest congenital and pediatric cardiac surgical clinical data registry in the world, The Society of Thoracic Surgeons Congenital Heart Surgery Database (STS CHSD) serves as a platform for reporting of outcomes and for quality improvement. In addition, it is an important source of data for clinical research and for innovations related to quality measurement. Each year, several teams of investigators undertake analyses of data in the STS CHSD pertaining to the surgical management of specific diagnostic and procedural groups, or to specific processes of care, and their associations with patient characteristics and outcomes across centers participating in the STS CHSD.

Novel Biomarkers Improve Prediction of 365-Day Readmission After Pediatric Congenital Heart Surgery.

Background: The purpose of this study was to evaluate the association between preoperative biomarker levels and 365-day readmission or mortality after pediatric congenital heart surgery.

Methods: Children aged 18 years or younger undergoing congenital heart surgery (n = 145) at Johns Hopkins Hospital from 2010 to 2014 were enrolled in the prospective cohort. Novel biomarkers suppression of tumorgenicity 2, galectin-3, N-terminal prohormone brain natriuretic peptide, and glial fibrillary acidic protein were measured.

The world database for pediatric and congenital heart surgery: A collaboration with the Registro Nacional de Cirugía Cardiaca Pediátrica.

Objective: Following the notable work accomplished by the Mexican Association of Specialists in Congenital Heart Disease (Asociación Mexicana de Especialistas en Cardiopatías Congénitas) with the development of a national registry for congenital cardiac surgery, the World Society for Pediatric and Congenital Heart Surgery has implemented an international platform to collect data and analyze outcomes of children with congenital heart disease.

Methodology: This manuscript proposes a possible collaboration between Mexico's national congenital cardiac database (Registro Nacional de Cirugía Cardíaca Pediátrica) and the World Database for Pediatric and Congenital Heart Surgery.

Conclusion: Such a partnership would advance the countries' desire for the ongoing development of quality improvement processes and improve the overall treatment of children with congenital heart disease.

Ten Years of Data Verification: The Society of Thoracic Surgeons Congenital Heart Surgery Database Audits.

Background: The completeness and accuracy of data contained within clinical databases and registries is critical to the reliability of reports emanating from these platforms. Therefore, vigorous data verification processes are a core competency of any mature database or registry. The Society of Thoracic Surgeons Congenital Heart Surgery Database (STS CHSD) has conducted audits of participant data for just over ten years.